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Mild cylindrical bronchiectasis: Bronchiectasis Treatment, Definition, Symptoms, Causes, Prognosis

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Diagnostic imaging in adult non-cystic fibrosis bronchiectasis

Abstract

Radiology plays a key role in the diagnosis of bronchiectasis, defined as permanent dilatation of the bronchial lumen. Volumetric thin-section multidetector computed tomography is an excellent noninvasive modality to evaluate bronchiectasis. Bronchiectasis is categorised by morphological appearance. Cylindrical bronchiectasis has a smooth tubular configuration and is the most common form. Varicose bronchiectasis has irregular contours with alternating dilating and contracting lumen. Cystic bronchiectasis is the most severe form and exhibits saccular dilatation of bronchi. Bronchial dilatation is the hallmark of bronchiectasis and is evaluated in relation to the accompanying pulmonary artery. A broncho–arterial ratio exceeding 1:1 should be considered abnormal. Normal bronchi are narrower in diameter the further they are from the lung hila. Lack of normal bronchial tapering over 2 cm in length, distal from an airway bifurcation, is the most sensitive sign of bronchiectasis. Findings commonly associated with bronchiectasis include bronchial wall thickening, mucus plugging and tree-in-bud opacities. Bronchiectasis results from a myriad of conditions, with post-infectious bronchiectasis being the most common. Imaging can sometimes discern the cause of bronchiectasis. However, in most cases it is nonspecific or only suggestive of aetiology. While morphological types are nonspecific, the distribution of abnormality offers clues to aetiology.

Key points

  • Bronchiectasis is a chronic progressive condition with significant disease burden and frequent exacerbations, for which the diagnosis relies on cross-sectional imaging.

  • The major imaging findings include bronchial dilatation, bronchial contour abnormalities and visualisation of the normally invisible peripheral airways.

  • Bronchiectasis is the end result of various conditions, including immunodeficiencies, mucociliary disorders and infections. Imaging is often nonspecific with regard to aetiology but can be suggestive.

  • Distribution of abnormality in the lung offers helpful clues for establishing aetiology.

Educational aims

  • To review the cross-sectional imaging appearance of bronchiectasis and the common associated findings.

  • To get a sense of how radiology can aid in establishing the aetiology of bronchiectasis.

Abstract

Bronchiectasis is a chronic progressive condition with significant disease burden and frequent exacerbations for which the diagnosis relies on cross-sectional imaging http://bit.ly/2NxOLky

Introduction

Diagnostic imaging plays a key role in the diagnosis of bronchiectasis, defined as permanent dilatation of the bronchial lumen. Bronchiectasis is increasing in incidence and frequently encountered on imaging [1]. Bronchiectasis carries significant disease burden, with reduction in health-related quality of life and frequent exacerbations often requiring inpatient management, and it is associated with increased mortality [2]. Its pathogenesis is thought to result from an initial insult to the airway followed by a vicious cycle of repeated infection and ineffective mucociliary clearance. Disruption of the peribronchial interstitial scaffolding and outward traction is thought to be its pathogenesis in relation to fibrotic lung disease. Bronchiectasis is classically categorised by morphological appearance [3]. Cylindrical bronchiectasis has a smooth tubular configuration and is the most common form. Varicose bronchiectasis is often seen with fibrosis and has irregular contours with alternating dilating and contracting lumen. Cystic bronchiectasis is the most severe form and exhibits saccular dilatation of bronchi, often with air–fluid levels. Volumetric thin-section multidetector computed tomography (CT) is an excellent noninvasive modality to evaluate bronchiectasis and allows for contiguous millimetre image reconstructions, multiplanar reformations and minimal intensity projections [4].

In this concise pictorial review, we revisit the imaging hallmarks of bronchiectasis and associated imaging findings, along with pitfalls and clues to common aetiologies for the respiratory clinician. We limit the review to non-cystic fibrosis bronchiectasis in adults, with a focus on bronchiectasis in non-fibrotic lung disease.

Imaging findings

The diagnostic imaging findings in bronchiectasis are summarised in table 1. Bronchial dilatation is the hallmark of bronchiectasis and is evaluated in relation to the accompanying pulmonary artery (figure 1a). In practical terms, a broncho–arterial ratio exceeding 1:1 should be considered abnormal. Numerous physiological and disease-related conditions may affect the ratio and a cut-off of >1.3:1 or 1.5:1 results in increased specificity [5, 6]. Examples of other common causes of dilated bronchi and how to differentiate these from bronchiectasis are presented in table 2. Mild bronchial dilation can, for instance, be seen in high-altitude habitation and normal ageing. Pulmonary artery diameter can also be affected by vasoconstriction and pulmonary hypertension [6]. An important pitfall with the finding of bronchial dilatation is that mild reversible bronchial dilatation can be associated with atelectasis or inflammation, such as infectious pneumonia. This may be difficult to distinguish from true bronchiectasis, which is permanent, and precaution should be used in patients with signs of infection (figure 2).

Table 1

Diagnostic imaging findings in bronchiectasis

Figure 1

Imaging findings in bronchiectasis. a) The arrow points to cylindrical bronchiectasis in the right lower lobe. The bronchi are dilated in relation to the pulmonary artery, producing a signet ring sign. There are also visible airways in the subpleural 1 cm of lung, demarcated by the dashed line. Airways are normally not visible peripherally and when seen are indicative of bronchiolectasis. b) Curved planar reformation shows lack of bronchial tapering in the right lower lung, in keeping with cylindrical bronchiectasis (arrow). This is the earliest and most sensitive sign of bronchiectasis. The aetiology of bronchiectasis in this patient was bronchiolitis obliterans after allogeneic haematopoietic stem cell transplantation. c) Different morphological types of bronchiectasis are often seen in the same patient. This patient had cylindrical bronchiectasis (arrow) with lack of bronchial tapering in the right lower lobe and varicose bronchiectasis in the right middle lobe (arrowhead). Tree-in-bud opacities, a common associated finding, are also noted (#).

Table 2

Examples of other common causes of dilated bronchi

Figure 2

An important pitfall: mild reversible bronchial dilatation due to infection. This patient’s clinical presentation gave concern for lower respiratory infection. a) Initial CT demonstrated bronchial dilatation with bronchial wall thickening (arrows) and areas of air space opacification (arrowhead), in keeping with bronchopneumonia. b) Subsequent imaging after antibiotic treatment revealed resolution of the air space opacification and a return of the bronchi to normal calibre. This does not reflect true bronchiectasis, which is irreversible.

Normal bronchi are narrower in diameter the further they are from the lung hila. Lack of normal bronchial tapering over 2 cm in length, distal from an airway bifurcation, is the most sensitive sign of bronchiectasis and is helpful in evaluating subtle cylindrical bronchiectasis (figure 1b) [7]. Visible airways in the 1-cm vicinity of costal subpleural lung are another useful sign of bronchiectasis (figure 1a). Airways are normally not visible peripherally in the lung and when visible are suggestive of dilatation of the non-cartilage-containing small airways, i.e. bronchiolectasis. Visible airways in the 1-cm vicinity of mediastinal subpleural lung are seen in many patients with bronchiectasis but also in a significant number of normal subjects and should, therefore, be considered a sign of bronchiectasis only when directly abutting the mediastinal pleural surface [8].

Findings commonly associated with bronchiectasis include bronchial wall thickening, mucus plugging and tree-in-bud opacities (figure 1c and table 1). These findings serve as indirect signs and can increase the radiologist’s confidence in diagnosing mild bronchiectasis. Bronchial wall thickening is a potentially reversible finding and correlates with patient-reported symptoms, health status and ­frequency of exacerbation [9–11]. This finding, often subjectively evaluated, usually represents airway inflammation and can be seen in asthma and bronchitis but is also occasionally observed in normal individuals [5]. Tree-in-bud opacities represent mucus plugging or inflammatory material within the bronchioles at the level of the secondary pulmonary lobule and are usually a sign of endobronchial infection.

Common causes and imaging clues to aetiology

Bronchiectasis can result from a myriad of conditions, including immunodeficiencies, mucociliary disorders and infections, with post-infectious bronchiectasis being the most common [12]. Despite thorough diagnostic workup, the cause in many cases remains unknown [2]. Imaging can sometimes discern the cause of bronchiectasis, such as in the case of foreign bodies, obstructing tumours or post-radiation fibrosis (figure 3). However, in most cases it is nonspecific or only suggestive of aetiology. Morphological types are mostly nonspecific. Distribution of abnormalities, however, offers clues to aetiology. Upper lobe predominance is seen in cystic fibrosis, sarcoidosis (figure 4a), post-tuberculous scarring (figure 4b) and post-radiation fibrosis (figure 3). Calcified hilar or mediastinal lymph nodes and calcified pulmonary granulomas suggest granulomatous infection, such as tuberculosis. Anterior segment distribution (middle lobe and lingula) is typically seen in atypical mycobacterial infection, which often affects middle-aged or elderly female patients (figure 4c). Mycobacterium avium–intracellulare complex (MAC) infections usually affect two or more lobes, often with scarring and a few small nodules up to 1 cm in size. Consolidation and cavitation may also be a feature (figure 4d). Lower lobe distribution is most often seen in post-infectious bronchiectasis, chronic aspiration (figure 5), immunodeficiencies (figure 6a) and primary ciliary dyskinesia (figure 6b). Allergic bronchopulmonary aspergillosis (ABPA) bears mention due to the characteristic imaging appearance and need for specific medical management (figure 7). This condition is caused by a hypersensitivity reaction to endobronchial Aspergillus and imaging typically demonstrates central bronchiectasis with extensive mucus plugging, which in up to a fifth of cases is characteristically high attenuating (>70–100 HU) and is thought to be due to iron and manganese accumulation in the fungal debris. Location of bronchiectasis can have treatment implications in some cases, as focal bronchiectasis may be considered for lobectomy or segmentectomy, with good clinical results such as in middle lobe syndrome [13].

Figure 3

Post-radiation fibrosis. a) This patient had non-small cell lung cancer in the right lower lobe (not shown). The arrows show ground-glass opacities arising after radiation therapy, representing radiation pneumonitis. b) 4 months later there was interval development of post-radiation fibrosis with varicose bronchiectasis and volume loss in the treatment field (#).

Figure 4

a) Varicose bronchiectasis (arrow) in a patient with sarcoidosis. Note the typical upper lobe location and associated reticulation and lung architectural distortion (#), suggesting fibrosis. b) Post-tuberculous bronchiectasis in a 90-year-old patient with a previous history of tuberculosis. The apical unilateral distribution is typical. c) Bronchiectasis in the right middle lobe and lingula in a 50-year-old female patient with a MAC infection. The anterior lung segment involvement is typical. d) Bronchiectasis in a different elderly female patient with severe MAC infection. Bilateral cylindrical bronchiectasis is noted as well as nodules (arrowhead), some of which have benign pattern central calcification. Consolidation with cavitation (arrow) is seen in the left lower lobe.

Figure 5

a) An 88-year-old female patient with chronic obstructive pulmonary disease (COPD), dysphagia and frequent lower respiratory infections. The arrow points to aspirated contrast material in the trachea. b) CT in the same patient shows bilateral lower zone bronchiectasis (arrowheads) and tree-in-bud opacities (#). The presumed aetiology was chronic aspiration.

Figure 6

a) Bronchiectasis in immunodeficiency. This patient had hyperimmunoglobulin E syndrome (Job’s syndrome) and extensive bronchiectasis in the right lower lobe complicated by consolidation and an abscess formation (arrow). b) Ancillary findings with bronchiectasis. This patient had primary ciliary dyskinesia with widespread mucus plugging and adjacent consolidation, suggesting infection in the right lower lobe and middle lobe (arrowheads). The arrow points to bronchial wall thickening. Note the situs inversus.

Figure 7

A 71-year-old male with a history of asthma presented with a 3-month history of productive cough. This patient had a positive sputum culture for Aspergillus and peripheral blood eosinophilia. Skin-prick test was positive for Aspergillus and serum IgE levels, including Aspergillus-specific IgE levels, were markedly elevated. a) Non-contrast-enhanced CT shows high-attenuation mucus plugging (>70 HU) within dilated central airways (arrow) and atelectasis typical for ABPA. b) Follow-up imaging after antifungal and steroid treatment revealed resolution of the mucus plugging and atelectasis with residual cylindrical bronchiectasis (arrow).

Complications

Bronchiectasis is a chronic progressive disease characterised by acute respiratory infections. Chronic airway inflammation promotes neovascularisation and arterial remodelling, resulting in fragile vessels that are prone to bleeding. This can cause haemoptysis, which is sometimes the presenting symptom in bronchiectasis and less commonly results in massive haemoptysis, requiring surgical intervention or therapeutic catheter angiography (figure 8a and b). Massive haemoptysis is due to bleeding from the bronchial arteries in the majority of cases (figure 8c) and less commonly from pulmonary arteries or non-bronchial systemic arteries [14].

Figure 8

a) CT pulmonary angiogram of a 70-year-old patient with Global Initiative for Chronic Obstructive Lung Disease (GOLD) stage four COPD on long-term oxygen therapy presenting with massive haemoptysis. Magnified sagittal reformation shows focal contour abnormality of a subsegmental pulmonary artery in keeping with a pseudoaneurysm associated with cylindrical bronchiectasis (arrowhead). The arrow points to adjacent air space opacities representing alveolar blood. b) Volume-rendered image of the pseudoaneurysm. The bleeding resolved with medical treatment and the pseudoaneurysm, bronchial wall thickening and air space opacities resolved on follow-up imaging. Case courtesy of Agusta Andresdottir (Landspitali University Hospital, Reykjavik, Iceland). c) Enlarged (>2 mm diameter) and tortuous bronchial arteries (arrow) in a different patient with severe bilateral bronchiectasis. This finding is not specific to bronchiectasis and is seen in disorders affecting the pulmonary circulation, and chronic infectious or inflammatory disease, as well as congenital conditions.

Conclusion

Bronchiectasis is a common finding on CT examinations of the chest. It is defined as permanent dilatation of the bronchial lumen, thus requiring radiological imaging for diagnosis. The condition carries significant disease burden, which makes it important for radiologists and clinicians to have detailed knowledge of its imaging appearance and associated findings, as well as findings that may aid in establishing aetiology.

Footnotes

  • Conflict of interest: G. Juliusson has nothing to disclose.

  • Conflict of interest: G. Gudmundsson has nothing to disclose.

  • Support statement: Funding was received from the Landspitali Research Fund (A-2018-023).

What is bronchiectasis | British Lung Foundation

Bronchiectasis is a long-term condition that affects the airways in your lungs.

On this page:


What is bronchiectasis?

When you breathe, air is carried into your lungs through your airways, also called bronchi. The bronchi divide again and again into thousands of smaller airways called bronchioles. Your airways contain tiny glands that produce a small amount of mucus. Mucus helps to keep your airways moist, and traps the dust and germs that you breathe in. The mucus is moved away by tiny hairs, called cilia, which line your airways.

If you have bronchiectasis, your airways are widened and inflamed with thick mucus, also called phlegm or sputum. Your airways may not clear themselves properly. This means mucus builds up and your airways can become infected by bacteria. Pockets in the airways mean that mucus gets trapped and is likely to get infected.

Sometimes, if the number of bacteria multiply, you’ll get a chest infection or a flare-up of your symptoms. It’s important to recognise and treat chest infections. If you don’t get treatment, your airways may be damaged further. The changes to your airways can’t be reversed, but there are ways you and your health care team can treat and manage bronchiectasis.

Bronchiectasis is sometimes called non-cystic fibrosis bronchiectasis. This is because there is a different condition called cystic fibrosis. People who have cystic fibrosis can have lung symptoms similar to those of bronchiectasis, but the treatments and outlook are different.


What are the symptoms of bronchiectasis?

Symptoms of bronchiectasis vary between people. You may have 1 or 2 on the list below, or you may have most of them.

The most common symptom is a long-term cough, usually coughing up sputum, sometimes called phlegm. The amount varies. For people with more severe bronchiectasis it can be quite large amount, for example an egg cup full or more every day. Some people might have a dry cough with no or very little sputum.

Frequent chest infections are also a common feature of bronchiectasis.

Other symptoms of bronchiectasis you might have are:

  • feeling very tired or finding it difficult to concentrate
  • breathlessness: having difficulty breathing or feeling short of breath
  • problems with your sinuses
  • cough incontinence, also called bladder leakage
  • anxiety or depression

Less common symptoms of bronchiectasis include:

  • coughing up blood
  • chest pain

What causes bronchiectasis?

For up to half of people diagnosed with bronchiectasis, there’s no clear underlying cause. This is called idiopathic bronchiectasis.

Some illnesses linked to bronchiectasis include:

  • having had severe lung infections such as pneumonia, whooping cough or tuberculosis (TB) in the past, for example when you were a child
  • inflammatory bowel disease, also called ulcerative colitis and Crohn’s disease
  • immune system deficiencies
  • some types of arthritis, such as rheumatoid arthritis
  • a problem with the normal structure or function of your lungs, such as primary ciliary dyskinesia
  • asthma, COPD or sarcoidosis

Other causes include:

  • a severe allergic response to fungus or moulds such as Aspergillus
  •  gastric reflux
  • a blockage of your airways, caused by breathing in a small item such as a nut

Next: diagnosing bronchiectasis >

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What is Bronchiectasis? | Mount Sinai

Patients with bronchiectasis cannot clear bacteria and mucus from their lungs because of a widening and inflammation of their airways.

Symptoms of Bronchiectasis

If left untreated, symptoms may progress to include worsening shortness of breath, deteriorating quality of life, and heart failure. If you think you may have this disease, Mount Sinai’s pulmonologists are experts in its diagnosis. Symptoms of bronchiectasis include: 

  • Cough, which may produce mucus (in the case of infections, the mucus may be discolored, foul-smelling, and contain blood)
  • Shortness of breath
  • Wheezing
  • Weight loss
  • Fatigue
  • Chronic sinusitis

Causes of Bronchiectasis

Bronchiectasis can be congenital (present from birth) or acquired (develop as the result of a cause such as an infection). The most common causes of bronchiectasis are:

  • Infections: Infections that could lead to bronchiectasis include viral infections (such as measles or influenza), bacterial infections (such as staphylococcus aureus), mycobacterial infections (such as tuberculosis), and fungal infections (such as histoplasmosis).
  • Immune Diseases: Patients suffering from immune deficiencies are more likely to experience recurrent lung infections, which can cause airway damage that leads to bronchiectasis.
  • Aspiration: Inhalation of materials from the mouth or stomach into the lungs can inflame airways, leading to bronchiectasis. Aspiration may be caused by conditions such as impaired swallowing, which may allow food or saliva into the lungs, or gastroesophageal reflux disease (GERD), which happens when the valve between the esophagus and stomach malfunctions, allowing stomach contents to flow up into the esophagus and enter the lungs.
  • Autoimmune Diseases: Rheumatologic, autoimmune, or connective tissue conditions including rheumatoid arthritis or lupus could cause bronchiectasis.
  • Genetic Diseases: Conditions such as cystic fibrosis, primary ciliary dyskinesia, and alpha1 antitrypsin deficiency can cause recurrent lung infections that lead to bronchiectasis.
  • Airway Obstructions: Obstructions resulting from a growth like a tumor or a disease such as chronic obstructive pulmonary disease (COPD) could trap mucus and infections causing airway damage the leads to bronchiectasis.

Screening & Diagnosis

In order to evaluate a patient for bronchiectasis, your doctor will perform a thorough physical examination and take a comprehensive medical history. Tests for bronchiectasis often include the following:

  • Chest CT scan of the lungs
  • Pulmonary function tests, which are breathing tests that determine the presence and severity of abnormal airflow from the lungs
  • Specific diagnostic or screening tests for the possible underlying conditions that could cause bronchiectasis

Following diagnosis, your doctor will design an individualized treatment plan to meet your needs.

What is NTM?

Nontuberculous mycobacteria (NTM) are types of germs found in water and soil. While they do not usually make people sick,  NTM can cause infections in some patients, particularly those with an underlying illness or compromised lung function, bronchiectasis.

The NTM infection can become chronic, with a severe case requiring ongoing treatment, but NTM is not contagious.

Symptoms of NTM

The symptoms of an NTM infection may develop gradually and include the following:

  • Fever
  • Cough (which may include blood)
  • Loss of appetite and weight loss
  • Night sweats
  • Fatigue

Diagnosis of NTM

In order to diagnose a nontuberculous mycobacteria (NTM) infection, your healthcare provider will perform a thorough physical examination and take your medical history. Tests for NTM often include the following:

  • CT scan of the chest
  • Lab tests, such as a sputum culture
  • Bronchoscopy

If you receive a diagnosis of NTM, your doctor will develop an individualized treatment plan for you to meet your needs.

Bronchiectasis – Physiopedia

Original Editor – Kate Wright Top ContributorsKim Jackson, Jordan Tremblay, Remy Valiaveettil, Admin, Kate Wright, WikiSysop, Vidya Acharya, Lucinda hampton, Laura Ritchie, Evan Thomas, Michelle Lee and Karen Wilson

Bronchiectasis is an obstructive lung disease that results from the presence of chronic inflammatory secretions and microbes leading to the permanent dilation and distortion of airway walls, as well as recurrent infection [1]. It is associated with frequent acute exacerbations, which are an independent predictor of progressive decline in respiratory function and a poorer prognosis[2]

Mechanism of Injury / Pathological Process[edit | edit source]

Bronchiectasis is chronic irreversible dilation of the bronchi on the lungs. It follows a severe lung infection or aspiration. It is more common in conditions such as Cystic Fibrosis, Rheumatoid Arthritis, Immunodeficiency, Young’s syndrome and Allergic Bronchopulmonary Aspergillosis, and after childhood diseases such as whooping cough, TB and measles. Bronchiectasis that is not associated with Cystic Fibrosis is known as non-CF Brochiectasis[2]

[3]

Clinically Relevant Anatomy[edit | edit source]

Bronchiectasis involves inflammation of the airway walls, specifically the bronchial walls [4]. The airway walls are the ‘tubes’ that run from the mouth and nose and travel to the lung. The main area that is affected in bronchiectasis is the bronchi [4]. The trachea bifurcates into the right and left main bronchi, which then further divide into secondary bronchi; one for each lobe of the lung [5]. They then divide into tertiary bronchi; one for each bronchopulmonary segment [5]. For more detailed anatomy see Lung Anatomy

The cilia are also damaged in bronchiectasis. Cilia line the airway and are attached to the epithelium. They are hair-like with tiny hooks on the tip to grab the mucous and help move the mucus up to the throat [4].

It is underestimated in prevalence, incidence, and morbidity because symptoms are often ascribed to smoking. A study by Hill [6]found that one in 1000 people in the UK have bronchiectasis, with associated deaths increasing at a rate of approximately 3% per year in England and Wales [7]. Though the overall mortality rate is increasing in these countries, rates in older groups are rising whereas rates in younger groups are falling  [7]. A disproportionate amount of cases found in developing countries and in Aboriginal populations in affluent communities [8]. In some populations, an improvement in socio-economic status, housing, and education can improve overall health and reduce the incidence of respiratory infection and development of bronchiectasis [8].

The cause is unknown for 50% of cases, but it has been linked to inflammatory bowel disease, rheumatoid arthritis, and in 29-50% of patients, COPD [9]. There are several associated conditions with bronchiectasis including; cystic fibrosis, primary ciliary dyskinesia, lung tuberculosis, allergic bronchopulmonary aspergillosis, rheumatoid diseases, symptomatic lupus erythematosus, immune deficiency, severe childhood respiratory infection and exposure to a foreign body or corrosive substance [4]. It has also been linked to an x1-antitrypsin deficiency [4]. Bronchiectasis tends to predispose patients to acquired and congenital lung infections [4].

The process begins with inflammatory damage to the bronchial walls, which then stimulates the formation of excess thick mucus [4]. The warm and moist environment of the lungs combines with the mucus to cause further inflammation and obstruction, creating an excellent environment for infection [4]. The thick mucus crushes the cilia and causes further damage. The immune response releases toxic inflammatory chemicals (i.e. neutrophils), as well as leads to fibrosis and bronchospasm if persistent[4].

Currently, a high-resolution CT scan is the gold standard for diagnosing bronchiectasis [4]. Parallel tramlines and ring shadows may be present on the scan, indicating thickened airway walls and dilated airways, respectively[4]. There may also be ‘glove finger shadows’, which are finger-like projections that represent the dilated bronchi filled with solidified secretions[4].
Spirometry will simply identify if there is an airway obstruction, but this can be indicative of many other diseases[4].
Microbiology can also be used to identify the bacteria present during an exacerbation [4]. A sputum sample is sent off and the results are returned with the incidence of bacterial colonization measured [4].

As delicate cilia are often damaged due to the presence of thick mucus, the voluminous quantities of sputum formed cannot be efficiently cleared[4]. These secretions will lead to the production of wheezes, coarse crackles and squeaks on auscultation. Often times, patients will also present with muscular dysfunction due to inflammation, gas exchange abnormalities, inactivity, malnutrition, hypoxia and medications [10]. Dyspnoea on exertion seems to be a major clinical manifestation as it is experienced by three out of four patients. Other physical manifestations include chest pain, snoring, finger clubbing, fatigue, persistent cough, recurrent infection, and loss of appetite[4]. Stress incontinence can also be a result of excess coughing. Bronchiectasis tends to also have psychological manifestations, such as anxiety, depression, reduced confidence, altered relationships and time off work[4].
Exacerbations can occur several times a year and are identified by four or more of the following signs and/or symptoms: change in sputum, increased dyspnoea, increased cough, fever of greater than 38°, increased wheeze, decreased exercise tolerance, fatigue, lethargy, and radiographic signs of a new infection[4].

Physiotherapy and Other Management[edit | edit source]

The key to the management of bronchiectasis is through education and systemic management. Currently, antibiotics are used at the first sign of change in sputum colour[4] and longterm use of antibiotics is recommended for people who experience 3 or more exacerbations a year[11]. Since antibiotics do not help with the persistent inflammation in the airways, inhaled steroids are taken as an anti-inflammatory and to decrease sputum production[4]. Brochodilators tend to help patients who also have co-existing COPD and asthma[11]. A review and a controlled study found that many people with bronchiectasis also have a Vitamin D deficiency and prescribing Vitamin D may have an anti-inflammatory and anti-infective role[12][13].

Physiotherapy has a very valuable role in aiding with symptoms of bronchiectasis. Since mucociliary clearance is reduced to about 15% of normal, patients tend to cough more[4]. Physiotherapy treatments are aimed at aiding secretion clearance, managing fatigue induced by the effort of ineffective clearance and increased coughing. The most common and effective treatments are:

Active Cycle of Breathing Technique[edit | edit source]

(ACBT) is a commonly taught technique and is often used with Postural drainage and manual drainage[14]. Its purpose is to loosen and clear excess pulmonary secretions, improve the effectiveness of a cough and to improve lung ventilation and function. It consists of 3 main stages:

  • Breathing Control
  • Deep Breathing Exercises or Thoracic Expansion Exercises
  • Huffing or Forced Expiratory Technique (FET)[15]
  • Forced expiration technique, sometimes referred to as a huff. It is part of the ACBT but can be used alone. A huff is very effective at clearing secretions especially when combined with other airway clearance techniques.[16]
  • Manual Therapy is a popular treatment technique and is often used when the patient is fatigue or experiencing an exacerbation of symptoms. It describes techniques that involve external forces to the chest wall to loosen mucus and includes any combination of percussion, shaking, rib springing, vibrations and over pressure[17]. Because of the nature of the technique, it is contraindicated in patients that are taking anticoagulants or that have osteoporosis[18]. The aim of treatment is to:
    • Loosen secretions
    • Reduce fatigue
    • Increase the effectiveness of other treatment techniques
  • Postural Drainage (or modified postural drainage) is an effective treatment that incorporates gravity-assisted techniques to help clear secretions from specific segments of the lungs, and often requires tilting the head down to clear secretions from the middle and lower lobes. Parkinson’s is commonly used in conjunction with other techniques like ACBT, percussion, coughing or huffing and studies have found using Parkinson’s with these techniques produced a greater amount of sputum[19][20]. Although Parkinson’s is effective in secretion clearance it is time-consuming and often found to be less tolerated than techniques that are performed in sitting[20]. Where tilting the head down is contraindicated or not tolerated then modified postural drainage (mPD) positions can be used instead and in cases where gastroesophageal reflux disease GORD) is present and exacerbated even when using modified Parkinson’s then it is suggested to use a technique that can be performed in sitting.
  • Autogenic Drainage is a technique that utilises breathing control to clear secretions from the airways. The aim is to vary the depth, rate and location of lung volumes during respiration to move secretions from the smaller airways to the larger airways for easier expectoration. It consists of three phases:
    1. Mobilising (unstick) phase – involves breathing as much air out of the lungs as possible and resisting the urge to cough. During this phase, crackles may be heard.
    2. Collecting phase – as the secretions get louder the rate and depth of the breaths change, the speed of breathing out is faster (but not too fast to stimulate a cough) and are felt more in the middle of the chest. This assists the movement of secretions from the smaller airways to the larger airways.
    3. Clearing phase – as the secretions get louder the aim is now to take full, slow deep breaths in, followed by a fast breath out. Suppress the urge to cough and after three deep breaths huff to expel the secretions. Often the huff is enough to clear secretions but if they are ineffective this is the stage where a cough may be effective in clearing secretions.
  • Positive Expiratory Pressure (PEP) is a technique that describes breathing against resistance and can be performed either through a device or against pursed lips. It is a technique which is recommended when other techniques have not been effective or are contraindicated; it has been found to have no adverse effect on GORD. [21] The increase in pressure creates back pressure in the airways during expiration. This may cause a build-up of gas behind the mucus, temporarily increasing functional capacity, and improving mucus clearance.[22]

Studies suggest better results with a combination of Bubble PEP Device and ACBT in sputum clearance with these patients[23].

High-Frequency Chest Wall Oscillation is achieved by wearing a vest that emulates chest physiotherapy. The vest applies positive pressure air pulses to the chest which causes vibrations that loosen and thin mucus, This along with an intermittent cough or huff assists with the clearance of secretions. This device allows people to perform therapy in their own time, allowing them some control and less dependence on other people.

Intrapulmonary Percussive Ventilation is another technique that relies on a device to assist with the clearance of secretions. An intrapulmonary percussive ventilator (IPV) is a machine that delivers short bursts of air through a mouthpiece that vibrates the airway walls[24]. It is indicated for short-term use when other techniques are contraindicated or have proved ineffective.

Intermittent Positive Pressure Breathing (IPPB), is an expensive piece of equipment[25] and usually only used when all other clearance techniques have proved to be ineffective[11]. It is commonly seen in Intensive Care and gives positive pressure as the patient breathes in, and during expiration it creates negative pressure making the cough stronger and more effective

Physical Exercise is recommended for respiratory conditions, including bronchiectasis with the aim of improving aerobic capacity and, fitness and endurance. A study by Lee et al concluded that exercise resulted in short term improvements and had an impact on dyspnoea and fatigue, and resulted in fewer exacerbations over a 12 month period[26].

In order to assess the effectiveness of treatment and changes in quality of life, there are several outcome measures available:

  • St. George’s Respiratory Questionnaire (SGRQ) [4]. The SGQ is a commonly used questionnaire to measures health-related quality of life in patients with respiratory conditions [27].
  • The Quality of Life Questionnaire-Bronchiectasis (QOL-B)[28]
  • Leicester Cough Questionnaire[29]
  • Chronic Respiratory Disease Questionnaire (CRDQ)[30]
  1. ↑ Barker AF. Bronchiectasis. New England Journal of Medicine 2002; 346: 1383-93.
  2. 2.02.1 Martinez-Garcia M, Soler-Cataluna J, Perpina-Tordera M, Roman-Sanchez P, Soriano J: Factors associated with lung function decline in adult patients with stable non-cystic fibrosis bronchiectasis. Chest 2007, 132:1565–1572
  3. ↑ salamhossein. Bronchiectasis Animation – What is Bronchiectasis? Video.mp4. Available from: https://www.youtube.com/watch?v=uNeprw1rsgE [last accessed 18/5/15]
  4. 4.004.014.024. 034.044.054.064.074.084.094.104.114.124.134.144.154.164.174.184.194.204.214.22 Hough A. Physiotherapy in Respiratory and Cardiac Care: an evidence-based approach. 4th ed. Hampshire: Cengage Learning EMEA, 2014.
  5. 5.05.1 Palastanga N, Soames R. Anatomy and human movement: structure and function. 6th ed. New York: Churchill Livingstone; 2012.
  6. ↑ Hill AT, Welham S, Reid K, Bucknall CE. British Thoracic Society national bronchiectasis audit 2010 and 2011. Thorax 2012; 1:1-3.
  7. 7.07.1 Roberts HJ, Hubbard R. Trends in bronchiectasis mortality in England and Wales. Respiratory Medicine 2010; 104: 981-5.
  8. 8.08.1 Chang AB, Marsh RL, Vaughan-Smith HC, Hoffman LR. Emerging drugs for bronchiectasis. Informa healthcare 2012; 17: 361-378.
  9. ↑ Goeminne P, Dupont L. Non-cystic fibrosis bronchiectasis: Diagnosis and management in the 21st century. Postgrad Med J 2010; 86: 493-501.
  10. ↑ Ozalp O, Inal-Ince D, Calik E, Vardar-Yagli N, Saglam M, Savci S, et al. Extrapulmonary features of bronchiectasis: muscle function, exercise capacity, fatigue, and health status. Multidisciplinary Respiratory Medicine 2012; 7: 1-6.
  11. 11.011.111.2 Hill AT, Sullivan AL, Chalmers JD, De Soyza A, Elborn JS, Floto RA, Grillo L, Gruffydd-Jones K, Harvey A, Haworth CS, Hiscocks E. British Thoracic Society Guideline for bronchiectasis in adults. Thorax. 2019 Jan 1;74(Suppl 1):1-69.
  12. ↑ Bartley J, Garrett J, Grant CC, Camargo CA. Could vitamin D have a potential anti-inflammatory and anti-infective role in bronchiectasis?. Current infectious disease reports. 2013 Apr 1;15(2):148-57.
  13. ↑ Chalmers JD, McHugh BJ, Docherty C, Govan JR, Hill AT. Vitamin-D deficiency is associated with chronic bacterial colonisation and disease severity in bronchiectasis. Thorax. 2013 Jan 1;68(1):39-47.
  14. ↑ O’Neill B, Bradley JM, McArdle N, et al. The current physiotherapy management of patients with bronchiectasis: a UK survey. Int J Clin Pract 2002;56:34–5.
  15. ↑ Larner E, Galey P. Active cycle of breathing technique. Available from: http://www.nnuh.nhs.uk/publication/download/active-cycle-of-breathing-technique-v3 (accessed 24 March 2019)
  16. ↑ Van der Schans CP. Forced expiratory manoeuvres to increase transport of bronchial mucus: a mechanistic approach. Monaldi archives for chest disease= Archivio Monaldi per le malattie del torace. 1997 Aug;52(4):367.
  17. ↑ Syed N, Maiya AG, Siva Kumar T. Active Cycles of Breathing Technique (ACBT) versus conventional chest physical therapy on airway clearance in bronchiectasis–a crossover trial. Advances in Physiotherapy. 2009 Jan 1;11(4):193-8.
  18. ↑ Diehl N, Johnson MM. Prevalence of Osteopenia and Osteoporosis in Patients with Noncystic Fibrosis Bronchiectasis. South Med J. 2016;109(12):779-83
  19. ↑ Ramos EM, Ramos D, Moreira GL, Macchione M, Guimarães ET, Rodrigues FM, de Souza AA, Saldiva PH, Jardim JR. Viscoelastic properties of bronchial mucus after respiratory physiotherapy in subjects with bronchiectasis. Respiratory care. 2015 May 1;60(5):724-30.
  20. 20.020.1 Flude LJ, Agent P, Bilton D. Chest physiotherapy techniques in bronchiectasis. Clinics in chest medicine. 2012 Jun 1;33(2):351-61.
  21. ↑ Lee AL, Denehy L, Wilson JW, et al. Upright positive expiratory pressure therapy and exercise: effects on gastroesophageal reflux in COPD and bronchiectasis. Respir Care 2012;57:1460–7.
  22. ↑ McIlwaine M, Button B, Dwan K. Positive expiratory pressure physiotherapy for airway clearance in people with cystic fibrosis. Cochrane Database of Systematic Reviews. 2015(6).
  23. ↑ Santos MD, Milross MA, McKenzie DK, Alison JA. Bubble‐positive expiratory pressure device and sputum clearance in bronchiectasis: A randomised cross‐over study. Physiotherapy Research International. 2020 Feb 29:e1836.
  24. ↑ Paneroni M, Clini E, Simonelli C, et al. Safety and efficacy of short-term intrapulmonary percussive ventilation in patients with bronchiectasis. Respir Care 2011;56:984–8.
  25. ↑ Eggertsen SC. Intermittent positive pressure breathing and the treatment of acute asthma. The Journal of family practice. 1983 May;16(5):909-13.
  26. ↑ Lee AL, Hill CJ, Cecins N, et al. The short and long term effects of exercise training in non-cystic fibrosis bronchiectasis–a randomised controlled trial. Respir Res

    2014;15:44.

  27. ↑ Ferrer M, Miravitlles M, Villasante C, Alonso J, Sobradillo V, Gabriel R, et al. Interpretation of quality of life scores from the St George’s Respiratory Questionnaire. Eur Respir J 2002;19(3):405-13.
  28. ↑ Quittner AL, O’Donnell AE, Salathe MA, Lewis SA, Li X, Montgomery AB, O’Riordan TG, Barker AF. Quality of Life Questionnaire-Bronchiectasis: final psychometric analyses and determination of minimal important difference scores. Thorax. 2015 Jan 1;70(1):12-20.
  29. ↑ Birring SS, Prudon B, Carr AJ, Singh SJ, Morgan MD, Pavord ID. Development of a symptom specific health status measure for patients with chronic cough: Leicester Cough Questionnaire (LCQ). Thorax. 2003 Apr 1;58(4):339-43.
  30. ↑ Vodanovich DA, Bicknell TJ, Holland AE, Hill CJ, Cecins N, Jenkins S, McDonald CF, Burge AT, Thompson P, Stirling RG, Lee AL. Validity and reliability of the chronic respiratory disease questionnaire in elderly individuals with mild to moderate non-cystic fibrosis bronchiectasis. Respiration. 2015;90(2):89-96.

Bronchiectasis – a guide for primary care

Graeme Maguire

Background

While bronchiectasis not related to cystic fibrosis remains a significant cause of chronic respiratory disease in low to middle income countries, it has a lower profile in Australia. Nonetheless, there is increasing recognition that people living in Australia can present for the first time with noncystic fibrosis bronchiectasis at all stages of life. In addition, clinicians are often faced with the conundrum of minor changes consistent with bronchiectasis incidentally reported on computed tomography scan.

Objective/s

This article aims to provide advice regarding when to suspect bronchiectasis, how to proceed with confirming or refuting a diagnosis, and the principles of management to minimise disease progression and manage the acute exacerbations, symptoms and associated disability and impaired quality of life.

Discussion

Delay in the diagnosis, investigation and management of bronchiectasis in both children and adults is common, and this delay has been shown to be associated with more rapid progression of disease. General practitioners have a key role in suspecting and accurately diagnosing and assessing bronchiectasis, discussing potential cases with specialist respiratory colleagues early and leading a multidisciplinary team to help patients with bronchiectasis manage their disease and minimise disability and premature death.

Case study

Jane, 67 years of age, lives in Sydney. You have known her for years and she is a lifelong nonsmoker. Over the past 4 years, each time you see her to review her hypertension, she is coughing delicately into her handkerchief and finding it difficult to talk in full sentences after walking from the waiting room to your office.

General practitioners are frequently faced with a broad range of differential diagnoses when a patient presents with chronic shortness of breath or chronic respiratory symptoms such as cough, sputum or wheeze. Before considering a diagnosis of lung disease, other diagnoses including heart failure and anaemia, and simple poor fitness, need to be considered. Even when the focus is more likely lung disease, there remain a number of conditions including chronic obstructive pulmonary disease (COPD), asthma, interstitial lung disease and pulmonary hypertension that need to be considered. Bronchiectasis should be included in this broad list of differentials.

While clinicians may automatically think of children and young adults with cystic fibrosis (CF) when bronchiectasis is mentioned, it is now recognised that there are an increasing number of patients who are diagnosed with non-CF bronchiectasis when they reach adulthood. Delay in the diagnosis, investigation and management of bronchiectasis is common and this delay has been shown to be associated with more rapid progression of disease.1,2

This article will focus on non-CF bronchiectasis and aims to provide advice regarding in whom to suspect bronchiectasis and how to proceed with confirming or refuting a diagnosis. It also discusses the principles of management to minimise disease progression and the managment acute exacerbations, symptoms, and the associated disability and impaired quality of life.

However, before addressing these areas it is first important to outline the concepts that underlie the pathology, pathophysiology and diagnosis of bronchiectasis.

What is bronchiectasis?

Bronchiectasis is classically defined as a pathological diagnosis typically confirmed by radiology. Bronchiectasis is characterised by abnormal, irreversible bronchial dilatation or a fixed increase in airway diameter. Bronchiectasis is currently usually diagnosed by a chest high-resolution computed tomography (c-HRCT) scan (Figure 1).

Figure 1. Changes of bronchiectasis on c-HRCT can be subtle. A) Dilatation and loss of normal tapering of right middle lobe bronchi (arrows) or obvious; B) Bilateral saccular dilatation of bronchi with associated collapse and parenchymal destruction

While the primary site of damage detected by c-HRCT is the larger airways, this is likely to be a later or parallel manifestation of a disease process involving other components of the lung, including the smaller airways not well visualised by c-HRCT and the bronchial mucosa. Particularly in the earlier stages of disease, chronic airway infection and inflammation consistent with bronchiectasis may not be accompanied by airway dilatation on c-HRCT, as seen in children who are at an elevated risk of developing later radiologically confirmed bronchiectasis. Chronic suppurative lung disease (CSLD) is an all-encompassing term, used particularly in children, to define a clinical syndrome of chronic airway inflammation and suppuration with or without evidence of bronchiectasis on c-HRCT.

Given that bronchiectasis is a pathologic diagnosis it is possible to have evidence of bronchiectasis on c-HRCT without symptoms of chronic airway inflammation and suppuration. This may be related to relatively quiescent or minor disease or the fact that the cause of the airway dilatation is not related to a process affecting the airway directly, but rather a process involving the lung parenchymal with secondary fibrosis and retraction of the structures supporting the airway. This entity, typically seen in interstitial lung diseases and termed, ‘traction bronchiectasis’, is not usually considered as bronchiectasis per se unless there is evidence of associated airway suppuration and its management it typically directed at the underlying interstitial lung disease.

Burden of disease

Bronchiectasis can be caused by a broad range of disparate and esoteric conditions and is often idiopathic in nature. Although increasingly recognised, there is a lack of data regarding the burden of non-CF bronchiectasis in Australia. Aboriginal people living in remote Australia are at particular risk. While a study3 of Central Australia Aboriginal children found a prevalence of 1470/100 000, the estimated prevalence for Australians overall remains unknown. In the United States, estimates range from 4.2/100 000 in the 18–34 years age group to 272/100 000 in those over 75 years of age.4

The impact on the healthcare system also appears to be increasing. In the US, hospitalisations for bronchiectasis have increased between 1993 and 2006 and now occur at an annual rate of 16.5/100 000.5

In light of the limited understanding of the prevalence of bronchiectasis in Australia, it is impossible to assess the impact this has on disability and premature mortality at a national level.

A national bronchiectasis register being developed by the Australian Lung Foundation should facilitate more accurate estimates of Australian disease burden.

Pathophysiology

While the underlying cause of bronchiectasis may vary (or is even often undefined or idiopathic) it is ultimately due to injury to the airways, which is typically associated with chronic and recurrent inflammation because of an abnormality of airway anatomy, immunity or function. Despite extensive investigation, more than 80% of patients with bronchiectasis will have no clearly identified cause for their disease.6 Nonetheless, it is important to consider secondary causes as these may be associated with reduced progression if managed, can be associated with other nonrespiratory complications, or can have a familial association. The range of possible causes of bronchiectasis is listed in Table 1.7












Table 1. Aetiologies and factors associated with bronchiectasis7

  • Congenital causes (eg. Mounier-Kuhn syndrome, Young syndrome)

  • Chronic obstructive pulmonary disease and smoking

  • Mucociliary dysfunction (eg. primary ciliary dyskinesia)

  • Primary or secondary immune deficiency (eg. hypogammaglobulinaemia, lung and bone marrow transplantation, malignancy, HIV/AIDS, HTLV1)

  • Pulmonary fibrosis and pneumoconiosis (eg. silicosis)

  • Postobstruction (eg. with a foreign body)

  • Postinfection (eg. tuberculosis, adenovirus, recurrent pneumonia)

  • Recurrent small volume aspiration (eg. from upper airway secretions or gastric contents)

  • Allergic bronchopulmonary aspergillosis

  • Systemic inflammatory diseases (eg. rheumatoid arthritis, sarcoidosis)

Overlap syndromes

Bronchiectasis can frequently occur in parallel with more common forms of chronic lung disease including COPD and asthma. The increasing availability and use of c-HRCT has shown that up to 50% of patients with severe COPD will have co-existent bronchiectasis.8 In an Australian cohort of adult bronchiectasis patients, 15% had a co-existent diagnosis of asthma or COPD and 25% demonstrated significant (>15%) improvement with bronchodilators, which is suggestive of airway hyperactivity if not asthma.6 Bronchiectasis is more likely to co-exist with COPD in patients with moderate to severe disease, an acute exacerbation requiring hospitalisation in the past year, and if sputum contains potential pathogens even when clinically stable.9 There nonetheless remains debate whether this is indeed ‘true’ bronchiectasis or coincidental airway dilatation associated with advancing age or associated hypoxaemia. 10,11

When to suspect bronchiectasis

Given the broad range of conditions that can cause bronchiectasis, the fact many patients can have idiopathic disease, the presence of mild disease and overlap syndromes, it is not surprising that it is difficult to identify which patients warrant further investigation.

The first group in whom to suspect bronchiectasis are those with a confirmed diagnosis of a condition that may predispose to disease, such as a confirmed immune deficiency. Another group that may not immediately spring to mind is men with primary infertility, particularly when related to azospermia or immotile sperm. In these cases, milder forms of CF, Young syndrome or primary ciliary dyskinesia/immotile cilia syndrome should be considered.

More difficult and more common is the patient presenting with chronic respiratory symptoms, especially cough and sputum production. While there is no absolutely accurate way to differentiate between chronic bronchitis, COPD and asthma and bronchiectasis (and indeed as noted all these conditions can co-exist with bronchiectasis) a number of clinical features may help and these are listed in Table 2.

  • Diagnosis of asthma that is unresponsive to usual management







Table 2. Features that may suggest bronchiectasis in a patient presenting with chronic respiratory symptoms

  • Digital clubbing (this is rare in COPD and asthma)

  • Lack of a significant smoking history (less than an average of 20 cigarettes per day for 10 years) in a person with suspected COPD

  • History of recurrent and/or severe pneumonia including tuberculosis

  • Presence of ‘unusual organisms’ in sputum (eg. Aspergillus, atypical/nontuberculous mycobacteria, Pseudomonas aeruginosa, Escherichia coli, Klebsiella pneumoniae)

  • Childhood associated with significant environmental and social disadvantage*
* This includes Aboriginal and Torres Strait Islander people, as well as people who have immigrated from low income countries. 52 In this group of people, tuberculosis as the cause of chronic respiratory symptoms should also be considered

Case study continued

You examine Jane and she has bibasal crackles, occasional wheeze, an oxygen saturation of 95% and no digital clubbing. You perform spirometry. It is of good quality and reproducibility and demonstrates moderate airflow obstruction that is not fully reversible with salbutamol. A plain chest X-ray is reported as demonstrating minor right basal fibrotic changes. Given the presence of wheeze and airflow obstruction you suspect a combination of asthma with an element of COPD. You commence Jane on a short acting beta-agonist and combination long acting beta-agonist/inhaled corticosteroid inhaler. On review 1 month later her condition has not improved.

Diagnosis and assessment

In general, a diagnosis of bronchiectasis requires a clinically consistent history in association with evidence of fixed and abnormal bronchial dilatation. As highlighted earlier, evidence of bronchial dilatation is typically confirmed on c-HRCT (Figure 1).

Plain chest X-ray

While plain chest X-ray may demonstrate large airway dilatation it is not sensitive (ie. a normal chest X-ray does not exclude bronchiectasis) nor does it provide an accurate measure of the extent of disease or any associated interstitial damage (eg. pulmonary fibrosis).

Chest high-resolution CT scan

Chest HRCT allows an assessment of a number of features of airway anatomy including diameter, wall thickness and the presence or absence of normal tapering. Despite its benefits, c-HRCT has inherent limitations, such as the inability to confirm fixed airway dilatation on a single study. In patients with concurrent infection, c-HRCT may demonstrate temporary airway dilatation that may resolve on subsequent studies. To avoid the need for repeated c-HRCT it is recommended diagnostic c-HRCT be performed when a patient is clinically stable and not in the setting of an acute exacerbation.

The definition of what is an abnormally dilated airway can provide further room for confusion. In general, this is said to be present in adults when the internal diameter of the airway on c-HRCT is greater than that of the accompanying branch of the pulmonary artery (termed a ‘bronchoarterial ratio greater than one’).12,13 While this is an important and accepted definition of airway dilatation used in reporting c-HRCT, it is also apparent this ratio increases with age11 and other factors including altitude and co-existent hypoxia.10 Too strict adherence to this ratio may produce poorer diagnostic sensitivity for c-HRCT in younger people and lower specificity in older adults. In general, if the results of c-HRCT are discordant with expectations or the changes are incidentally noted in a patient without clinical features of bronchiectasis, then further discussion with the reporting radiologist and a respiratory physician is warranted.

While the adage ‘manage the patient not the X-ray’ should be applied to patients without symptoms and only minor and incidentally detected changes of bronchiectasis on c-HRCT, it should be noted that bronchiectasis is often a progressive condition where minor changes can potentially be a harbinger of the development of later more extensive disease. Careful clinical review, searching for secondary causes if any chronic respiratory symptoms are present, and ongoing clinical follow up is warranted. In otherwise well patients with normal spirometry, a case may be made for follow up c-HRCT after 2 years to confirm a lack of radiologic progression.

The final issue that should be considered before ordering a c-HRCT is radiation. A c-HRCT has an effective radiation dose of up to 8 mSv, the equivalent of 400 plain chest X-rays or 3.6 years of background radiation.14,15 This is of particular importance for children and younger adults where the effective radiation dose and the lifetime implications associated with cancer induction are higher. Before ordering a c-HRCT in children and younger adults, specialist input is encouraged to ensure the potential risk of the radiation exposure is balanced by any possible benefit.16

Spirometry

Unlike conditions such as COPD, where the demonstration of airflow obstruction is an essential component of the diagnosis, in bronchiectasis, spirometry findings can vary and even be normal in the early stages of disease. Bronchiectasis is not always associated with evidence of airflow obstruction – as demonstrated by a reduced ratio of the volume expired in the first second (FEV1) compared with the total expired volume (FVC) during a forced expiration from total lung capacity  on spirometry. A restrictive (reduction in both FEV1 and FVC with normal or elevated FEV1/FVC ratio) or mixed obstructive/restrictive pattern may often seen. While spirometry and more detailed lung function testing is useful in assessing severity of disease, monitoring progression and predicting prognosis (particularly in adults) it cannot be used to exclude or confirm a diagnosis of bronchiectasis.

Although there is no agreed definition for stratifying the severity of bronchiectasis on spirometry alone, it is reasonable to base this, in part, on the severity of impairment in FEV1 used in the Australian COPD guidelines17 as outlined in Table 3.





Table 3. Spirometry based stratification of bronchiectasis severity based on the COPDX Plan17
Severity % predicted FEV1*
Mild 60–80%
Moderate 40–59%
Severe <40%
* FEV1 expiratory volume in the first second of a forced expiratory manoeuver

Investigating for secondary causes

The broad range of secondary causes of bronchiectasis has been outlined in Table 1.7 The minimum recommended investigations for these is listed in Table 4.7 While all patients with a confirmed diagnosis of bronchiectasis warrant exclusion of secondary and preventable causes, many of these investigations require specialist support. The investigations that might be performed before specialist referral to expedite management are highlighted in bold.















Table 4. Recommended investigations for secondary causes of bronchiectasis7

  • Immunoglobulin classes IgG, IgA, IgM, and IgG subclasses

  • Sputum culture including mycobacterial culture

  • Serological tests for Aspergillus and total IgE level in adults, especially if there is a history of wheeze/asthma

  • Test for primary ciliary dyskinesia in children
In addition, consider the following:

  • Test for cystic fibrosis transmembrane conductance regulator gene mutations

  • Bronchoscopy for foreign body or airway abnormality and to obtain specimens for culture of respiratory pathogens, including mycobacteria

  • Additional immunological tests – total IgE level in children, neutrophil function tests and lymphocyte subsets, and antibody responses to protein and polysaccharide antigens

  • Test for primary ciliary dyskinesia in adults
Note: suggested investigations before specialist referral are highlighted in bold

Case study continued

Given Jane’s condition has not improved you call your local respiratory physician. She suggests full blood examination (FBE), immunoglobulin levels, aspergillus serology and total IgE, one sputum for usual microbiology and three sputums for atypical mycobacterial microbiology, and a c-HRCT. She arranges an appointment to see Jane in 6 weeks with the results.

Management of bronchiectasis in primary care

The complexities associated with suspecting and confirming a diagnosis of bronchiectasis and assessing for secondary causes have already been highlighted. Even once this is achieved, the ongoing monitoring and management of bronchiectasis includes a plethora of treatment options. This section outlines available therapies and provides advice as to when they should be considered.

The principles of ongoing management of bronchiectasis are based on the monitoring of severity, reducing progression and complications, early treatment of acute exacerbations, minimising disability, considering transplantation in appropriate patients, managing comorbidities and early utilisation of palliative care services when necessary. As with any complex chronic disease, patients benefit from a multidisciplinary care approach. In the case of bronchiectasis, this may comprise input from a respiratory physician, a physiotherapist, a palliative care and mental health team and respiratory nurse.

When to refer

In general, all patients should be discussed or referred to a specialist paediatric or adult respiratory physician for initial assessment and advice regarding the development of an individualised management plan. In the case of children and young adults this should occur when the diagnosis is suspected and before organising a c-HRCT. While a specialist review may be difficult for some patients, a focused telephone discussion or telehealth consultation can often suffice.

The role of antibiotics

While bacteria and fungi are often found in the sputum of people with bronchiectasis, their role in disease development, acute exacerbations and progression is variable. In some cases they may be contributors to airway inflammation, damage and the progression of bronchiectasis (the ‘vicious cycle’ hypothesis).18 However, such organisms may also be commensals, colonising damaged airways but not contributing to acute exacerbations or progressive airway damage. Decisions regarding the use of antimicrobials must therefore be based on an individual patient’s response in addition to the results of airway microbiology.

Acute exacerbations

While there is little evidence to support the use of antibiotics in acute exacerbations of bronchiectasis, there is general consensus that they should be used.19 An acute exacerbation can be defined as two or more of: increasing cough, shortness of breath, increasing volume/purulence of sputum. An unexplained significant (>10%) reduction in FEV1 or FVC over days or weeks should also raise suspicion of an acute exacerbation. In a patient who is not unwell or at risk of sudden deterioration (Table 5), initial oral antibiotic therapy is reasonable. The choice of antibiotic should be based on the most recent sputum culture. If this result is negative or not available, commencing treatment with amoxicillin-clavulanate or doxycycline is recommended.7 The course of therapy should be prolonged (at least 10 days). Early follow up (within 4 days) and regular review is required to ensure response and to consider inpatient management early if there is deterioration. While the rate of response varies, most patients would be expected to begin to improve within 7 days, although it can take up to 4 weeks to return to a baseline state.











Table 5. Features of an acute exacerbation of bronchiectasis that should prompt early discussion regarding referral for inpatient management

  • Hypotension (systolic BP <90 mmHg or diastolic BP
    <60 mmHg)

  • Respiratory rate ≥30/minute

  • Previous need for noninvasive ventilation/ICU

  • Failure to improve after 7 days of oral therapy

  • Hypoxia (new onset of oxygen saturation ≤93% on room air)

  • Severe disease (FEV1 <40% predicted)

  • Limited home and social supports or difficulty ensuring follow up/review

  • Substantial disability (new onset of being unable to meet self care needs)

Long term and other antibiotic dosing strategies

There remains debate regarding the role of long term suppressive, intensive intermittent or eradicative antibiotic therapy in non-CF bronchiectasis and whether these can reduce acute exacerbation frequency and or severity, or disease progression, or enhance quality of life or survival. While a systematic review suggested little overall benefit20 from long term suppressive antibiotics, more recent studies have suggested macrolides, including azithromycin, can reduce acute exacerbation frequency and improve lung function.21,22 In patients with frequent exacerbations (three or more per year), a trial of long term antibiotic therapy may be considered. However, the use of antibiotics in this way is outside approved indications, can pose significant cost to patients and is best undertaken in partnership with a specialist respiratory service, which can organise hospital based supply of azithromycin with achievement of clear clinical endpoints before a decision regarding ongoing use. Any potential benefits need to be balanced against the risks associated with antibiotic side effects, drug interactions and the development of microbial resistance.

An alternative approach, particularly utilised in CF related bronchiectasis, has been the regular and intermittent use of intensive antibiotic therapy (typically intravenous) in stable patients.23 Such regular ‘tune ups’, while commonly used in CF, are supported by little evidence and not routinely recommended for non-CF bronchiectasis.

An additional strategy is intensive and prolonged antibiotic treatment with the aim of eradicating Pseudomonas aeruginosa when it is first isolated. This is based on the premise that P. aeruginosa is associated with more difficult and expensive treatment of acute exacerbations in the long term and worse outcome both in CF and non-CF related bronchiectasis. There is evidence in CF that prolonged nebulised, oral and/or intravenous antibiotics can lead to at least short term eradication of both nonmucoid24–27 and mucoid28 strains of P. aeruginosa. In turn, this is associated with a slower decline in lung function.28 A similar strategy of early and aggressive treatment of

P. aeruginosa outside the setting of an acute exacerbation in non-CF bronchiectasis has not been shown to be effective. Nonetheless, despite the lack of evidence, some specialist respiratory bodies recommend attempting eradication.29 In light of this controversy, it is reasonable to seek specialist advice regarding eradicative strategies when P. aeruginosa is initially isolated in patients with non-CF bronchiectasis. Regimens can involve a combination of longer term oral and nebulised antibiotics and can be provided in the community under the supervision of the primary care doctor.

Atypical mycobacterial disease

Mycobacteria other than M. tuberculosis and M. leprae (termed atypical mycobacteria or nontuberculous mycobacteria [NTM]) are generally free living environmental organisms that, unlike

M. tuberculosis, are not associated with person-to-person transmission. The NTM most commonly associated with lung disease in patients without HIV/AIDS is M. avium complex (MAC) with

M. kansasii and M. abscessus being less frequently encountered.30

It is unclear whether other NTM can cause lung disease, in particular bronchiectasis in patients without HIV/AIDS.

While the exact contribution of NTM in the development or progression of bronchiectasis is poorly understood, these organisms (particularly MAC) are increasingly noted in the sputum of people with bronchiectasis and can be associated with progressive lung damage. While NTMs can be isolated in the sputum of people with pre-existing lung disease, they can also be associated with the development of progressive lung disease, typically in older women, without pre-existing disease. This syndrome is characterised by pulmonary infiltrates, nodules and bronchiectasis with a predilection for the lingular segment of the left upper lobe and the right middle lobe.31

Also termed ‘Lady Windermere syndrome’ in reference to Oscar Wilde’s play Lady Windermere’s Fan it is hypothesised that this is caused by suppression of cough in self conscious, overly polite women.32

Differentiating airway colonisation with NTM from active disease requiring treatment is difficult and requires specialist input. It is based on consistent or significant isolation of the organism with typical and progressive radiologic changes.30 If NTM are thought to be driving the development or progression of bronchiectasis, treatment may be considered. Nonetheless, it is prolonged, expensive and complicated, typically involving three-drug therapy for 1–2 years and until the sputum remains negative for NTM for 1 year. Macrolide therapy is an important component of most treatment regimens and the use of prior long term suppressive macrolide therapy (such as azithromycin) may result in the selection of macrolide resistant NTMs with associated less effective and more prolonged and complicated therapy.

Inhalational and other therapies

Inhalational therapy for bronchiectasis can be divided into agents typically used in asthma and COPD, inhalational antibiotics and those used to enhance sputum clearance. Given the existence of overlap syndromes it is not surprising that many patients may benefit from management relating to asthma or COPD. If there is felt to be co-existant asthma (demonstrated by wheeze and a significant >12% and 200 mL increase in FEV1 with bronchodilators) or COPD (emphysema seen on c-HRCT or significant smoking history) then a trial of short and long acting beta agonists, anticholinergics and/or inhaled corticosteroids with assessment of response many be warranted. While there is some evidence that inhaled corticosteroids, with or without associated long acting beta-agonists, may reduce symptoms in non-CF bronchiectasis, this effect is small. Overall there is little evidence to support the routine use of these inhalational therapies, particularly inhaled corticosteroids, in patients without co-existent asthma or COPD.33,34

Inhalational antibiotics have often been used in bronchiectasis. Long term use of nebulised gentamicin for 12 months in patients with non-CF bronchiectasis who have chronic sputum bacterial colonisation and at least two acute exacerbations per year has been shown to reduce sputum bacterial colonisation (which is associated with poorer outcome), improve exercise tolerance and reduce acute exacerbation frequency.35 While a trial of inhaled gentamicin in such patients may be warranted, this should be commenced in consultation with a specialist respiratory service given this is not a licensed use for gentamicin in Australia and may result in the development of initial worsening of lung function in some patients. Inhalational antibiotics are also often an adjunct to oral antibiotics or home based intravenous antibiotics in the primary healthcare based management of acute exacerbations of bronchiectasis and as eradicative or suppressive therapy in selected patients.

Mucolytics and other agents used to enhance sputum clearance that have been considered for non-CF bronchiectasis include recombinant human deoxyribonuclease, bromhexine, n-acetyl cysteine and hyperosmolar agents including hypertonic (6–7%) saline and mannitol. In contrast to CF related bronchiectasis, recombinant human deoxyribonuclease has not shown benefit in non-CF bronchiectasis and indeed may be harmful with greater acute exacerbation frequency and decline in lung function.36 This highlights the fact that interventions shown to be effective in CF related bronchiectasis cannot necessarily be extrapolated to non-CF bronchiectasis.

While mannitol37 and hypertonic saline have shown promise in CF related bronchiectasis, their role in non-CF bronchiectasis is yet to be well demonstrated.38 Hypertonic saline demonstrated short term benefits in non-CF bronchiectasis39 but was not superior to isotonic saline based on quality of life, lung function and acute exacerbation frequency in long term studies.40 Pending the outcome of current trials the use of mucolytics and other agents used to enhance sputum clearance in non-CF bronchiectasis should be limited to those patients in whom sputum expectoration is a major issue and occur in consultation with a specialist respiratory service.

While there is little evidence specifically relating to bronchiectasis and the benefit of pneumococcal and influenza vaccination, the risk of concomitant infection in patients with pre-existing lung disease would support their use in all patients. Children and adults with bronchiectasis should have enhanced influenza and pneumococcal vaccination schedules.41

Physiotherapy and pulmonary rehabilitation

Physiotherapy to enhance sputum clearance has been a longstanding mainstay of management and there is evidence it improves cough, exercise tolerance and, in children, lung function.42,43 Given the risk of silent aspiration, head-down sputum clearance techniques are now discouraged.

There is evidence that pulmonary rehabilitation and tailored exercise programs improve exercise tolerance in people with bronchiectasis.44 Patients with bronchiectasis affecting their exercise tolerance or activities of daily living should be referred for pulmonary rehabilitation and/or have a tailored exercise program developed in consultation with a physiotherapist. This may occur in the community using available local exercise facilities.

Other forms of physiotherapy-based intervention including focused inspiratory muscle training have not shown benefit in bronchiectasis and are not advocated.44,45

Severe disease

Many people with bronchiectasis will have progressive disease1,6 and develop severe bronchiectasis. While all the aspects of management outlined have a clear role in those with severe disease, a range of additional interventions may be appropriate. In turn, it is important that people with bronchiectasis are regularly reviewed to assess the severity of their disease. Such assessment should, as a minimum, include assessment of disability, frequency of acute exacerbations and spirometry. While there is no agreed spirometry based classification for bronchiectasis severity, an FEV1 <40% should prompt enhanced monitoring for complications of severe disease and, if it has not already occurred, discussion with a specialist respiratory service.

Domiciliary long term oxygen therapy

In patients with significant disability or a severe reduction in FEV1 (<40% predicted) regular clinical review should include an assessment of suitability for domiciliary oxygen therapy. While there is no evidence specifically relating to the benefits of long term oxygen therapy in bronchiectasis, this can reasonably be extrapolated from the evidence available for COPD46 and supported by Australian recommendations.47 While assessment for long term oxygen therapy usually requires arterial blood gas analysis, in general, if the oxygen saturation of is >93% on room air when the patient is clinically stable then an arterial puncture is unlikely to reveal sufficient hypoxaemia to warrant this therapy. If the oxygen saturation is 93% or less in nonsmoking patients, then referral to a specialist respiratory service for assessment of the appropriateness of long term oxygen therapy should occur.

Surgery and transplantation

In the past, localised resection/lobectomy of focal bronchiectasis was occasionally undertaken in the hope this would reduce symptoms and prevent extension of disease to unaffected lobes. Local resection remains an option for the management of localised disease and is is generally reserved for bronchiectasis related to NTM resistant to medical management.48 Nonetheless, 26% of these highly selected patients will develop recurrent and progressive disease following surgery.49 If considered, such surgery should be undertaken by specialist respiratory and cardiothoracic services with experience in this area.

In selected patients with severe bronchiectasis lung transplantation also has a role. While there are no national criteria regarding suitability for transplantation, patients aged less than 65 years with severe disease and few or no comorbidities should be assessed for suitability. While many patients with non-CF bronchiectasis may not be suitable for transplantation, in those who are transplanted, the peri-operative mortality is low (<2%) and median survival is greater than 5 years.50

Palliative and end-of-life care

Palliative care focusing on symptomatic management, particularly relating to shortness of breath, anxiety and sputum production, can be invaluable in improving quality of life. In patients with severe disease with symptoms resistant to the medical management as outlined above, fans, benzodiazepines, narcotics and anticholingerics can reduce symptoms. As with any chronic disease, bronchiectasis is often complicated by depression and early detection and treatment of this can also enhance quality of life.

In patients with advanced disease in whom transplantation is not an option, the development of a health management directive with the patient and family is important to ensure acute exacerbations are appropriately and sensibly managed. Management of advanced disease is also often aided by involvement of a palliative care team to facilitate end-of-life care, aid symptom management and provide additional home based support. Given the heterogeneous nature of bronchiectasis and its gradual progression, it is extremely difficult to provide clear guidance regarding prognosis and patient survival. While this may make planning end-of-life care support difficult, there is a clear rationale for early referral to palliative care services for symptom control in patients with severe disease.

Prognosis

It is not surprising that in such a heterogenous group prognosis can vary greatly. Nonetheless, approximately 10% of adults with non-CF bronchiectasis will die within 5–8 years of diagnosis with this being directly attributed to their lung disease in over half.6,51 Factors associated with poorer prognosis include tobacco smoking, Gram negative organisms (especially Escherichia coli and P. aeruginosa) and aspergillus on sputum culture and greater impairment in FEV1 and FVC.34,51

Summary

While many patients with bronchiectasis present with chronic respiratory symptoms, there are a range of clinical factors that should prompt primary care clinicians to suspect this condition. A lack of a smoking history in someone with chronic cough and sputum production, ‘asthma’ resistant to therapy and unusual organisms on sputum culture should raise the suspicion of bronchiectasis. While c-HRCT is increasingly accessible it can pose its own problems. Minor disease in particular needs to be assessed in the clinical context and all scans are associated with a significant radiation dose that is of particular concern in children and younger adults. Once a diagnosis is confirmed it is important to evaluate for secondary causes. While many people will have no cause identified, in the minority who do, this can alter management and outcome. Given its complexity specialist advice is often useful.

The management of bronchiectasis is complicated, multifaceted and requires the coordination of a broad range of healthcare providers. As such it is ideally managed and coordinated in primary care with primary healthcare providers acting as brokers and coordinators of care. Management plans need to be tailored to the individual based on the severity of their disease, acute exacerbation frequency, sputum microbiology, respiratory and nonrespiratory comorbidities and patient preference. Given bronchiectasis is a chronic disease that can be associated with both long term disability, as well as less marked premature mortality, the importance of structured care as part of regular primary care review and reassessment is the key to successful management.

Key points

Bronchiectasis can present for the first time at any age and can often coexist with other lung diseases including COPD and asthma.

It is important to exclude and, where possible, treat secondary causes.

Diagnosis, assessment and ongoing management are best delivered as part of a multidisciplinary approach that includes primary, allied health and specialist respiratory care.

Ongoing management may include:

  • management of acute exacerbations
  • pneumococcal and influenza vaccination
  • treatment of nontuberculous mycobacteria
  • long term oral and inhaled antibiotics
  • physiotherapy/exercise training/pulmonary rehabilitation
  • palliative care
  • surgery and transplantation.

Conflict of interest: none declared.

References

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A pediatric disease to keep in mind: diagnostic tools and management of bronchiectasis in pediatric age | Italian Journal of Pediatrics

Antibiotics

NCFB requires multidisciplinary management in order to control symptoms, reduce exacerbations and preserve lung function.

Although only one organ is concerned, the disease can be complicated by a reduction in statural weight gain and poor overall quality of life.

Redding and coll. in a prospective study monitoring 93 children with NCFB for 3 years, reported that more than two exacerbations occurred in the majority of cases (74%) and a complete medical care was associated with a reduction of exacerbation rate [38].

Antibiotics and airway clearance techniques (ACTs) represent the milestones of NCFB management although the guidelines in children are only a few (Fig. 2).

Fig. 2

Antibiotics should be used in the treatment of acute exacerbation or as prophylaxis treatment to reduce the frequency of acute events. The use and choice of antibiotics should be guided by exacerbations severity, airway microbiology results (sputum culture) and whenever possible by BAL culture. The most commonly isolated bacteria in children are non-encapsulate Haemophilus influenzae, Streptococcus pneumonia, Moraxella catarrhalis and Staphylococcus aureus. Pseudomonas aeruginosa is rarely found in the childhood and it is more often detected in older children or associated with underlying diseases with increased lung damage [39, 40]. Non-typeable H. Influenzae is the major pathogen detected in children of all ages affected by NCFB. This microorganism is able to form a biofilm on the airway epithelia that damages cilia and impairs the bronchial clearance mechanism [41].

In our opinion, bacteria involved in exacerbations should always be identified in order to use a targeted antibiotic therapy that minimizes the future risk of resistance.

Sputum sample culture or nasopharyngeal aspirates (in children who are unable to expectorate) should be obtained before starting antibiotics. A pharyngeal swab after coughing may be a surrogate of the lower respiratory tract pathogens in very young children and it is sometimes preferred in some older children [42].

To date, despite the advances in the management of many pediatric diseases antibiotic therapy remains one of the most effective tool for the treatment of pediatric NCFB.

Prolonged cycles of antibiotics (very often more than 4 weeks) are often necessary to eradicate some respiratory pathogens responsible for exacerbation [39].

A recent review involving 15 studies (925 participants), showed a significantly reduction in the rate of exacerbations and hospitalization in children and adults with NCFB who had been treated with prolonged antibiotics (four or more weeks). However, the risk of emerging drug resistance was increased more than threefold, therefore an accurate patient selection is essential [43].

Unfortunately, there are currently no randomized controlled trials (RCTs) evaluating the efficacy of inhaled antibiotics in children with NCFB, even though this option in adult patients has been largely used and showed several benefits. The rationale for using inhaled antibiotics in lung diseases lies in the possibility to administer a relatively high dose of drugs directly to the site of disease reducing systemic absorption and related toxicity.

A systematic review conducted by Brodt et al. comprising 12 trials with 1264 adults with clinically stable NCFB and chronic bronchial infection, showed that inhaled antibiotics were more effective than placebo in reducing sputum bacterial load and the risk of acute exacerbations [44].

According to current BTS guidelines, nebulized antibiotics should be considered in children who experience recurrent exacerbations (or deteriorating lesions) unaffected by prolonged oral antibiotic or if colonized with Pseudomonas Aeruginosa [45].

Nebulized gentamicin in children with NCFB seems to be well tolerated and produce satisfactory drug level in the sputum. Nebulized tobramycin was mainly used in children with CF and demonstrated good efficacy in reducing the concentration of Pseudomonas and improving the FEV1 [46]. However, there are a few data in NCFB and in any case, the choice of the nebulized antibiotic should be guided by culture results.

In the recent years, there is an increased interest in the use of macrolides for the treatment of NCFB, mainly due to their antinflammatory effects and their well-established direct antimicrobial effect on Gram-positive cocci and atypical pathogens but also for the properties to decrease mucus production.

Two meta-analyses that included seven studies evaluating various macrolides (azithromycin, erythromycin, roxithromycin) reported a significant reduction in the frequency of exacerbations compared with placebo [39, 43].

Similarly, the EMBRACE trial (Effectiveness of Macrolides in patients with Bronchiectasis using Azithromycin to Control Exacerbations) involving three centers in New Zealand, showed that the use of Azithromycin three times a week for 6 months in adult patients with NCFB has resulted in a significant reduction in event-based exacerbations and increase of the time of the first event-based exacerbation compared with placebo [47].

Despite these encouraging evidences, the risk of drug resistances should not be underestimated. In that regard, in BLESS study (Bronchiectasis and Low-dose Erythromycin Study) Rogers and colleagues reported changes in microbiota composition of patients with NCFB (determined by 16S rRNA gene sequencing of sputum samples from 86 participants) after 48 weeks of low-dose erythromycin. Moreover, long-term erythromycin treatment seems to induce replacement of H. influenzae with more macrolide-tolerant pathogens such as P. aeruginosa. These evidences suggest that chronic macrolide therapy should be used with caution in subjects with NCFB but without P. aeruginosa infection [48].

Airway clearance techniques

Airway clearance techniques (ACTs) are usually recommended in children with bronchiectasis even though evidences supporting this practice are lacking and mainly derives from study on Cystic Fibrosis.

In the childhood, the family compliance may significantly influence the adherence to ACT and the non-adherence is the primary cause of treatment failure in chronic pediatric lung conditions. Other factors to consider when choosing ACT are child’s age, level of collaboration and maturity.

Lee et colleagues showed that ACTs seemed to be safe for adults and children with stable bronchiectasis leading to improvements in sputum expectoration, selected measures of lung function, patient symptoms and HRQoL (health related quality of life) [49].

Different types of ACTs include: active cycle of breathing techniques, autogenic drainage, forced expiration techniques, postural drainage, oscillating positive expiratory pressure, high-frequency chest wall oscillation, and physical exercise. This latter is highly recommended for all ages in the management of bronchiectasis and chronic suppurative lung disease.

Furthermore, the application of positive expiratory devices and high-frequency chest wall oscillation showed an improvement of FEV1 as indices of lung function. However, all the studies were conducted in individuals in a stable clinical state, so the effects of these techniques during an acute exacerbation remain unclear and further studies are necessary, especially in children [50, 51].

Finally, the use of both beta2 agonists and hypertonic solutions before performing airway clearance techniques are all considered useful strategies for improving clearance of bronchial secretions in NCFB.

Mucolytic agents

The mucolytic agents could theoretically be useful in the management of bronchiectasis due to their ability to reduce the viscosity of the mucus and thus make it easier to expectorate. Among these drugs, recombinant human DNase, acts in degrading DNA released by neutrophils in the infection site.

A recent Cochrane review involved four trials with 528 adult participants, concluded that little evidence is available to recommend the routine use of mucolytic agents in bronchiectasis [52].

In 2015 Welsh et al. in an “overview of Cochrane systematic reviews” regarding the various treatments currently available for non-cystic fibrosis bronchiectasis, reported that RhDNase seems to be associated with an increase in exacerbations, although three reviews only involved pediatric population.

Current BTS guidelines conclude that due to a potential worsening of pulmonary function, RhDNase should not be used in management of bronchiectasis in pediatric patients [53].

Two reviews reported beneficial effects for other agents such as hypertonic saline (exacerbations), erdosteine (FEV1) and mannitol (QoL) [52].

About this last one, a recent RCT showed that the use of mannitol 400 mg inhaled twice daily for 12 months in adult patients with bronchiectasis seems to improve the quality of life and increase the time to the first exacerbation even though it did not reduce exacerbation rates [54, 55].

The role of hypertonic saline in the management of NCFB is still debated. Although the recent Cochrane systematic reviews of Welsh et al. concluded that there is inconclusive evidence on the use of nebulized hypertonic for reducing exacerbation, several studies suggested that in concentrations of 3–14%, hypertonic saline could improve tracheobronchial clearance.

A probable mechanism of action consists in the reduction of sputum viscosity by inducing liquid flux from the epithelium into the mucus so that the mucus is cleared more easily by the cilia.

Kellet and coll. Demonstrated that nebulized hypertonic saline significantly reduced sputum viscosity and can be used safely and effectively as an adjunct to physiotherapy in patients with stable bronchiectasis [56, 57].

Combined ICS/LABA therapy

Several studies have reported the presence of a certain degree of bronchial hyper-responsiveness and chronic airflow bronchial obstruction in subjects with bronchiectasis. For this reason, inhaled corticosteroids (ICS) therapy may potentially improve several bronchial inflammatory parameters and clinical symptoms, even though high doses with increased risk of side effects are often required [58].

The use of combined therapy with long-acting beta2-agonist (LABA) and ICS administered together in a single device showed a synergistic effect in decreasing bronchial inflammation.

A Cochrane’s systematic reviews of all randomized controlled trials on the use of combined ICS and LABA compared to a control (placebo, no treatment, ICS as monotherapy) in children and adults with bronchiectasis, concluded that in adults combined therapy was partially more effective than high-dose ICS in improving some clinical symptoms like cough free-day and dyspnea. However, no significant improvement in the rate of hospitalization and no change in lung function indices have emerged. Moreover, no data are provided in children with bronchiectasis in a stable or acute state [59].

A single-center observational study performed by Wei et al. involving 120 adult patients with NCFB, reported that subjects who received salmeterol-fluticasone combined therapy versus routine therapy showed significant improvement in HRQoL and rates of exacerbations, without risk of severe adverse events [60].

Previously, Martinez-Garcia also reported in a 12-month randomized and double-blind trial including 40 patients with NCFB, that inhaled medium-dose of formoterol-budesonide combined therapy was more effective and safe compared with high-dose of budesonide in improving clinical symptoms such as dyspnea, cough and wheeze [61].

Despite these encouraging findings, there is a lack of safety data about the combined ICS/LABA therapy in children, therefore more robust evidence is needed to recommend its routinely use in NCFB.

Surgical treatment

Finally, surgical treatment such as segmentectomy and lobectomy may be reserved for patients with localized bronchiectasis with persistent symptoms, recurrent infections despite maximal therapy and hemoptysis. Nevertheless, further studies are needed before surgery can be recommended as a safe treatment for NCFB. Factors that seem to be relative contraindications for surgical resection of bronchiectasis include non-cylindrical disease, persistent infection of Pseudomonas documented by sputum culture, residual disease after resection and non-localized disease [62,63,64,65].

Bronchiectasis: the Consequence of Late Diagnosis in Chronic Respiratory Symptoms | Journal of Tropical Pediatrics

Abstract

Bronchiectasis is still common among some developing countries like Turkey. The aim of this study was to document the number of children with non-cystic fibrosis (CF) bronchiectasis, to evaluate the risk factors and to emphasize early diagnosis and treatment. All children, except those diagnosed with CF, with bronchiectasis established by chest radiogram, bronchography and/or computed tomography or biopsy material, were retrospectively reviewed. They were tested for serum total eosinophil count, nasal smear, serum levels of immunoglobulins A, G, M, E, and serum alpha-1 antitrypsin level. Pulmonary function tests, rigid bronchoscopy, nasal biopsy, lung scintigraphy, and echocardiogram were also performed. There were 204 patients whose most common presenting symptoms were cough, sputum expectoration, and dyspnea. Bronchiectasis was present mostly in the left lower lobe. The cause could not be determined in 49 per cent of patients. Among the identified causes, infection was present in most patients, followed by asthma, primary ciliary dyskinesia, congenital immune deficiency, and foreign body aspiration. It is possible to prevent bronchiectasis in children with vaccinations and improved nutrition in developing countries. Early diagnosis and treatment will increase the quality of life and survival of patients with bronchiectasis, which has irreversible and progressive complications if untreated.

Introduction

Bronchiectasis is a progressive condition that causes permanent changes in the structure and function of the airways. It is characterized by frequent bacterial infections and inflammatory destruction of the bronchial and peribronchial tissue.1 Barker and Bardana2 referred to bronchiectasis as an ‘orphan disease’ as it is an uncommon disease that is neglected in research and treatment development. Bronchiectasis is rarely seen in developed countries except in patients with cystic fibrosis (CF), primary ciliary dyskinesia, immune deficiency, or focal pulmonary abnormalities. In contrast, it remains common among some developing countries, such as Turkey.3,4 In such countries, it is important to recognize the high-risk groups as early treatment, early diagnosis, and effective treatment increase the survival and quality of life of the patients. The aim of this study was to document the number of children with non-CF bronchiectasis diagnosed in our hospital and to evaluate the risk factors that cause bronchiectasis and to emphasize early diagnosis and early treatment.

Materials and Methods

Children diagnosed with bronchiectasis at Hacettepe University Pediatric Pulmonary Medicine Unit in Ankara, Turkey over a period of 13 years were included in the study. The diagnosis of bronchiectasis was established by: clinical history of daily or frequent cough with sputum production for more than 3 months; radiological methods such as chest radiogram compatible with bronchiectasis (saccular changes or cylindrical outlines of airway that widen as airways extend into the periphery) and bronchography and/or computed tomography; and with biopsy material, if obtained. Bronchography was used before 1990 as it was the only radiological method available for the exact diagnosis of bronchiectasis. CF was excluded in all patients with sweat chloride test. All patients had chest radiograms. Serum total eosinophil count, nasal smear, and serum levels of immunoglobulins A, G, M, E were obtained. One hundred and nine patients aged more than 6 years old had pulmonary function tests with spirometry. Rigid bronchoscopy, bronchography, plain sinus graphy, nasal biopsy, lung scintigraphy, and echocardiogram were also performed. The age at diagnosis, sex, family history, presenting symptoms, physical examination findings, presence of associated abnormality, and etiologic factor were retrospectively evaluated. ‘Infection’ was defined as symptoms such as fever, cough and sputum expectoration together with radiological findings consistent with pneumonia.

Results

Among 204 patients diagnosed with bronchiectasis, 99 were females (48.5 per cent) and 105 were males (51.5 per cent). The mean age of all patients was 7.16 ± 3.72 years. The disease was diagnosed mostly at 8 years of age. There was consanguinity of parents in 76 patients (37.2 per cent). The most common presenting symptom was cough (83.3 per cent) followed by sputum expectoration (22.5 per cent) and dyspnea (8.8 per cent).

Failure to thrive was obtained in 94 patients (46.1 per cent) at the time of diagnosis. Body weight was under the third percentile in 55 patients (35 per cent) at follow-up. Furthermore, 18 patients had ottitis media, two had adenoid vegetation, and 10 had loss of hearing at different levels. Other physical examination findings are shown in Fig. 1.

Fig. 1.

Physical examination findings of the patients.

Fig. 1.

Physical examination findings of the patients.

Every patient had a chest radiogram. The diagnosis was made by bronchography and thoracal computed tomography (CT) in seven patients with atelectasis, and nine patients with chronic changes in chest radiograms. The diagnosis of bronchiectasis was confirmed by evaluation of lobectomy material in three patients who had atelectasis. In chest radiograms, bronchiectasis was present mostly in the left lower (148 patients, 72.5 per cent) and right lower lobes (115 patients, 56.4 per cent), followed by the right middle lobe in 25 patients (12.3 per cent), right upper lobe in 10 patients (4.9 per cent), and left upper lobe in 13 patients (6.4 per cent). More than one lobe was involved in 91 (44.6 per cent) of the patients. The most common combination was left lower and right lower lobe in 52 patients. Atelectasis was obtained in 57 (28.2 per cent) and dextrocardia was present in 13 patients. The distribution of lobes involved and types of bronchiectasis obtained in bronchographies is shown in Table 1.

Table 1

The distribution of lobes and types of bronchiectasis

Lobe
Cylindric
Fusiform
Saccular
Obstructive
Not defined
Right upper       
Right middle   
Right lower  10  11  11   
Left upper       
Left lower  16  11  28 
Total  20  26  49  12 
Lobe
Cylindric
Fusiform
Saccular
Obstructive
Not defined
Right upper       
Right middle   
Right lower  10  11  11   
Left upper       
Left lower  16  11  28 
Total  20  26  49  12 

Table 1

The distribution of lobes and types of bronchiectasis

Lobe
Cylindric
Fusiform
Saccular
Obstructive
Not defined
Right upper       
Right middle   
Right lower  10  11  11   
Left upper       
Left lower  16  11  28 
Total  20  26  49  12 
Lobe
Cylindric
Fusiform
Saccular
Obstructive
Not defined
Right upper       
Right middle   
Right lower  10  11  11   
Left upper       
Left lower  16  11  28 
Total  20  26  49  12 

Hypertrophia in maxillary sinuses or loss of aeration in unilateral or bilateral maxillary sinuses was obtained in 108 out of 125 tested patients (84.6 per cent). Bronchoscopy revealed copious mucus secretions in 29 and foreign bodies in seven out of 44 tested patients.

Immune deficiencies were obtained in 11 patients; four patients had ataxia telengiectasia, two had common variable immune deficiency, two had selective IgA deficiency, two had IgG subgroup deficiency, and one had X-linked agammaglobulinemia. Total eosinophil count was more than 400/mm3 in 27 patients and eosinophils were detected in 32 patients’ nasal secretions. IgE level was more than 300 IU/l in three patients.

Tuberculin skin test with 5 TU was more than 10 mm in 46 patients. This positivity was attributed to BCG vaccination in 24 patients and they were not treated. Twelve patients were treated with isoniasid 5 mg/kg for 9 months because of positivity of tuberculin skin test and 10 patients were treated with antituberculous drugs as they had a history of contact with a tuberculosis (TB) patient, clinical and radiological findings of TB in addition to a positive tuberculin skin test.

Nasal biopsy was taken in 16 patients, which revealed secondary changes due to upper respiratory tract infection and changes in spoke orientation in one patient. Primary ciliary dyskinesia was diagnosed in an additional 23 patients who had dextrocardia and a similar family history.

Pulmonary function tests with spirometry was performed in 109 patients, which showed obstructive and restrictive changes in 61 (55.9 per cent), obstructive in 35 (32.1 per cent), normal in 12 (11 per cent), and restrictive in one (0.9 per cent) patient.

The etiologic factors for bronchiectasis in our patients are shown in Table 2.

Table 2

Etiological factors for bronchiectasis


No. of patients
%
Infection  33  16.1 
    Non-specific  23   
    Tuberculosis  10   
Asthma  24  11.8 
Primary ciliary dyskinesia  24  11.8 
Congenital immune deficiency  11  5.4 
    Ataxia telengiectasia   
    Defiency of IgA and IgG2   
    Defiency of IgA   
    Common variable immune deficiency   
    X-linked gammaglobulinemia   
Foreign body aspiration  3.4 
Radiotherapy and chemotherapy 
Sulphur inhalation  0.5 
Chest deformity  0.5 
Yellow nail syndrome  0.5 
Unknown  100  49 
Total  204  100 

No. of patients
%
Infection  33  16.1 
    Non-specific  23   
    Tuberculosis  10   
Asthma  24  11.8 
Primary ciliary dyskinesia  24  11.8 
Congenital immune deficiency  11  5.4 
    Ataxia telengiectasia   
    Defiency of IgA and IgG2   
    Defiency of IgA   
    Common variable immune deficiency   
    X-linked gammaglobulinemia   
Foreign body aspiration  3.4 
Radiotherapy and chemotherapy 
Sulphur inhalation  0.5 
Chest deformity  0.5 
Yellow nail syndrome  0.5 
Unknown  100  49 
Total  204  100 

Table 2

Etiological factors for bronchiectasis


No. of patients
%
Infection  33  16.1 
    Non-specific  23   
    Tuberculosis  10   
Asthma  24  11.8 
Primary ciliary dyskinesia  24  11.8 
Congenital immune deficiency  11  5.4 
    Ataxia telengiectasia   
    Defiency of IgA and IgG2   
    Defiency of IgA   
    Common variable immune deficiency   
    X-linked gammaglobulinemia   
Foreign body aspiration  3.4 
Radiotherapy and chemotherapy 
Sulphur inhalation  0.5 
Chest deformity  0.5 
Yellow nail syndrome  0.5 
Unknown  100  49 
Total  204  100 

No. of patients
%
Infection  33  16.1 
    Non-specific  23   
    Tuberculosis  10   
Asthma  24  11.8 
Primary ciliary dyskinesia  24  11.8 
Congenital immune deficiency  11  5.4 
    Ataxia telengiectasia   
    Defiency of IgA and IgG2   
    Defiency of IgA   
    Common variable immune deficiency   
    X-linked gammaglobulinemia   
Foreign body aspiration  3.4 
Radiotherapy and chemotherapy 
Sulphur inhalation  0.5 
Chest deformity  0.5 
Yellow nail syndrome  0.5 
Unknown  100  49 
Total  204  100 

Discussion

In this study, the late presenting age of the patients, failure to thrive, digital clubbing, and the predominance of saccular lesions show that our patients presented with advanced and severe disease. When the presenting age of our patients was compared with other reports, we found that our patients presented at an older age than reported in the literature. Most patients are generally reported to present in preschool years.3–6

It is known that cylindric dilatation is seen in mild disease and is reversible, but saccular bronchiectasis occurs as the process continues and damage to muscular layers and destruction of elastic tissue, edema and inflammatory cell infiltration into the surrounding tissue occur.1 Our most common type of bronchiectasis was saccular, which is irreversible and has an unfavorable prognosis.

In our study the most common presenting symptom was cough followed by sputum expectoration, which is in concordance with the literature.1,3,7 Although hemoptysis is a common symptom in adults,1 its prevalence was found to be 4–7 per cent in pediatric series.3,8 In our study, prevalence was 3.9 per cent and was not massive. Although it has been reported that most patients present with infection,9 the percentage of our patients who presented with fever and severe infection symptoms was 7.8 per cent. In other series, recurrent infection was found in 35 per cent of patients,3,7 whereas it was only 6.4 per cent in our study.

Bronchiectasis is most commonly seen in the lower lobes, especially in the left lower lobe. The upper lobes are involved less frequently in contrast to CF, probably because of the faciliated mucociliary clearance by gravity.10 We found that the most common involved lobes were the left lower lobe and the right lower lobe. In the literature, it has been reported that left lower lobe and lingulae were most commonly affected, followed by the right middle lobe and the right lower lobe.3,6,9 However, in a study by Fleshman et al.11 the predominance of the right lung, especially the right upper lobe was demonstrated, which differed from the general experience.

The incidence of bronchiectasis has decreased due to vaccination and effective treatment of infections in developed countries. However, it is still an important cause of chronic suppurative pulmonary disease in developing countries.10 The most common cause of bronchiectasis among the identified cases in our report was infection, including tuberculosis. A different study from Turkey also showed that infection was the most common cause of bronchiectasis in children, with a percentage of 34.8 in the south-east of the country.10 This shows that infection is still the most important reason for development of bronchiectasis in our country. Among other reports, Fleshman et al.11 reviewed the clinical histories of 100 Alaska native children with bronchiectasis in 1968 and found that half were non-tuberculous. Among those 49 patients, 35 had pneumonia, five had measles, three had pertussis, one had Echinococcus granulosus, and 18 had undetermined causes. In a study by Singleton et al.12 recurrent pneumonia was obtained in 86 per cent of patients as a preceding medical event in Alaska native children; 80 per cent of those had at least one documented pneumonia or other lower respiratory tract infection in the first year of life before the diagnosis of bronchiectasis was made, and a majority of pneumonias did not have a pathogen. In our series, there were no cases with histories of known pertussis or measles. However, the presence of tuberculosis as a cause of bronchiectasis in our series was noticeable. Tuberculosis has not been considered as a cause of bronchiectasis in recent decades, because of a decrease in primary pulmonary tuberculosis and improved treatment regimens.10 But as seen from our report, it can still be an important cause of bronchiectasis in children and can be a threatening condition for the development of bronchiectasis unless its spread is controlled and appropriate treatment is established.

There are numerous causes of bronchiectasis; however, despite extensive investigations, the underlying disease for this disorder remains unidentified in some cases. A disorder such as congenital malformations, ciliary defects or immunodeficiency could be identified in 63 per cent of children in one study.13 A study from New Zealand showed that the cause of bronchiectasis could not be found in 50 per cent of children.14 In another report from north-west Turkey, only 40 per cent of patients with bronchiectasis had an underlying cause and immunodeficiency, measles, ciliary dyskinesia, asthma, foreign body aspiration, and chronic aspiration syndromes were the main identified causes.15 In this report, we were able to determine the etiology of bronchiectasis in only 51 per cent of our patients. The high prevalence of consanguinity in our patients may suggest a genetic cause for ‘idiopathic’ bronchiectasis, which needs further study.

In conclusion, it was found in this study that among the identified causes, the most common disease that caused bronchiectasis was infection. Besides non-specific infection, tuberculosis was an important cause of bronchiectasis. It is possible to prevent bronchiectasis in children with vaccinations and improved nutrition in developing countries. In addition, early diagnosis and treatment will increase the quality of life and survival of patients with bronchiectasis, which has irreversible and progressive complications if untreated.

References

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© The Author [2005]. Published by Oxford University Press. All rights reserved. For Permissions, please email: [email protected]

Bronchiectasis> Clinical Protocols 2006-2019 (Belarus)> MedElement

Appendix 5
to order
of the Ministry of Health
of the Republic of Belarus
05.07.2012 No. 768

CLINICAL PROTOCOL
diagnosis and treatment of bronchiectasis

This clinical protocol for the diagnosis and treatment of bronchiectasis is intended for the provision of medical care to the population in outpatient and inpatient conditions of district, regional and republican health organizations of the Republic of Belarus.
Bronchiectasis – a purulent-inflammatory process in the dilated deformed and functionally defective bronchi with infiltrative and sclerotic changes in the peribronchial space, leading to further irreversible expansion of the bronchi with impaired drainage function, the development of atelectasis, emphysema, cirrhosis.
Bronchiectasis is, as a rule, segmental expansion of the bronchi caused by destruction or disturbance of the neuromuscular tone of their walls due to inflammation, dystrophy, sclerosis or hypoplasia of the structural elements of the bronchi.Bronchiectasis can be primary (with congenital anomalies and genetic diseases of the lungs) and secondary (post-infectious, obstructive, aspiration, due to diffuse lung diseases). Infectious pathogens cause an exacerbation of the suppurative process in the altered bronchi (staphylococcus, pneumococcus, haemophilus influenzae and associations of microorganisms).
In the pathogenesis, changes in large bronchi (lobar, segmental) are important, which leads to a violation of the drainage function and the formation of obstructive atelectasis, followed by the development of pneumosclerosis and the progression of the inflammatory process up to ulceration and destruction of the muscular-elastic layer of the bronchial wall cartilage.
Airway obstruction and secretion retention lead to the development of a suppurative process distal to the obturation site. The destructive process is the second important factor in the pathogenesis of bronchiectasis, since it causes progressive irreversible changes in the walls of the bronchi (restructuring of the mucous membrane with damage to the ciliated epithelium, degeneration of cartilaginous plates, smooth muscles with their replacement with fibrous tissue). The walls of the bronchi, due to a decrease in their resistance, are not able to withstand an increase in endobronchial pressure during coughing, valvular bronchoconstriction.accumulation of secretion, stretching of the walls of the bronchi by the cirrhotic process, etc. Since the changes in the bronchial tree are irreversible, even after the restoration of patency in the dilated bronchi with impaired cleansing function, conditions that support the chronic suppurative process remain.
In bronchiectasis, pulmonary circulation is impaired. The lumen of the bronchial arteries and arterio-arterial anastomoses is significantly increased, which leads to the discharge of arterial blood into the pulmonary arteries and the subsequent development of regional and then general pulmonary hypertension.
Age category: adult population of the Republic of Belarus.
Name of the nosological form of the disease:
(code according to ICD-10):
Bronchiectasis (J47).
Men are more likely to suffer from bronchiectasis. The disease, as a rule, is detected in childhood and young age. The time of the onset of the disease is often difficult to establish, since the first exacerbations are often interpreted as respiratory diseases. With a well-collected anamnesis, it is possible to identify pneumonia transferred in early childhood, which caused the development of bronchiectasis.
The leading clinical symptom of the disease is cough, which is most pronounced in the morning, with sputum production. With cylindrical bronchiectasis, sputum is usually excreted without difficulty, and with saccular bronchiectasis, with difficulty. With “dry” bronchiectasis, characterized by the absence of a suppurative process, cough and sputum are absent. During the period of remission, the amount of sputum is small, usually not exceeding 30 ml per day. With an exacerbation of the disease, the amount of sputum can increase to 500 ml or more per day, it becomes purulent.Sputum can be discharged with a “full mouth” in the morning, as well as when the patient assumes drainage positions: tilt forward, turn to the “healthy” side. Putrid sputum occurs with abscess formation in severe patients.
In a severe course of the disease and during its exacerbation, the general condition worsens: malaise, lethargy and work capacity decrease. There is marked sweating and a prolonged increase in body temperature to high numbers.
The symptoms of bronchiectasis may be accompanied by a picture of complications – hemoptysis, pulmonary hemorrhage, spontaneous pneumothorax, abscess formation, pleural empyema, pulmonary heart disease, pulmonary heart failure, renal amyloidosis, myocardial dystrophy.
With a long course of bronchiectasis, changes are observed in the terminal phalanges of the fingers in the form of drumsticks and nails in the form of hour hands. The chest may be deformed as a result of emphysema and pulmonary fibrosis.

(PDF) Modern approaches to the management of patients with bronchoectasia

is a violation of the structure and function of movable cilia

, which leads to chronic –

mu otosinus-pulmonary disease.PCD

is manifested by respiratory disorders, according to –

standing cough and nasal congestion from an early

childhood. M. Kartagener in 1933 for the first time

described a clinical syndrome including the triad

(chronic sinusitis, EB and situs viscerum inversus),

A.P. Camner as the cause of this syndrome for the first time in 1975

discine –

zia [29].

PCD is a rare genetic disease with a prevalence of

oddity approximately 1: 15,000 newborns –

days [29].PCD is caused by autosomal recessive

or X-linked mutations in 1 of more than

than 35 genes, which lead to functional

and / or structural defects of cilia [30].

Mobile cilia are specialized

lysed structures present in many tissues, therefore their immobility or ineffective beating leads to a systemic disease

with heterogeneous signs. In the upper and lower

DP, insufficient mobility of the cilia leads

to a deterioration of mucociliary clearance, which

triggers a “vicious circle” of inflammation and infection,

which leads to chronic rhinosinusitis and medium otitis,

otitis media, progressive obstruction of the DP, BE

and, ultimately, respiratory insufficiency –

sti [31].Since embryonic nodular cilia

can also be underdeveloped, approximately

in 50% of patients, situs viscerum inversus

(complete transposition of internal organs) and hetero-

rotaxia are observed. In Kartagener’s syndrome, situs inversus

is accompanied by chronic sinusitis and EB [31].

Sperm flagella and cilia of fallopian

tubes have common axonemal structures, therefore

most of men and women suffering from PCD,

are infertile [32].

PCD diagnostics is difficult, some

diagnostic tests are quite expensive or require a high level of knowledge, only

are performed in specialized centers. According to the ERS recommendations

, PCD diagnostics includes the measurement of

NO in the nasal air (nNO) and the study of the

frequency and pattern of beating of cilia scraping from the mucous

using a high-speed

video analysis [1] …To confirm the diagnosis of

, an assessment of ultrastructural defects of

cilia using transmission electron microscopy or genetic testing is used,

which allow detecting PCD with a specificity of

80 and 70%, respectively. Recently,

is also used for the detection of ciliary proteins

on the ciliated cells of the respiratory epithelium

using immunofluorescence [33].

ERS restricts PCD testing to

individuals with clinical signs such as

persistent productive cough since childhood, chronic rhinosinusitis

, middle ear pathology with or without hearing loss, situs inversus, congenital

heart defects and neonatal respiratory

distress syndrome [1].All these clinical manifestations are included in the predictive detection scale

PCD (PICADAR) – a method for diagnosing PCD in patients

with suitable symptoms [34]. The

PICADAR scale and nNO analysis are easy for

to perform with tests in the pulmonary departments

, while further examination should be

but carried out in specialized centers

with experience in managing such patients. It is important to note,

, that the nNO level is often used as a screening

ning test for PCD, although in some cases it

may be normal.

In patients with suspected PCD, during

MSCT, EB is detected mainly in the middle

her lobe, reed segments and the lower lobes of the lungs

with a characteristic central or diffuse

spread. As follows from the pathophysiology of

diseases, the severity of EB increases with age,

although the data on the correlation between MSCT data from

and lung function are contradictory. In addition to changes in the lung parenchyma, suspicion of PCD is caused by situs inversus, heterotaxy and congenital heart defects.Funnel deformity of the chest

cells occurs in 9% of cases [35].

Diagnosis of PCD is a difficult task, since

in this multisystem disease requires

specific and interdisciplinary command

approach, including genetic and reproductive

counseling in specialized centers

[2, 29]. In the treatment of patients with PCD and BE, especially

, the clearance of the DP is important. The significance of the early diagnosis

is emphasized by the data showing

deterioration of pulmonary function and high

dissemination of P.aeruginosa in patients of older

age groups [36, 37].

Clinical observation No. 4

A 76-year-old patient with established idiopathic EB. Despite

on daily procedures to improve the clearance

DP, there were 3-4 exacerbations per year over the past 3 years.

Analyzes for collagenoses, NTMB, ABLA and other infections –

negative, persistent infection with P. aeruginosa

was found in sputum cultures.Despite active methods of tracheo-

bronchial clearance and secretion removal, she retained

sputum production in large volumes. This is one of the main

manifestations of EB, primarily during exacerbations of the disease, and P. aeru-

ginosa is the most common causative agent in severe EB in the world.

Fig. 4 shows different MSCT phenotypes of BE. Qi –

lindrical BEs – the most common morphological pattern found on MSCT [38], is recognized by

abnormal bronchial dilatation with a uniform caliber and no narrowing at the periphery (tram rails symptom)

(see.rice. 4A). Varicose (fusiform) BE is diagnosed

only in the longitudinal plane, characterized by the alternation of

sections of the dilated and narrowed bronchial lumen (see Fig.4B),

while cystic (saccular) BE is a mesh-

chaotic dilatation of the bronchial lumen , which can be traced

almost to the surface of the pleura (see Fig. 4C).

Localization and dissemination are also

key features in evaluating BE.Locals –

called BE, especially in 1 lobe, is detected in

obstructive disorders and after a transferred

pulmonary infection (Fig.5A). It is also possible to find cavities

, especially after the transferred tuber-

Pulmonology. 2020; 30 (1): 81–91. DOI: 10.18093 / 0869-0189-2020-30-1-81-91

86

Anaev E.Kh. Modern approaches to the management of patients with bronchiectasia

CLASSIFICATION OF BRONCHEECTASES: (A.I. Borokhov, N.R. Paleev, 1990) 1. By origin: 1.1. Primary

(A.I.Borokhov, N.R. Paleev, 1990)

1. By origin:

1.1. Primary (congenital cysts) bronchiectasis.

1.1.1. Solitary (solitary).

1.1.2. Multiple. l..l-Z. Cystic lung.

1.2. Secondary (acquired) bronchiectasis.

2. According to the form of bronchial dilatation:

2.1. Cylindrical.

2.2. Baggy.

2.3. Fusiform.

2.4. Mixed.

3. According to the severity of the course (clinical forms):

3.1 Mild form.

3.2. Pronounced form.

3.3. Severe form.

3.4. Complicated form.

4. Dry bronchiectasis.

5. By prevalence:

5.1. Unilateral bronchiectasis (indicating the exact localization of the process by segments).

5.2. Bilateral bronchiectasis.

6. According to the phases of the disease:

6.1. Aggravation.

6.2. Remission.

7. Presence of complications:

1) bleeding;

2) cor pulmonale;

3) amyloidosis;

4) pulmonary heart failure.

EXAMPLE FORMULATION OF DIAGNOSIS

1. Bronchiectasis in the acute stage with a predominant lesion of the right lung by saccular bronchiectasis, pronounced form.

2. Bronchiectasis with lesions of the left lung with dry bronchiectasis complicated by bronchiectasis.

CLINIC. The disease is recognized at the age of 5 to 25 years, men are sick more often.

The most characteristic complaint of patients is a cough with a large amount of sputum, mainly in the morning, purulent or mucopurulent. During an exacerbation, the amount of sputum separated can reach a liter or more with an unpleasant putrid odor. Periodically, many patients experience hemoptysis and blood streaks in the sputum. Shortness of breath occurs with moderate exercise.Chest pains are dull. Patients complain of lethargy, irritability, decreased performance. During the period of exacerbation, the temperature rises to subfebrile numbers, mainly in the evening hours.

During the external examination of patients with bronchiectasis, there is some delay in development and growth in children and adolescents, delayed sexual development of secondary sexual characteristics, amenorrhea in girls. With total damage to the lung, the patient has a decrease in the volume of one half of the chest and restriction of respiratory excursions.In patients with bronchiectasis with a widespread lesion, an earthy color of the skin is observed, the fingers take the form of “drumsticks” and the nails are deformed in the form of “watch glasses”.

Chest percussion data in bronchiectasis are not very typical.

During auscultation over the affected, more often the posterior lower parts of the lung, sonorous large and medium bubbling rales are heard. After coughing up sputum, the number of wheezing decreases, and sometimes they completely disappear.In the area of ​​the altered areas, hard or bronchial breathing is heard with atelectatic bronchiectasis.

DIAGNOSTICS. The severity of radiological manifestations in bronchiectasis depends on the prevalence of bronchiectasis, the degree of development of changes in the bronchi and surrounding tissue.

On conventional radiographs and tomograms, it is not always possible to detect bronchiectasis.

Indirect signs of bronchiectasis:

1. Decrease in the volume of the affected parts of the lung.

2. Increase the transparency above – or below the segments.

3. The appearance of peribronchial sclerosis. •4. Pulling up.

Against the background of a rough and reinforced pulmonary pattern, cellularity can be detected. h The decisive diagnostic method is bronchography.

In the saccular form of bronchiectasis, the pathologically altered bronchi look clavate dilated, a honeycomb pattern is determined. With cylindrical bronchiectasis, the bronchi are uniformly dilated.

TREATMENT.

Antibiotic therapy is carried out during an exacerbation of the disease (preferably after determining the sensitivity of pathogens to antibiotics). The intrabronchial route of administration of drugs through a bronchoscope is preferred.

Broad-spectrum antibiotics are prescribed: semi-synthetic penicillins, cephalosporins, aminoglycosides, tetracyclines, quinolones. It is advisable to combine endobronchial administration of drugs with intramuscular or intravenous administration. For endobronchial administration, dioxidine, derivatives of nitrofurans (furacilin, furagin) and antiseptics of natural origin (chlorophyllipt) are used.

Sanitation of the bronchial tree, removal of purulent bronchial contents and sputum, is carried out using instillations through a nasal catheter or during bronchoscopy, introducing therapeutic solutions of antiseptics, mucolytics (mucosolvin, acetylcysteine). In order to sanitize the bronchial tree, the following are used: postural drainage several times a day, expectorants, bronchodilators, chest massage.

Detoxification therapy. It is recommended to drink plenty of water up to 2-3 liters per day.Hemodez, isotonic sodium chloride solution, 5% glucose solution are injected intravenously.

Immunomodulatory therapy. For treatment, leva-mizol, diucifon, thymalin, T-activin are used. To normalize general and pulmonary reactivity, tincture of ginseng, extract of Eleutherococcus, pantocrine, etc. are used.

Rehabilitation of the upper respiratory tract. Thorough treatment of teeth, chronic tonsillitis, pharyngitis, diseases of the nasal cavity.

Exercise therapy, breathing exercises, massage, physiotherapy, spa treatment.Exercise therapy and breathing exercises are carried out regularly. Chest massage improves drainage function, sputum removal. Physiotherapy is carried out when the symptoms of exacerbation of the disease subside. The patient is prescribed electrophoresis with calcium chloride, potassium iodide, inductothermy, microwave therapy. Sanatorium treatment is carried out in the inactive phase of the disease in the warm season, best of all in the sanatoriums of the South Coast of Crimea.

Surgical treatment. Indication: limited within individual segments or lobes of bronchiectasis without pronounced chronic obstructive bronchitis.Contraindication: 1) decompensated cor pulmonale; 2) renal amyloidosis with renal failure.

Bronchiectasis – what is it?

According to medical terminology, bronchiectasis is a pathological expansion of the lumen of the bronchi due to the destruction or violation of the tone of their walls. They underlie the development of bronchiectasis (EBD), which is manifested by a suppurative process in the dilated bronchi.

Reasons

Until now, it has not yet been possible to establish the exact cause of the pathologically dilated bronchi.However, the most important predisposing factors can be identified:

  • Insufficient development of the bronchi as a whole or their individual structures. Genetically defective walls of the bronchial tree cannot effectively cope with their function and are more likely to undergo pathological changes.
  • Frequent infectious and inflammatory diseases of the upper and lower respiratory tract.
  • Congenital malformations of the respiratory system.

Children with chronic respiratory diseases are prone to bronchiectasis.It is also often observed in patients with congenital immunodeficiencies and defects in the bronchial tree. If during the period of gestation a woman smokes, drinks alcohol or has suffered any viral infections, then this significantly increases the risk of congenital bronchiectasis.

Most often bronchiectasis is diagnosed between the ages of 5 and 20 years.

Varieties

A prolonged pathological process in the lower respiratory tract, taking place in several successive stages (from catarrhal inflammation to deforming chronic bronchitis), leads to the formation of bronchiectasis.At the same time, the appearance of secondary bronchiectasis can provoke a lung abscess, tuberculous cavity, pneumonia, foreign bodies, etc.

By shape, saccular, fusiform, cystic, mixed and cylindrical bronchiectasis are distinguished. It has been clinically established that primary bronchiectasis is mainly represented by saccular (saccular) bronchial dilations, while secondary bronchiectasis are cylindrical.

Clinical picture

As a rule, bronchiectasis begins to make itself felt in early childhood.The appearance of this pathology can be associated with a previous pneumonia or a cold infection. The main clinical symptoms characteristic of bronchiectasis:

  • There is a productive cough, during which a lot of purulent sputum is released. The patient coughs up phlegm easily. Its greatest amount is observed in the morning hours. The volume of sputum is influenced by the shape and size of pathological foci. Most of the pus accumulates in cylindrical bronchiectasis.In one day, the patient can excrete from 10 to 400 ml of sputum. During remission, the cough usually becomes less productive.
  • Approximately every fourth case complains of hemoptysis. Excretion of blood with sputum is more pronounced during an exacerbation and during intense physical exertion. It should be noted that in the absence of a suppurative process in some patients, this is the only symptom of the disease.
  • Approximately 1/3 of patients experience shortness of breath. Usually fixed during physical exertion.The faster the disease progresses, the more pronounced it is.
  • Soreness in the chest area may be troubling. As a rule, the pain increases with inspiration.
  • In the phase of exacerbation of the disease, the temperature rises. It rarely comes to high numbers. Basically, subfebrile condition is determined (up to 38 ° C). During remission, temperature normalization is observed.
  • The general condition is deteriorating. Weakness, headaches, fatigue appear.

Chronic bronchitis with bronchiectasis is often diagnosed.

Features of the flow

The severity of clinical signs and symptoms of bronchiectasis will determine the severity of the course:

  • Mild form. There are only a few exacerbations throughout the year. Remissions are long enough, during which the patient feels practically healthy. The ability to work is not impaired.
  • The moderate form is characterized by more frequent and prolonged periods of exacerbation.The volume of sputum secreted per day is approximately 100 ml. The function of external respiration is moderately impaired. Physical endurance and performance are somewhat reduced.
  • Severe form. Exacerbations occur quite often. There are only short-term remissions after prolonged therapy. The condition of the patients is extremely difficult. Disabled even during remission.
  • Complicated form. All clinical symptoms of a severe form are present, to which various complications are also added.Serious disorders of the heart and kidneys are revealed, amyloidosis develops, etc.

Traction bronchiectasis is an irreversible expansion of the bronchi in the area of ​​pulmonary fibrosis.

Diagnostics

For the diagnosis of bronchiectasis, it is necessary to use instrumental research methods. The program of examination of a patient with suspected bronchiectasis includes the following diagnostic methods:

  • Radiography.
  • Bronchography.
  • Bronchoscopy.
  • Angiopulmonography.
  • Bronchial arteriography.
  • Spirography.

X-ray examination reveals pathological disorders that may indicate the presence of bronchiectasis. The main method by which the diagnosis of bronchiectasis is confirmed is bronchography. Thanks to it, it is possible not only to detect bronchiectasis, but also to determine the shape and size of pathological bronchial dilatations.Bronchoscopy plays an important role in the diagnosis of bronchiectasis.

The endoscopic method is able to identify the suppurative process in the bronchi and assess its extent. The condition of the pulmonary blood vessels is determined using angiopulmonography and bronchial arteriography. Spirography helps to assess the function of external respiration, which is often impaired with long-term bronchiectasis.

Bronchography makes it possible to determine the shape of bronchiectasis (cylindrical, saccular, fusiform or mixed).

Treatment

In accordance with standard clinical practice, treatment of bronchiectasis should be comprehensive and consistent. Today, in most cases, the following treatment program is adhered to:

  • The use of antibacterial drugs.
  • Reorganization of the upper and lower respiratory tract.
  • Activation of detoxification therapy.
  • Appointment of physiotherapy treatments.
  • Surgical intervention.

Adequate treatment of bronchiectasis is selected by the attending physician, taking into account the nature and severity of the disease, as well as the age and condition of the patient.

Conservative therapy

In the period of exacerbations, the use of antibiotic therapy is indicated. Preference is given to intrabronchial administration of drugs. However, in some cases, intramuscular and intravenous routes of administration are also not neglected.It is recommended to carry out antibiotic therapy after receiving the results of bacteriological analysis of sputum, when the pathogen and its sensitivity to antimicrobial drugs have been identified. Currently, antibacterial drugs with a broad spectrum of action are prescribed:

  • Semisynthetic forms of penicillins.
  • New generation cephalosporins.
  • Aminoglycosides.
  • Tetracyclines.

One of the most important therapeutic measures is the sanitation of the respiratory tract, which allows you to remove purulent sputum from the bronchi.Washing is carried out during bronchoscopy using antiseptic solutions (Furacilin, Dioxidin, etc.). The use of postural drainage several times a day also helps to remove phlegm from the bronchi. If necessary, use mucolytic and expectorant drugs (Mukaltin, Acetylcysteine).

Ingestion of liquid in the form of tea, juice, fruit drink helps to relieve symptoms of intoxication. The volume of fluid is recommended depending on the age and weight of the patient. In difficult situations, intravenous administration of special solutions (glucose, sodium chloride, etc.) may be required as detoxification therapy.).

Physical therapy and breathing exercises have a good therapeutic effect. In the absence of contraindications, they are recommended to be performed on a regular basis. To improve the drainage function of the lungs and the discharge of sputum, massage sessions are prescribed for the chest area. Outside the stage of exacerbation, physiotherapy procedures are prescribed. A positive result is noted from the use of microwave therapy, drug electrophoresis, inductothermy and other procedures.If possible, it is advisable to use spa treatment in institutions specializing in the pathology of the respiratory system.

Surgery

In some cases it is necessary to resort to surgical intervention. Surgical treatment of bronchiectasis is indicated in the following situations:

  • The presence of limited unilateral bronchiectasis without severe bronchial obstruction and in the absence of the effect of conservative therapy.
  • Pathological conditions that threaten the patient’s life (for example, pulmonary hemorrhage).

Pulmonary emphysema, severe pulmonary and heart failure, amyloid kidney damage with serious impairment of their function are considered contraindications to the operation.

Self-treatment of bronchiectasis in the lungs often does not bring positive results, but only worsens the patient’s current condition.

Prevention

Due to the peculiarities of the development of bronchiectasis, it is unlikely to completely prevent the development of this pathology, since it is based on a genetically determined inferiority of the bronchial wall.