Treatment of bronchiectasis in adults

Summary points

• Bronchiectasis refers to abnormal bronchial dilatation caused by a vicious cycle of transmural infection and inflammation

• Symptoms include chronic productive cough, wheeze, and dyspnoea; repeated respiratory infections may dominate the clinical picture

• Diagnosis is based on daily production of mucopurulent phlegm and dilated and thickened airways on computed tomography

• Diagnosis should lead to investigation and treatment of possible causes and associated conditions

• Acute exacerbations should be treated promptly with short courses of antibiotics

• Frequent exacerbations may be treated with prolonged and aerosolised antibiotics

• The role of mucolytics, anti-inflammatory agents, and bronchodilators is not clear

• Surgery is a possibility if the area of bronchiectasis is localised and symptoms are debilitating or life threatening

Patients with bronchiectasis usually need lifelong medical support from their doctor, especially given the frequent episodes of infection. This article focuses on the treatment of bronchiectasis in adults and does not include a discussion of bronchiectasis caused by cystic fibrosis. The prevalence of bronchiectasis not linked to cystic fibrosis is unclear, but every general practitioner in the United Kingdom probably has a few patients.

How do we diagnose bronchiectasis?

Bronchiectasis refers to the permanent abnormal dilatation of the central and medium sized bronchi as a result of a vicious cycle of transmural infection and inflammation with mediator release.¹ Symptoms include chronic productive cough, wheeze, and dyspnoea. Infective exacerbations are associated with worsening of symptoms and signs of pneumonia. Haemoptysis can occur, but amounts of blood are usually small, and serious haemoptysis requiring selective arteriography and embolisation or surgery is rare.

The most frequently used classification system distinguishes between cylindrical, varicose, and saccular or cystic bronchiectasis. ^w1 Although insightful, this classification has no clinical or therapeutic uses. A modern clinical definition includes the daily production of mucopurulent phlegm and chest imaging that demonstrates dilated and thickened airways.^w2 The clinical suspicion of bronchiectasis can be confirmed by high resolution computed tomography. Characteristic findings include internal bronchial diameters greater than that of the adjacent pulmonary artery, lack of bronchial tapering, presence of bronchi within 1 cm of the costal pleura, presence of bronchi abutting the mediastinal pleura, and bronchial wall thickening.^w3

A diagnosis of bronchiectasis should prompt an investigation of possible causes and associated conditions (table 1), some of which can be treated.² Extrinsic factors, particularly childhood respiratory infections, were an important cause of permanent bronchial damage in the past. These days, especially in Western countries with early immunisation and widespread use of antibiotics, post-infectious damage is a less prominent cause of the disease, and intrinsic defects are more common causes. A study of 150 adults with bronchiectasis in the UK found that 53% of cases were idiopathic; 29% were post-infectious; 8% were caused by an immune defect, 7% by allergic bronchopulmonary aspergillosis, 4% by aspiration, 3% by Young’s syndrome, 3% by cystic fibrosis, 3% by rheumatoid arthritis, 1.5% by ciliary dysfunction, and <1% by miscellaneous causes.³

Table 1

Causes of bronchiectasis and associated conditions

How is it treated?

Bronchiectasis can be treated by pharmacological and non-pharmacological means (tables 2 and 3). ^w4 The box shows an arbitrary step-up scheme that takes into account the level of evidence and safety risks.

Table 2

Pharmacological and non-pharmacological treatments for stable bronchiectasis

Table 3

 Revised grading system for levels of evidence and recommendations in evidence based guidelines

Suggested step-up scheme for treating bronchiectasis

• Step 1: Inhaled fluticasone (500 µg twice daily)

• Step 2: Physical therapy including exercise training

• Step 3: Prolonged use of oral antibiotics. The choice of antibiotic should depend on sputum culture results, with a preference for clarithromycin

Antibiotics

Antibiotics are used to treat acute exacerbations, to prevent exacerbations, or to reduce the bacterial burden. In general, the outcome of treatment with antibiotics depends on the severity of the disease. In mild to moderate bronchiectasis the infection can be completely eradicated, whereas in severe disease the bronchial tree remains chronically colonised. Antibiotics with a high penetrance (macrolides, azalides, and quinolones) are recommended in severe cases because high concentrations of bacteria are located intraluminally in association with mucus, and because thickening and scarring of the bronchial wall may reduce local bioavailability. Pseudomonas aeruginosa is inherently resistant to most antibiotics at concentrations that are achieved in vivo. The organism is susceptible to (oral) quinolones,² but after one or two courses resistance may develop. Patients who are clinically unwell or do not respond to oral antibiotics should be admitted to hospital and given intravenous antibiotics.

Patients who relapse quickly might need prophylactic antibiotics. Three strategies have been described—a high oral dose for a prolonged period (at least four weeks), aerosolised antibiotics (for example, during alternate months), or regular pulsed courses of intravenous antibiotics (for example, two to three week courses with one or two months in between). Prolonged use of oral antibiotics for purulent bronchiectasis was investigated in a recent meta-analysis. It identified six trials that included 302 patients.⁵ The results of five studies were positive in terms of parameters pertaining to sputum, such as volume or purulence. However, no positive effects were seen on rates of exacerbation, lung function, or death. This contradicts the findings of a recent controlled study that investigated 500 mg of azithromycin given twice a week for six months in 30 patients. ⁶ Pulmonary function tests did not change, but azithromycin significantly decreased the incidence of exacerbations compared with usual care (5 v 16). Inhalation of nebulised antibiotics might be a better route of administration because of its favourable benefit-risk ratio. Gentamicin 40 mg inhaled twice daily for three days improved sputum production, sputum neutrophil activity, airway obstruction, exercise capacity, and nocturnal desaturation compared with placebo.⁷ Tobramycin 300 mg inhaled twice daily for four weeks eradicated P auruginosa in 35% of patients and improved the medical condition of 62%.⁸ Ceftazidime 1 g and tobramycin 100 mg inhaled twice daily over 12 months decreased the number of hospital admissions and inpatient days.⁹

Flu vaccinations reduced the number of flu related exacerbations in chronic obstructive pulmonary disease,^w5 so vaccination might have a beneficial effect in bronchiectasis. However, randomised controlled trials of vaccination in patients with bronchiectasis are lacking.

Mucolytics

Mucolytics target hypersecretion or the physiochemical characteristics of sputum seen in bronchiectasis. They aim to improve tracheobronchial clearance. In one study, oral bromhexine (30 mg three times daily) was added to an antibiotic during acute infective exacerbations. It improved expectoration, the quantity and quality of sputum, and auscultatory findings.¹⁰ In another study, nebulised recombinant human DNase I (2.5 mg once or twice a day for two weeks) was given to patients with stable bronchiectasis. The study found no improvements in spirometry, quality of life, dyspnoea, safety, or ciliary transportability of the sputum.¹¹ In a large placebo controlled study, 349 patients with idiopathic bronchiectasis in a stable condition inhaled 2.5 mg recombinant human DNase I for 24 weeks. Pulmonary exacerbations were more frequent and the decline in FEV₁ (forced expiratory volume in one second) was greater in patients who received DNase than in controls. These results contrast greatly with the positive effects of this drug in cystic fibrosis.¹²

Inhaled mannitol may improve impaired mucociliary clearance by inducing an influx of fluid into the airways, and thereby changing the mucous rheology. One study found that mucociliary clearance was doubled in the central and intermediate lung regions of 11 patients with bronchiectasis directly after inhaling 300 mg mannitol.^w6 A follow-up study showed that these effects lasted for at least 24 hours.^w7 Inhalation of 400 mg mannitol once daily for 12 days improved the tenacity and hydration of sputum, as well as health status.^w8 Mannitol is therefore a promising new drug, especially as it is easier and more hygienic to inhale a dry powder than to use a nebuliser. Large randomised controlled studies are needed, however, to establish its efficacy and tolerability during long term administration.¹³

Anti-inflammatory agents

Inhaled corticosteroids may reduce inflammation and improve airway obstruction, as they do in asthma. To date, three randomised controlled studies have investigated this hypothesis.^w9-w11 Inhaled fluticasone decreased the density of leucocytes and concentrations of the inflammatory mediators interleukin 1β, interleukin 8, and leukotriene B4 in sputum.^w11 However, a systematic review found no significant improvements in lung function.¹⁴ Recently, a large study was published in which 86 patients inhaled fluticasone 500 µg twice daily or placebo for 12 months.¹⁵ Twenty four hour sputum volume improved significantly in patients treated with fluticasone, but no change was seen in the frequency of exacerbations, the purulence of sputum, or lung function. Systemic corticosteroids may be better at penetrating the bronchial wall and therefore be more effective, but data from randomised controlled trials are available only for bronchiectasis associated with cystic fibrosis.¹⁶

Leukotriene receptor antagonists are a new class of drugs that is effective in the treatment of asthma. These drugs may be of benefit in bronchiectasis because they inhibit neutrophil mediated inflammation. To date, no randomised controlled trials of these agents have been published, however.¹⁷

Non-steroidal anti-inflammatory drugs may also be of use because they inhibit neutrophil function and the release of neutrophil elastase. In a non-controlled open study, eight patients with stable bronchiectasis received 25 mg oral indometacin three times daily for four weeks.^w12 Indometacin had a pronounced effect on two of the three neutrophil functions studied—neutrophil chemotaxis and fibronectin degradation were inhibited by more than 50%. However, lung inflammation, sputum volume, and sputum quality did not change. Interestingly, high dose ibuprofen taken consistently for four years significantly slowed the progression of lung disease in patients with cystic fibrosis.^w13 Ibuprofen has not yet been investigated in non-cystic fibrosis bronchiectasis.

Macrolides suppress inflammation—independent of their antimicrobial actions—in airway diseases like asthma, chronic obstructive pulmonary disease, cystic fibrosis, and diffuse panbronchiolitis. Indeed, macrolides improved clinical status and lung function in a few small studies of bronchiectasis.^w14-w18 In addition, a randomised placebo controlled trial in children showed that three months of treatment with clarithromycin decreased the total number of leucocytes, proportion of neutrophils, and the concentration of interleukin 8 in bronchoalveolar lavage fluid.^w18 Because bacterial growth was not eliminated the authors suggested that direct anti-inflammatory effects of clarithromycin were responsible. This contradicts a randomised placebo controlled study in adults, however, which showed that eight weeks of low dose erythromycin did not reduce the number of leucocytes or the concentrations of interleukin 1α, interleukin 8, tumour necrosis factor α, or leukotriene B4 in sputum.^w15

Bronchodilators

The mechanisms of expiratory airflow obstruction in non-cystic fibrosis bronchiectasis are not clear, but may include excessive production of mucus, distortion of the bronchial wall, and constriction of smooth muscle. Increased bronchial hyper-reactivity and some reversibility of the airflow obstruction with an inhaled bronchodilator are common. As bronchiectasis may coexist with asthma or chronic obstructive pulmonary disease, some studies have a high degree of uncertainty as to whether airway obstruction is due to asthma, chronic obstructive pulmonary disease, or bronchiectasis (or a combination). Nevertheless, as many patients show signs of airway obstruction and hyper-responsiveness, they often receive bronchodilators.

One small Malaysian study gives some information about the reversibility of airway obstruction in bronchiectasis. It included 24 patients with confirmed bronchiectasis but no signs of an acute exacerbation. The study compared 400 µg fenoterol, followed by 5 mg fenoterol 30 minutes later, with 40 µg ipratropium, followed by 500 µg ipratropium.^w19 FEV₁ increased more than 15% in response to one or both bronchodilators in 11 patients—five responded to both, three to fenoterol alone, and three to ipratropium alone. This small study suggests a significant response to bronchodilators in a subset of patients.

Cochrane reviews found no randomised controlled trials on short acting β2 adrenergic agonists,¹⁸ long acting β2 adrenergic agonists,¹⁹ anticholinergic therapy,²⁰ or oral methylxanthines²¹ in patients with non-cystic fibrosis bronchiectasis. Good quality studies are therefore urgently needed in this field.

Bronchopulmonary hygiene physical therapy

Bronchopulmonary hygiene physical therapy is a form of chest physical therapy that aims to remove lung secretions in patients with acute and chronic airway diseases. Many active and passive techniques are available (table 2), and the technique chosen varies between institutes and physical therapists.^{w20 w21} The evidence in support of these techniques is variable and the literature is conflicting.^w20 Two systematic reviews found insufficient evidence to support or refute this form of therapy. ²²²³

Exercise training

The role of pulmonary rehabilitation and inspiratory muscle training has been investigated in only one randomised controlled trial. It compared an eight week training programme of pulmonary rehabilitation alone, pulmonary rehabilitation plus inspiratory muscle training, and a control group.^w22 The authors concluded that pulmonary rehabilitation does improve exercise tolerance. Simultaneous inspiratory muscle training offered no additional benefit.

Surgery

If the area of bronchiectasis is localised and the patient’s symptoms are debilitating or life threatening, surgical resection has long been thought to be of benefit. This assumption was based on several non-randomised controlled studies of uncertain value. A Cochrane review updated in 2002 found no randomised clinical trials—just case series or case-control studies.²⁴ The largest case-control study included pneumonectomy in 190 cases, lobectomy in 202 cases, bilobectomy in 23 cases, and lobectomy combined with segmental resection in 72 cases. ²⁵ Overall mortality was 3.5%, and 71% of patients had no symptoms during follow-up (four months to 10 years). The authors therefore concluded that surgery was preferable to conventional medical treatment.

Diagnostic imaging in adult non-cystic fibrosis bronchiectasis

Breathe (Sheff). 2019 Sep; 15(3): 190–197.

Gunnar Juliusson

¹Dept of Radiology, Landspitali University Hospital, Reykjavik, Iceland

Gunnar Gudmundsson

²Dept of Respiratory Medicine, Landspitali University Hospital, Reykjavik, Iceland

³Faculty of Medicine, University of Iceland, Reykjavik, Iceland

¹Dept of Radiology, Landspitali University Hospital, Reykjavik, Iceland

²Dept of Respiratory Medicine, Landspitali University Hospital, Reykjavik, Iceland

³Faculty of Medicine, University of Iceland, Reykjavik, Iceland

Breathe articles are open access and distributed under the terms of the Creative Commons Attribution Non-Commercial Licence 4. 0.

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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.

Short abstract

Bronchiectasis is a chronic progressive condition with significant disease burden and frequent exacerbations for which the diagnosis relies on cross-sectional imaging
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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 . Bronchial dilatation is the hallmark of bronchiectasis and is evaluated in relation to the accompanying pulmonary artery (). 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 . 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 ().

Table 1

Diagnostic imaging findings in bronchiectasis

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

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 () [7]. Visible airways in the 1-cm vicinity of costal subpleural lung are another useful sign of bronchiectasis (). 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 ( and ). 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 (). 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 (), post-tuberculous scarring () and post-radiation fibrosis (). 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 (). 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 (). Lower lobe distribution is most often seen in post-infectious bronchiectasis, chronic aspiration (), immunodeficiencies () and primary ciliary dyskinesia (). Allergic bronchopulmonary aspergillosis (ABPA) bears mention due to the characteristic imaging appearance and need for specific medical management (). 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].

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 (#).

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.

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.

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.

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 (). Massive haemoptysis is due to bleeding from the bronchial arteries in the majority of cases () and less commonly from pulmonary arteries or non-bronchial systemic arteries [14].

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).

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Bronchiectasis: Practice Essentials, Background, Pathophysiology

Bronchiectasis; Causes, Symptoms, Treatment & Prevention

Overview

Normal versus damaged bronchus with bronchiectasis.

What is bronchiectasis?

Bronchiectasis is a lung condition that causes coughing up of mucus. It is (pronounced brong-kee-ek-tuh-sis). In the lungs, the bronchi are the passages that allow air to enter the lungs. In bronchiectasis, the inside surfaces of the bronchi get thicker over time from inflammation that leave scars. Thicker walls cause mucus to collect in these passages because the walls are not strong enough to make the mucus move out of the lungs. In addition, the cilia (thin strands that look like hair and that help move mucus) are destroyed. When that happens, infections can happen more easily and breathing becomes difficult. Times that breathing or coughing gets worse are called exacerbations.

How common is bronchiectasis?

It is estimated that there are 500,000 individuals in the United States with bronchiectasis and one person out of 150 people aged 75 years old and older has bronchiectasis. The risk of getting this condition increases with age. However, younger people can and do get bronchiectasis. In adults, it is much more common in women than in men. In children, it is more common in boys than girls. It is possible, but rare, for children to be born with bronchiectasis.

What are risk factors for bronchiectasis?

People with the following conditions are considered to have risk factors for bronchiectasis:

• Cystic fibrosis
• Chronic and inflammatory lung disease
• Chronic or severe lung infections, like tuberculosis, non-tuberculous mycobacteria (NTM) or pneumonia
• Deficits in the immune system
• Repeated aspiration (breathing in) of things other than air, such as food particles, that cause damage to the lungs

Symptoms and Causes

What causes bronchiectasis?

Bronchiectasis may be caused by cystic fibrosis (CF), a genetic condition that results in long-lasting lung infections and reduced ability to breathe. CF gets worse over time.

For non-CF bronchiectasis, the cause is not always known. This is called idiopathic bronchiectasis. However, in other cases, causes include:

• Past severe infection that has damaged the lung
• Genetic diseases like primary ciliary dyskinesia or alpha-1 antitrypsin deficiency
• Immune system conditions that make it difficult to fight off infections
• Aspirating (breathing in) things like fluids, stomach acid, or foods into the lungs
• Allergic bronchopulmonary aspergillosis, an allergy to a particular type of fungus
• Other conditions like rheumatoid arthritis, Crohn’s disease, and Sjogren’s syndrome
• Obstructed airways (airways blocked by something like a tumor or an inhaled object)

What are the symptoms of bronchiectasis?

Some of the most common symptoms are:

• Coughing that results in a lot of mucus
• Coughing up mucus that has blood in it (known as hemoptysis)
• Chest pain or tightness because it is harder to breathe
• Wheezing or making whistling noises when breathing
• Clubbing of nails
• Loss of weight
• Flare-ups that usually include:

Diagnosis and Tests

How will a doctor diagnose bronchiectasis?

Bronchiectasis is believed to be underdiagnosed. If your symptoms lead your doctor to suspect this disease, the following tests are likely to be ordered:

• Chest CT scan or X-ray, imaging tests to show the state of the lungs.
• Blood tests and sputum cultures to find out if there are infections.
• Lung function tests to determine how well you are breathing/how well your lungs are working.
• Bronchoscopy, which is a way to see inside the lungs. The method uses a light and a camera on a tube inserted through the nose or mouth and then down the trachea into the lungs. In the case of blockages, a bronchoscopy can also find and remove the object blocking the airways. The procedure can also be used to obtain lung secretions.

Management and Treatment

What are the treatments for bronchiectasis?

Bronchiectasis cannot be cured, but it can be managed. Your doctor might prescribe medication, the use of certain devices, or a combination of these methods and possibly others.

If bronchiectasis is caused by an underlying condition, like aspergillosis or an immune system disease, then that condition must be treated first.

Treatment choices might include medications, such as:

• Antibiotics, drugs used to treat bacterial infections. These include oral (pill) forms and inhaled forms (breathed in using a nebulizer machine).
• Macrolides, drugs used to treat infections and inflammations at the same time.
• Drugs that affect mucus and help you cough the mucus out.

Treatment might also include:

• Airway clearance devices to break up mucus and help you cough it out
  • Positive expiratory pressure (PEP) devices that you can hold in your hands
  • Percussive devices like vests you can wear
  • Physical therapy moves like chest clapping to help move mucus out of the body

In addition, bronchiectasis treatment is broken down further by what stage you are in: there are things that you do daily (maintenance) and things that you do when you are having an exacerbation (worsening).

Prevention

How can you prevent bronchiectasis?

There is no way to prevent congenital bronchiectasis (the kind you are born with). However, there are ways to help you avoid developing the lung damage that leads to bronchiectasis.

• Make sure you vaccinate your children against diseases such as measles and whooping cough.
• If you or your child develop any kind of lung infection, get medical treatment.
• Be aware of the risks of breathing in any object. If your child or any adult breathes in an object, get immediate medical help.
• Stay away from airborne substances that can hurt your lungs like smoke, fumes and gases.

Outlook / Prognosis

What is the prognosis/outlook for patients who have bronchiectasis?

The outlook for people with bronchiectasis is better now than it used to be. People who are treated live relatively normal lifespans. The outlook is also based on the person’s age, general health, how many exacerbations happen during a specified period of time, and how bad the exacerbations are.

Living With

What are some tips for living well with bronchiectasis?

• Do not smoke. If you do smoke, ask your doctor for help in stopping.
• It is important to follow the daily maintenance therapy that your doctor recommends.
• Eat a healthy diet.
• Drink plenty of water to stay hydrated and to keep mucus from getting sticky.
• Follow a regular exercise routine.
• Make sure you get a flu shot every year and the pneumonia vaccine as directed by your doctor.

When should I see a healthcare provider about bronchiectasis?

If at any time you find yourself coughing a lot, coughing up mucus frequently, coughing up blood, or having trouble breathing, you should consult a healthcare provider.

If you have been diagnosed with bronchiectasis, you should contact your healthcare provider if:

• You have signs of infection, like a fever or chills
• You have more trouble breathing than usual
• You have pain in your chest
• You are much more tired than usual
• You are losing weight unintentionally
• You are coughing up more mucus, mucus with blood in it, or mucus that is yellow or green
• You do not have an appetite

Resources

Are there organizations that support people who have bronchiectasis and their caregivers?

You might find the following organizations to be helpful:

Bronchiectasis – NHS

Bronchiectasis is a long-term condition where the airways of the lungs become widened, leading to a build-up of excess mucus that can make the lungs more vulnerable to infection.

The most common symptoms of bronchiectasis include:

The severity of symptoms can vary widely. Some people have only a few symptoms that do not appear often, while others have wide-ranging daily symptoms.

The symptoms tend to get worse if you develop an infection in your lungs.

Read more about the symptoms of bronchiectasis.

Information:

Coronavirus advice

If you have bronchiectasis and you’re worried about coronavirus, you can get advice about coronavirus and bronchiectasis from the British Lung Foundation.

When to see a GP

You should see a GP if you develop a persistent cough. While this may not be caused by bronchiectasis, it requires further investigation.

If the GP suspects you may have bronchiectasis, they’ll refer you to a specialist in treating lung conditions (a respiratory consultant) for further tests.

Read more about diagnosing bronchiectasis.

How the lungs are affected

The lungs are full of tiny branching airways known as bronchi. Oxygen travels through these airways, ends up in tiny sacs called alveoli, and from there is absorbed into the bloodstream.

The inside walls of the bronchi are coated with sticky mucus, which protects against damage from particles moving down into the lungs.

In bronchiectasis, one or more of the bronchi are abnormally widened. This means more mucus than usual gathers there, which makes the bronchi more vulnerable to infection.

If an infection does develop, the bronchi may be damaged again, so even more mucus gathers in them and the risk of infection increases.

Over time, this cycle can cause gradually worsening damage to the lungs.

Why it happens

Bronchiectasis can develop if the tissue and muscles that surround the bronchi are damaged or destroyed. 

There are many reasons why this may happen. The 3 most common causes in the UK are:

• having had a lung infection in the past, such as pneumonia or whooping cough, that damages the bronchi
• underlying problems with the immune system (the body’s defence against infection) that make the bronchi more vulnerable to damage from an infection
• aspergillosis – an allergy to a certain type of fungi that can cause the bronchi to become inflamed if spores from the fungi are inhaled

But in many cases, no obvious cause for the condition can be found. This is known as idiopathic bronchiectasis.

Read more about the causes of bronchiectasis.

Who is affected

Bronchiectasis is thought to be uncommon. It’s estimated around 1 in every 100 adults in the UK have the condition.

It can affect anyone at any age, but symptoms do not usually develop until middle age.

How bronchiectasis is treated

The damage caused to the lungs by bronchiectasis is permanent, but treatment can help relieve symptoms and stop the damage getting worse.

The main treatments include:

• exercises and special devices to help you clear mucus out of your lungs
• medicine to help improve airflow within the lungs
• antibiotics to treat any lung infections that develop

Surgery is usually only considered for bronchiectasis in rare cases where other treatments have not been effective, the damage to your bronchi is confined to a small area, and you’re in good general health.

Read more about the treatment of bronchiectasis.

Possible complications

Complications of bronchiectasis are rare, but they can be serious.

One of the most serious complications is coughing up large amounts of blood, caused by one of the blood vessels in the lungs splitting.

This can be life-threatening and may require emergency surgery to treat it.

Read more about the complications of bronchiectasis.

Outlook

The outlook for people with bronchiectasis is highly variable and often depends on the underlying cause.

Living with bronchiectasis can be stressful and frustrating, but most people with the condition have a normal life expectancy.

For people with very severe symptoms, however, bronchiectasis can be fatal if the lungs stop working properly.

Around 1,500 deaths reported in UK each year are thought to be caused by bronchiectasis.

Information:

Social care and support guide

If you:

• need help with day-to-day living because of illness or disability
• care for someone regularly because they’re ill, elderly or disabled – including family members

Our guide to care and support explains your options and where you can get support.

Page last reviewed: 27 July 2021
Next review due: 27 July 2024

Bronchiectasis – an overview | ScienceDirect Topics

DIAGNOSTIC METHODS

Bronchiectasis should be considered in all children with chronic respiratory symptoms, particularly if associated with a history of a severe respiratory infection in the past, digital clubbing, and malnutrition.

HRCT of the chest has largely replaced bronchography for the diagnosis of bronchiectasis. It can accurately localize lesions, identify mucus plugging and bronchiolar abnormalities to the level of sixth-order bronchi and also identify focal areas of air trapping secondary to small airway disease.^11,21 Occasionally, bronchography is performed as a presurgical evaluation and when the diagnosis is uncertain.²⁴ Bronchography is associated with a risk of allergic reactions to the contrast media and with the possibility of ventilatory failure in patients who already have some degree of respiratory insufficiency. Most surgical series currently published do not mention bronchography as a mandatory preoperative test.²⁵

Ventilation-perfusion scintigraphy can be used to evaluate vascular perfusion and the extent of gas exchange in different areas.²⁶

Chest radiographs can be indicative of bronchiectasis, but they are not very sensitive. In the series by Eastham and coworkers,²² 66% of the HRCT diagnosed cases would have been missed by chest radiography alone; HRCT should be performed when the clinical picture is compatible, despite a normal chest radiograph. Usual radiographic findings are increased linear markings, the so-called tram lines, crowding of bronchi, cystic spaces, air-fluid levels, and honey-combing.^4,27

The standard HRCT technique is a thin section (1.0- to 1.5-mm collimation), at 10-mm intervals, with a high-frequency reconstruction algorithm. Usual window levels are -600 to -700HU, and window width is 1000 to 1500HU.^27,28

One of the classic signs of bronchiectasis in HRCT is a bronchial/adjacent pulmonary artery ratio greater than 1, which corresponds to bronchial dilatation; this is also known as the “signet ring” sign. It has been suggested that a 1.5 ratio should be used instead, because healthy people can have ratios as great as 1.5. Other classic signs include parallel bronchial walls that do not taper, the tram line appearance, or demonstration of bronchi in the peripheral third of the lung, mainly within 1 cm of the pleura.^4,11,28,29 In the retrospective series of bronchiectasis by Kang and coworkers,²⁸ 87% of 47 resected lobes from adolescents and adults were correctly identified by the presurgical HRCT scans. Because all the patients had clinical indication for surgery, they probably represent the more symptomatic children.

Areas of decreased attenuation can often be seen in HRCTs of patients with bronchiectasis. Pifferi and coworkers were able to identify attenuations in 37% of pulmonary lobes from 16 patients with bronchiectasis.²⁹ These areas correlated well with ventilatory and perfusional scintigraphy findings but did not necessarily correspond to the same areas where bronchiectatic changes could be observed. These changes could represent hemodynamic changes surrounding bronchiectasis.

Pathologically, bronchiectasis consists of dilated bronchi, with inflammation and fibrous distortion of the bronchial wall, but biopsy specimens are not usually required, because image studies usually suffice for the diagnosis.²⁸

Interestingly, many cases of HRCT-diagnosed bronchiectasis have been demonstrated to be reversible. Either more strict criteria should be used to interpret HRCT in childhood, or some mild cases may actually be reversible. Gaillard and coworkers suggest that the persistence of clinical and CT findings for a period of 2 years is required to confirm the presence of true bronchiectasis.^1,22,30

Although some diseases can show a more frequent pattern of distribution of bronchiectatic lesions, none of the HRCT findings should be considered diagnostic, because there is great overlap.^4,11 In CF, upper lobe involvement is frequent, in contrast with other diseases where bronchiectasis tends to be more evident at other sites, especially in the lower lobes.^7,11,25 Aspiration typically causes more lower lobe bronchiectasis. In the series of Li and coworkers,¹¹ 80% of such patients had bronchiectasis in the lower lobes. According to Hansel,⁴ the middle lobe was more commonly involved in hypogammaglobulinemia. Li and coworkers¹¹ found a preponderance of lower and right middle lobe involvement in children with various types of immunodeficiency. Typical cases with an underlying cause such as CF, primary ciliary dyskinesia, or immunodeficiency usually show a multilobar involvement, in contrast to localized disease caused by infection.⁵

Bronchiectasis: a case-based approach to investigation and management

Abstract

Bronchiectasis is a chronic respiratory disease characterised by a syndrome of productive cough and recurrent respiratory infections due to permanent dilatation of the bronchi. Bronchiectasis represents the final common pathway of different disorders, some of which may require specific treatment. Therefore, promptly identifying the aetiology of bronchiectasis is recommended by the European Respiratory Society guidelines. The clinical history and high-resolution computed tomography (HRCT) features can be useful to detect the underlying causes. Despite a strong focus on this aspect of treatment a high proportion of patients remain classified as “idiopathic”. Important underlying conditions that are treatable are frequently not identified for prolonged periods of time.

The European Respiratory Society guidelines for bronchiectasis recommend a minimal bundle of tests for diagnosing the cause of bronchiectasis, consisting of immunoglobulins, testing for allergic bronchopulmonary aspergillosis and full blood count. Other testing is recommended to be conducted based on the clinical history, radiological features and severity of disease. Therefore it is essential to teach clinicians how to recognise the “clinical phenotypes” of bronchiectasis that require specific testing.

This article will present the initial investigation and management of bronchiectasis focussing particularly on the HRCT features and clinical features that allow recognition of specific causes.

Abstract

Bronchiectasis is a heterogeneous disease with diverse clinical presentation. Careful history, review of radiological features and laboratory testing are required to identify the underlying diagnosis. http://ow.ly/RDF730koTxu

Introduction

Bronchiectasis is a progressive respiratory disease characterised by permanent dilatation of the bronchi and associated with a clinical syndrome of cough, sputum production and recurrent respiratory infections [1]. The causes of bronchiectasis are varied with important differences between the presentation and natural history of the disease depending on aetiology.

Bronchiectasis is increasing in prevalence with current rates estimated between 53 and 566 cases per 100 000 inhabitants depending on the population studied [2, 3]. These differences in reported prevalence may be due to the long period of neglect and growing awareness or could represent a true rise in prevalence. It should therefore be expected that cases of bronchiectasis will be encountered more frequently by the general physician, as well as the respiratory specialist. Bronchiectasis is a heterogenous disease with many causes and associations. The most commonly associated conditions are shown in table 1. Although the final clinical syndrome is similar, there are many clinical and radiological features which give clues as to aetiology. The presentation of post-infective bronchiectasis can be very different to the presentation of chronic obstructive pulmonary disease (COPD)-related bronchiectasis and the features of a computed tomography (CT) scan of post-tuberculous bronchiectasis are different to the features seen with nontuberculous mycobacteria (NTM) related disease, for example. Identifying the underlying cause accurately and quickly is a key recommendation of international guidelines, as many causes of bronchiectasis are treatable or have specific prognostic implications (table 1).

TABLE 1

Aetiologies of bronchiectasis

Our understanding of the pathophysiology of bronchiectasis is limited. The so-called “vicious cycle hypothesis” first proposed in 1986 by Cole [4] remains central to our understanding. The key components of the disease are chronic inflammation, impaired mucociliary clearance, chronic bronchial infection and structural lung damage. Chronic airways infection, most frequently with Haemophilus influenzae and Pseudomonas aeruginosa, stimulates and sustains lung neutrophilic inflammation and is related with a higher frequency of exacerbations, worse quality of life and increased mortality [5]. This is particularly the case with P. aeruginosa infection where chronic infection is associated with a three-fold increase in mortality and seven-fold increase in hospitalisation [6].

Recognised aetiologies include post-infection, COPD, primary ciliary dyskinesia (PCD), allergic bronchopulmonary aspergillosis (ABPA), NTM infections, immune deficiencies and connective tissue diseases [7]. However, despite extensive testing, up to 53% of patients may have no identifiable cause and the diagnosis of idiopathic bronchiectasis remains common [8].

The recent European Respiratory Society (ERS) guidelines suggest the following minimum bundle of aetiological tests to perform in adults with a new diagnosis of bronchiectasis: measurement of differential blood count, immunoglobulins (IgA, IgM and IgG) and screening for ABPA (total IgE, specific IgE to Aspergillus, IgG to Aspergillus and eosinophil count). Additional tests may be appropriate in specific clinical features or in patients with severe or rapidly progressive disease. Sputum culture is recommended for monitoring bacterial infections and when NTM infection is suspected [9]. Standardised tests are important to seek causes of underlying bronchiectasis because they lead to a change in treatment in 7–37% of cases [7, 8, 10, 11].

Chest high-resolution computed tomography (HRCT) features can be useful to detect the underlying causes. HRCT is now the accepted standard to establish the diagnosis of bronchiectasis [1]. The prerequisite is the identification of dilation of the airways, seen as an increased ratio between the internal lumen of a bronchus and its immediately adjacent pulmonary artery. The lack of normal tapering, mucus plugging, nodules, bronchial wall thickening, “tree-in-bud” pattern, lung volume loss and mosaicism pattern are all additional features useful to support a diagnosis of bronchiectasis. Furthermore, all these signs can be associated with particular distributions of bronchiectasis and can guide us to a specific cause [12].

As the ERS guidelines recommend only a small number of tests are performed routinely, it is important that clinicians know how to recognise other treatable causes and “phenotypes”, as listed in table 1. This article presents examples of clinical phenotypes, integrating history and HRCT features to illustrate the importance of identifying the underlying cause of bronchiectasis and key components of management.

Case 1

Case 1 is a 42-year-old man. He works as a gardener but has been finding it difficult to maintain his business recently due to recurrent respiratory infections. He has had a long history of respiratory problems starting in early childhood. He thinks he was told that he had asthma and previously used an inhaler but stopped due to it being ineffective. He struggled at school due to frequent absence due to “chest infections”. He is unaware of any neonatal issues but believes that he was born at home without complications and is unsure of any previous tests he has had as he is now estranged from his parents. He believes he has a cousin with a “lung disease”.

He tends to cough most days and has three to four significant chest infections per year. He has often struggled to gain weight. He is married but has not had any children.

His initial screening blood tests did not reveal any abnormalities. His CT scan showed extensive cystic and varicose bronchiectasis affecting all lobes including the upper lobes (figure 1). His sputum culture taken in clinical stability grew P. aeruginosa. No previous cultures are available to determine the duration of this infection. Due to his family history, infertility, severity of disease and young age of onset cystic fibrosis (CF) genetics were performed. His genotype was F508del/R117H, with a sweat chloride of 73 mEq·L⁻¹. A diagnosis of CF was made.

FIGURE 1

Varicose and cystic bronchiectasis with mucus plugging in upper lobes.

Cystic fibrosis

CF is the most common life-threatening autosomal recessive disease in the USA and Europe [13, 14].

CF is a multisystem disorder caused by mutations in the gene that encodes the CF transmembrane conductance regulator (CFTR) protein, a chloride channel expressed in epithelial cells [15]. More than 2000 CFTR mutations have been identified to date, but only the functional importance of a small number is known to cause the disease [16].

Clinical manifestations of CF can be very different between patients according to their genotype and the consequent highly variable levels of CFTR dysfunction [15, 17].

Bronchiectasis associated with cough, sputum production and recurrent respiratory infections are the hallmark of CF lung disease. An upper lobe predominant distribution of cylindrical, cystic and varicose bronchiectasis associated with airway wall thickening, mucus plugging and parenchymal opacities on a HRCT scan should raise the suspicion of CF disease [18]. The presence of nasal polyposis and/or chronic rhinosinusitis, recurrent pancreatitis, malabsorption, diabetes, osteoporosis and male infertility are other typical features of CF and the diagnosis of CF should be considered in any adult who presents with these signs and symptoms, especially if they started in childhood.

According to the recent guidelines published by the Cystic Fibrosis Foundation in the USA [19], CF is diagnosed when an individual has both a clinical presentation of the disease and evidence of biochemical and genetic markers of CFTR dysfunction. If an individual with clinical features of the disease has a concentration of chloride >60 mmol·L⁻¹ at the sweat test or a concentration in the intermediate range (30–59 mmol·L⁻¹) but two disease-causing CFTR mutations the diagnosis of CF can be made. If the CFTR genotype is undefined, CFTR physiologic tests, such as nasal potential difference and intestinal current measurement, should be performed.

However, because of widespread CF newborn screening based on the measurement of immunoreactive trypsinogen in blood spots, 55.5–73% of new CF diagnoses in Europe now occur in asymptomatic or minimally symptomatic infants [20–22], so that it has become unusual to diagnose CF in patients with classic symptoms of respiratory disease and emaciation at an advanced age [23]. Nevertheless, the diagnosis can be challenging or inconclusive in some individuals [24]. For positive screened individuals with inconclusive CFTR functional and genetic testing the CFTR–related metabolic syndrome definition should be used, whereas for non-screened individuals presenting with monosymptomatic clinical entity associated with CFTR dysfunction that does not fulfil the diagnostic criteria for CF, a diagnosis of CFTR-related disorder should be considered [19].

Diagnosing CF is an important goal in adults with bronchiectasis. Around 7% of patients with CF are diagnosed as adults [23]. Patients affected by CF should be referred to a CF specialist centre because CF has a distinct pathophysiology, prognosis and treatment pathway. In many countries, CF patients have more access to respiratory therapies than patients with non-CF bronchiectasis. Furthermore, new CFTR modulator therapies can be provided to patients with specific mutations in order to improve their clinical outcomes.

The presence of a single CFTR mutation in the presence of a normal sweat test is relatively common in bronchiectasis and its clinical significance is uncertain.

The authors’ current practice is to screen for CF in all patients presenting with bronchiectasis before the age of 50 years, as in this case, and all patients with bronchiectasis symptoms onset during childhood irrespective of the age of presentation. In addition, the presence of upper lobe disease, Staphylococcus aureus or P. aeruginosa in sputum, or extrapulmonary features such as malabsorption, pancreatitis or infertility should also prompt CF testing irrespective of the age of the patient.

Case 2

Case 2 is a 45-year-old architect. He has had asthma since childhood. It has generally been well controlled throughout most of his adult life with only one or two exacerbations and no hospital admissions. In recent years he has noticed a decline in his exercise tolerance and an increase in cough which has become productive of purulent sputum with occasional thick/solid components. He has had several significant respiratory exacerbations which have not responded well to standard steroid and antibiotic treatment. One of these episodes was so severe he required emergency hospital admission due to respiratory distress. This improved when he managed to expectorate some very thick sputum. He was noted to have variable pulmonary infiltrates on chest radiographs during these episodes.

He attended a general respiratory clinic. A CT scan showed significant central bronchiectasis (figure 2). He was noted to have a marked eosinophilia on initial blood tests. This prompted Aspergillus serological testing. His total IgE was >2000 IU·mL⁻¹, his Aspergillus IgE was 750 IU·mL⁻¹ and Aspergillus IgG was 45 IU·mL⁻¹. A diagnosis of ABPA was made and he was commenced on high-dose oral corticosteroids.

FIGURE 2

Central tubular bronchiectasis in upper lobes.

Allergic bronchopulmonary aspergillosis

ABPA is an inflammatory disease caused by hypersensitivity to the ubiquitous fungus Aspergillus fumigatus. [25].

ABPA occurs most commonly in patients with asthma [26] and CF [27], but many patients do not have a history of asthma. ABPA is the cause of 1–10% of cases of bronchiectasis [8, 28, 29], but it can also complicate pre-existing bronchiectasis. Most ABPA cases occur in the third and fourth decade without a sex predilection.

Clinically, patients affected by ABPA may present with symptoms such as malaise, weight loss, low-grade fever, cough, purulent sputum containing brownish-black mucus plugs, pleuritic chest pains and haemoptysis [30].

There is not a single test to diagnose ABPA or a universally recognised set of criteria. Integration of patient’s history, clinical, radiological and serological features is used to diagnose ABPA. When ABPA is suspected, total IgE, specific IgE to Aspergillus or Aspergillus skin prick testing, IgG to Aspergillus and eosinophil count should be performed [9]. Total serum IgE levels >1000 IU·mL⁻¹ are the hallmark of ABPA and total IgE levels are also the most useful test for follow-up [30]. Caution is recommended in interpreting total IgE levels in patients recently treated with oral corticosteroids as “partially treated” ABPA may result in lower levels. A. fumigatus-specific IgE levels are also elevated and more sensitive than skin-prick test to Aspergillus antigen [31]. Peripheral eosinophilia and raised IgG antibodies against Aspergillus are also supportive of diagnosis [30].

HRCT is particularly useful in identifying ABPA as central bronchiectasis is a classical finding, even though peripheral bronchiectasis may occur [32]. The upper lobes are most frequently affected [33] but bronchiectasis may be present in all lobes [34]. Additional findings on HRCT are mucus plugging with “finger-in-glove” appearance, transient consolidation, centrilobular nodules associated with tree-in-bud, atelectasis, mosaicism due to air trapping on expiration and fibrosis in end-stage disease [30]. In some cases though, ABPA without bronchiectasis can be recognised [35]. Distinguishing between ABPA diagnosed serologically (ABPA-S) and ABPA with central bronchiectasis (ABPA-CB) may have prognostic implications: ABPA-S may be a more benign phenotype of ABPA, but most experts consider it a precursor of ABPA-CB [36].

The clinical course of ABPA is variable. There are five recognised stages of ABPA: stage I defines new, active ABPA; stage II is clinical and serological remission; stage III is recurrent active ABPA; stage IV defines patients with chronic, steroid-dependent ABPA; and stage V is end-stage disease with fibrocavitary lesions [37]. ABPA is important to identify as progressive lung damage occurs rarely once treatment is started [36].

Many drugs have been tried in the treatment of ABPA, such as: systemic and inhaled corticosteroids with the aim to reduce the inflammatory response; antifungal agents, that decrease the antigen burden and subsequent immune response; and omalizumab, a monoclonal antibody directed against IgE [38]. The initial treatment for ABPA is usually with oral corticosteroids [1]. Treatment is typically started at high doses, e.g. 40 or 50 mg daily, with weaning of the dose over several months with monitoring of total IgE and clinical symptoms.

ABPA remission is defined by improvement in symptoms, decrease in total serum IgE level, resolution of radiographic infiltrates and improvement in lung function [39]. Failure to respond to therapy should prompt consideration of comorbidities or alternative diagnoses. Infective exacerbations of bronchiectasis are associated with cough, wheeze and mucus plugging so that it may be challenging to discern if the primary driver of sputum is bacterial or allergic.

The role of antifungal treatment in ABPA is controversial. A double-blind randomised controlled trial previously demonstrated that treatment response, defined as a reduction of corticosteroid dose of >50%, was more rapid in patients taking a combination of steroids plus itraconazole compared to corticosteroids alone. This study suggests that itraconazole can be used as a steroid sparing agent [40]. Many clinicians use itraconazole in combination with corticosteroids as initial therapy for ABPA. Evidence for this approach is limited. A recent randomised controlled trial with 131 patients included found a similar rate of response in terms of IgE and exacerbations with initial therapy using prednisolone alone versus itraconazole alone, which does suggest that reducing fungal burden may be important alongside reducing inflammation [41]. We do not currently advocate treatment with antifungals alone. Concerns with antifungals include liver toxicity, photosensitivity and drug–drug interactions. Patients taking itraconazole should not take proton-pump inhibitors since they require an acidic environment to be absorbed.

Case 3

Case 3 is a 19-year-old student. He arrived in the UK from Pakistan aged 13 years. He has had respiratory problems since early childhood. His grandparents describe him as a small child with chronic cough since birth, as well as recurrent ear and sinus infections which have led to partial hearing loss. His brother and one of his cousins are similarly affected. His parents are first cousins. Since arriving in the UK he has had two hospital admissions with pneumonia. He has had a provisional diagnosis of asthma due to frequent symptoms even when well, but standard treatment has not been effective.

Examination revealed dextrocardia which was confirmed on radiographs and CT. CT scans also showed minor lingula bronchiectasis (figure 3). He grew H. influenzae on several occasions on sputum culture. Due to his age and family history, he was screened for CF (which was negative) and PCD. His nasal nitric oxide (nNO) was inconclusive and he went on to have a high-speed video analysis (HSVA) examination of nasal epithelial cells. This demonstrated static cilia. Electron microscopy and genetic testing revealed an outer dynein arm defect and biallelic pathogenic mutations in DHAH5 which confirmed the diagnosis of PCD. His sibling was also later diagnosed.

FIGURE 3

a) Chest radiograph showing dextrocardia. b) High-resolution computed tomography showing cylindrical bronchiectasis in the lingula.

Primary ciliary dyskinesia

PCD is a rare genetic disorder with an estimated prevalence of 1:10 000 [42]. PCD is caused by recessive autosomal or X-linked mutations in one of more than 35 genes which lead to functional and/or structural defects of cilia [43]. Motile cilia are specialised structures present in many tissues, so their immotility or ineffective beating results in a systemic disease with heterogeneous features. In upper and lower respiratory airways, failure of motile cilia leads to poor mucociliary clearance, which triggers a vicious cycle of inflammation and infection that leads to chronic rhinosinusitis and otitis media, progressive airway obstruction, bronchiectasis and ultimately respiratory failure [44]. A history of neonatal respiratory distress is often reported [45]. Since embryonic nodal cilia can also be defective, ∼50% of patients have situs inversus totalis or heterotaxy. When situs inversus is accompanied by chronic sinusitis and bronchiectasis, this is known as Kartagener’s syndrome [44]. Sperm flagella and cilia of the fallopian tubes share common axonemal structures with motile cilia, so a proportion of PCD-affected males and females are infertile and female patients that are fertile are at a higher risk of complications such as ectopic pregnancy [46].

The PCD diagnostic pathway is complex and some of its tests are very expensive or require a high level of expertise, so only a few tertiary centres can perform all of them. According to the 2017 ERS PCD guidelines, a diagnostic PCD step-wise approach includes firstly the measurement of nNO and the performance of nasal brushing for the evaluation of ciliary beat frequency and pattern with HSVA. The evaluation of ciliary ultrastructural defects with transmission electron microscopy or genetic testing can be used to confirm the diagnosis. Neither modality is able to detect all cases of PCD, with ∼80% and 70% of specificity, respectively. Therefore, in cases where diagnosis remains inconclusive, repeating the high-speed video microscopy on a new sample or after in vitro culture of the airway epithelium is suggested. Detection of presence or absence of ciliary proteins on ciliated respiratory epithelial cells by immunofluorescence is also increasingly used [47]. In the ERS bronchiectasis guidelines, testing for PCD is limited to patients with clinical features consistent with the disease (persistent productive cough since childhood, chronic rhinosinusitis, chronic middle ear disease with or without hearing loss, situs anomalies, congenital cardiac defects and a history of neonatal respiratory distress or neonatal intensive care admittance in term infants) [9]. All these clinical factors are included in the PICADAR score, a simple diagnostic tool to predict whether symptomatic patients have PCD [48]. PICADAR score and nNO are suitable and easy-to-perform tests in respiratory clinics, whereas further tests should be performed in PCD or tertiary centres with high expertise. Importantly, although nNO is often used as a screening test, it can be normal or inconclusive in some cases of PCD, as in this case. Therefore, where there is a high index of suspicion as in this case, further investigations should be performed irrespective of the nNO result.

HRCT features may be helpful in raising the suspicion of PCD. Bronchiectasis in PCD is predominantly in the middle lobe, lingula and lower lobes, with a central or diffuse distribution. As expected from the underlying pathophysiology of the disease, the severity of bronchiectasis increases with age, whereas data regarding the correlation between HRCT features and pulmonary function are conflicting [49, 50]. Besides lung parenchymal alterations, situs inversus, heterotaxy and congenital heart disease could support the suspicion of PCD. Pectus excavatum is identified in 9% of cases [49].

Investigation and diagnosis of PCD is an important goal because it is a multi-system disease which benefits from a specific and multidisciplinary team approach, including genetic and reproductive counselling, in a PCD referral centre [1]. Although airway clearance is an important aspect of the management of all bronchiectasis patients, it is particularly emphasised in the management of patients with PCD. The importance of making the diagnosis is emphasised by data showing worse lung function and higher rates of P. aeruginosa infection the later patients are diagnosed [51–55].

Case 4

Case 4 is a 77-year-old retired librarian. She has had cough for many years, although she has only sought medical attention recently as she has developed more systemic symptoms. Her new symptoms are fatigue, weight loss and fever. A chest CT scan was performed looking for a possible occult malignancy and bronchiectasis was found (figure 4).

FIGURE 4

Cylindrical bronchiectasis and tree-in-bud pattern in lower lobes and middle lobe.

Following the CT scan features of cylindrical bronchiectasis and tree-in-bud pattern in middle and lower lobes, she attended a respiratory clinic. A sputum sample was sent and a positive acid-alcohol-fast bacilli staining was found. After 4 weeks Mycobacterium avium complex (MAC) grew on the culture. Several subsequent cultures were also positive. As NTM can complicate pre-existing bronchiectasis as well as being a cause of bronchiectasis, further testing for immunodeficiency, ABPA and other causes was performed. No abnormalities were found. In view of the clinical history, radiological findings and absence of an alternative cause, a diagnosis of bronchiectasis due to NTM lung disease was made.

Nontuberculous mycobacteria

NTM are ubiquitous organisms in the environment and can be inhaled or ingested from water, soil and dust with different consequences according to individual and organism characteristics.

The exact prevalence of NTM disease is difficult to obtain because reporting these infections is not mandatory in many countries and discriminating between colonisation and active infection can be challenging. Certainly, the recovery of NTM from the respiratory tract is increasing as a result of a real growing prevalence of pulmonary disease due to these organisms [56], but also because of improving microbiological techniques and the increasing attention to this topic. In a systematic review, the overall prevalence of NTM in patients with bronchiectasis was 9.3%, with higher rates in larger cohorts and in Asian populations [57]. The most frequent species is MAC (which includes M. intracellulare, M. avium and M. chimaera), with a percentage up to 80% of all NTM lung disease cases [58, 59] whereas the isolation of other species, e.g. M. abscessus, M. kansasii, M. chelonae and M. fortuitum, varies from study to study.

The outcome of respiratory exposure to NTM, colonisation or pulmonary disease, probably depends on a complex relationship between host-related factors (genetics, immune status and lung damage), exposure-related factors (duration, number of organisms, inhaled or ingested particle size) and organism-related factors (capability of mycobacteria to cause pulmonary disease, e.g. M. malmoense has more pathogenicity than M. chelonae [60]). NTM can be both a cause and a consequence of bronchiectasis. NTM can cause bronchiectasis by destroying the bronchial wall and bronchiectasis can predispose to NTM colonisation/disease due to an impairment of bronchial anatomy and local host defence [61, 62].

The diagnosis of NTM pulmonary disease is based on the presence of clinical criteria (respiratory symptoms consistent with the diagnosis), radiological features consistent with NTM, exclusion of other diagnoses and microbiological criteria (a positive culture from one bronchial lavage, at least two NTM-positive sputum cultures or a lung biopsy with mycobacterial histopathologic features plus a NTM-positive culture) [63]. NTM pulmonary disease is often challenging to diagnose, due to the presence of organisms in the environment and the consequent possibility of sample contamination and the wide spectrum of clinical manifestations. If a diagnosis of NTM pulmonary disease is difficult in healthy individuals, it is even more difficult in bronchiectasis patients, where distinguishing between bronchiectasis-related and NTM-related symptoms can be challenging. According to the ERS guidelines, three sequential daily sputum cultures for mycobacteria or a single bronchoalveolar lavage should be considered in patients with persistent fever, weight loss, haemoptysis, symptoms non-responsive to standard therapy or rapid clinical deterioration [9]. Up to 50% of patients will not produce sputum spontaneously and such patients should be investigated by bronchoscopy or sputum induction.

HRCT features should also be investigated when NTM pulmonary disease is suspected. Two major radiological patterns are related to NTM infection: the nodular/bronchiectatic and fibrocavitary forms. The first one is characterised by multiple small centrilobular nodules and cylindrical bronchiectasis, especially localised in middle lobe and lingula. [64]. This pattern is frequently associated with MAC infection [65] and with the “Lady Windermere syndrome”: women with elderly age, low body mass index and chronic cough [66]. The fibrocavitary form is characterised by increased opacity areas and cavitations, usually in the upper lobes, with or without calcifications. In addition, apical pleural thickening and fibrosis with volume loss can be found. Pleural effusion, adenopathies and lower lobes involvement are uncommon [67]. This pattern is frequently associated with M. abscessus, M. chelonae and M. kansasii infections [65]. The underlying pulmonary disease in these patients is often advanced COPD.

Once a diagnosis of NTM disease is established, the institution of therapy is not always mandatory. Since NTM treatment is usually prolonged and based on multiple drugs with significant side effects, a careful evaluation of risks and benefits should be made. Considering NTM species, patient’s conditions, radiological pattern and disease severity is crucial in the decision-making process. In the case of NTM treatment, patients should be closely monitored with visits, sputum cultures, lung function and blood tests to assess the response to therapy and possible side effects. Follow-up should also be prolonged after treatment since relapse or new infections are possible and the underlying bronchiectasis requires long-term management. In case of no treatment, patients should be monitored to evaluate disease progression.

This patient was treated with rifampicin 600 mg daily, ethambutol 15 mg·kg⁻¹ daily and clarithromycin 500 mg twice daily. This, sometimes with the addition of intravenous amikacin for 3 months or nebulised amikacin, is the regimen recommended for severe MAC pulmonary disease where acid-fast bacilli is smear positive or there are severe symptoms of systemic illness or cavitation. Intermittent therapy three times per week (rifampicin 600 mg three times per week, ethambutol 25 mg·kg⁻¹ three times per week and azithromycin or clarithromycin three times per week) are recommended in patients with mild disease who are smear negative with the absence of cavitation and no systemic symptoms. Patients who have intolerance to drugs or refractory disease should be referred to an NTM speciality clinic.

Case 5

A 66-year-old woman with established idiopathic bronchiectasis has had three to four exacerbations per year for the past 3 years despite performing daily chest physiotherapy. Testing for NTM, ABPA and other complications were negative, but sputum shows persistent infection with P. aeruginosa. She produces large volumes of sputum daily despite performing the active cycle of breathing technique.

This is one of the most common presentations of bronchiectasis, as exacerbations are one of the most important manifestations of bronchiectasis and P. aeruginosa is the most frequent organism in severe bronchiectasis worldwide. This patient could present any of the CT scans shown above (figure 5).

FIGURE 5

Different radiological “phenotypes” of bronchiectasis. a) Tubular bronchiectasis in lower lobes, b) varicose bronchiectasis in lower lobes, c) cystic bronchiectasis in right lower lobe, d) cystic bronchiectasis with tree-in-bud pattern and mucus plugging in lower lobes (cavitation in right lower lobe), e) mucus plugging and tree-in-bud pattern in lower lobes, f) varicose bronchiectasis in upper lobes and lingula.

Cylindrical bronchiectasis is the most common morphological pattern identified on CT scans [68] and is recognised by an abnormal dilatation of bronchus with uniform calibre and lack of tapering at the periphery, producing a tramline (tram track sign) (figure 5a). Varicose bronchiectasis has no regular form or size, with distortion and irregular bulging (figure 5b), whereas cystic bronchiectasis is a saccular dilatation with ballooned cutline that can be traced almost to the pleura (figure 5c). In the context of bronchiectasis cavitation can also be seen, especially in NTM pulmonary disease or previous M. tuberculosis infection (figure 5d). Another frequent finding is mucus plugging, the presence of mucoid secretions in peripheral airways (figure 5e), that can produce V- and Y-opacities, the so-called tree-in-bud pattern (figures 5d and e). The distribution and extension of bronchiectasis are also key components in bronchiectasis evaluation. For instance, localised bronchiectasis, especially in a single lobe, should raise the suspicion of obstructive or post-infective bronchiectasis (figure 5f).

Management of frequent exacerbations

Frequent exacerbations should prompt a review of all aspects of management including reviewing current airway clearance regime, repeat sputum microbiology and repeat testing for NTM, ABPA and ensuring the all possible treatable causes and comorbidities have been identified.

Intensification of treatment should address all aspects of the vicious cycle including targeting airway infection, airway clearance and airway structural damage. Interventions that have been shown to reduce exacerbations and improve quality of life in either controlled trials or observational studies include macrolides [69–71], inhaled antibiotics [72–74], mucoactive drugs (e.g. hypertonic saline) [75, 76], airway clearance [77, 78] and pulmonary rehabilitation [79, 80]. All of these interventions were recommended in appropriate patients in the 2017 ERS guidelines [9].

The first-line recommendation for P. aeruginosa with frequent exacerbations is an inhaled antibiotic [9] and this patient was commenced on twice daily colistin treatment. Airway clearance was optimised through an appointment with a respiratory physiotherapy and nebulised saline was added to her daily regimen. She was referred to and completed pulmonary rehabilitation. These interventions resulted in a reduction of her exacerbation frequency and an improvement in quality of life. Important issues with inhaled antibiotics include an approximately 10% risk of bronchospasm [72] and the burden of administering a daily nebulised therapy that takes some time. Antibiotic resistance is also a theoretical risk with any long-term antimicrobial treatment. Treatment of these patients with multiple nebulised therapies plus airway clearance results in a large burden of treatment and increases the risk of non-adherence. Macrolides are a reasonable alternative with a lower treatment burden but have less evidence in the context of chronic P. aeruginosa infection [69–71]. Furthermore, it is important to exclude NTM prior to commencing macrolide therapy to avoid inducing resistance. A detailed discussion of management of bronchiectasis is beyond the scope of this review. Further information on treatment including antibiotic therapy, thoracic surgery, physiotherapy management and treatment of acute exacerbations can be found in the 2017 ERS guidelines [9].

Conclusion

Bronchiectasis is no longer a neglected disease. Interest and awareness are increasing in scientific and patient communities. Chest physiotherapy and antibiotic treatment when an exacerbation occurs remain the cornerstone of bronchiectasis therapy. Discovering the underlying aetiology of bronchiectasis could make a real difference in the management and prognosis of patients and could delay the progression of lung involvement when treated. Since routine testing of all patients for all possible underlying disorders has not been shown to be cost-effective, current guidelines recommend using clinical judgement and recognition of patient phenotypes to guide testing for disorders such as CF, PCD, NTM and others. Therefore, physicians should be familiar with the key clinical history and CT features that can raise the suspicion of a specific cause of bronchiectasis and lead to improved treatment.

Footnotes

• Provenance: Commissioned article, peer reviewed.

• Conflict of interest: J.D. Chalmers reports grants and personal fees (for COPD) from GlaxoSmithKline, Boehringer Ingelheim and Pfizer, grants (for COPD) from AstraZeneca, grants and personal fees (for research into bronchiectasis) from Bayer Healthcare and Grifols, and personal fees (for consultancy) from Napp, outside the submitted work.

• Received February 23, 2018.
• Accepted June 4, 2018.

ERR articles are open access and distributed under the terms of the Creative Commons Attribution Non-Commercial Licence 4.0.

90,000 What is bronchiectasis? | FBUZ Treatment and Rehabilitation Center of the Ministry of Economic Development of Russia. Official site

One of the components of the human respiratory system is the bronchi. These are air tubes with a relatively flat inner surface through which air passes. They are part of the lungs.
As a result of various diseases, deformities of the bronchi of various shapes can occur. These deformities are called bronchiectasis.

Bronchiectasis is persistent irreversible dilatation of the bronchi caused by changes in the walls of the bronchi

Reasons for the development of bronchiectasis
There are two main groups of reasons for the development of bronchiectasis:

• congenital – the presence of changes in the lungs in a newborn as a result of impaired formation of the fetal bronchi, alpha 1-antitrypsin deficiency and cystic fibrosis.

90,021 acquired – arising in children or adults, as a result of past bronchopulmonary diseases.

What is bronchiectasis
Bronchiectasis can be detected by chance and do not show any complaints. If in the course of further examination the pulmonologist does not reveal signs of an inflammatory process, then in this case bronchonchiectasis does not require treatment. Only a special complex of breathing exercises and preventive vaccinations are recommended.
If an inflammatory secret accumulates in bronchiectasis, inflammation constantly exists around the bronchial tubes in the lung tissue, scars form, then this condition is called bronchiectasis . This disease is prone to constant progression, that is, the spread of changes in the bronchi and lung tissue, with the formation of new bronchiectasis. Bronchiectasis always requires prophylactic treatment or treatment during an exacerbation by a pulmonologist.

Acquired bronchiectasis
I will try to explain the mechanism of formation of bronchial deformation (bronchiectasis) if they appear in the lung tissue after birth.We have already said that changes in the shape and lumen of the bronchi in this disease are persistent, irreversible. Most often, the mechanism of development of bronchiectasis is as follows: first, damage to the bronchial wall develops (mainly due to severe or sluggish infection or toxic damage). Then its lumen can expand due to “pressure” on the wall of the bronchus from the inside of sputum accumulated in its lumen or gross cicatricial changes in the lung tissue formed against the background of inflammation can “stretch” the bronchi and lead to a change in their shape.

Manifestations of bronchiectasis
Symptoms that may help suspect bronchiectasis:

• Cough with profuse purulent expectoration. If the bronchiectasis is predominantly on one side, the sputum may flow better at a certain position of the body (for example, on the right or left side).
• Blood in sputum occurs in 20-25% of cases.
• Dyspnea develops most often with an exacerbation of the disease or a high prevalence of the process in the lungs.
• Repeated pneumonia, most often with the same localization. This is a very characteristic and frequent sign of bronchiectasis, because mucus constantly accumulates in the deformed dilated bronchi, and when any infection is attached, inflammation develops in the lung tissue.

The main manifestations of bronchiectasis are:

• Cough with a large amount of purulent or mucopurulent sputum
• Repeated pneumonia, most often with the same localization

Diagnosis of bronchiectasis

A reliable diagnosis of bronchiectasis or bronchiectasis is made only after high-resolution computed tomography of the chest organs

Bronchoscopy (examination of the bronchi using special equipment) does not apply to methods for diagnosing bronchiectasis, but it can help to identify the localization of the bronchus – a source of increased secretion of mucopurulent secretions and / or blood.During bronchoscopy, damaged bronchi can also be sanitized by injecting antimicrobial solutions into them.
Bronchography (the introduction of a contrast agent into the bronchi) is currently not performed as a method for diagnosing bronchiectasis due to a large number of side effects and insufficient information content.

Additional studies to be carried out after the detection of bronchiectasis

• Sputum culture with antibiotic susceptibility testing
• Sputum culture for Mycobactérium tuberculósis (tuberculous bacillus) and causative agents of non-tuberculous mycobacteriosis
• Function of external respiration with a bronchodilator test to detect bronchospasm and resolve the issue of the need to prescribe inhalers.
• With frequent colds and sinusitis (inflammation of the paranasal sinuses), it is necessary to undergo an immunological examination (determine the levels of immunoglobulins A, M, G) in order to identify a significant decrease in immunity.
• If multiple bronchiectasis or bronchiectasis is detected at a young age, it is necessary to undergo an examination to exclude cystic fibrosis. With a mild course of this congenital disease, the diagnosis may not have been made in childhood.
• Determination of the level of alpha 1 antitrypsin in the blood
• Determination of antibodies to Aspergillus fumigatus of immunoglobulin E, G classes to exclude bronchopulmonary aspergillis (fungal infection of the lungs) and blood test for gallactamanan.

Patients with bronchiectasis should be observed and treated by a pulmonologist

The question of the surgical treatment of bronchiectasis is taken by a torocal surgeon in conjunction with a pulmonologist

Non-drug treatment of bronchiectasis
The less mucus accumulates in the deformed bronchi, the less likely there will be exacerbations and progression of the disease. For this purpose, a postural drainage technique has been developed. This is a therapeutic procedure that facilitates the discharge of bronchial secretions and increases the productivity of cough by giving the body special drainage positions.In these positions, the sputum is propelled by gravity towards the main bronchi and trachea and, when coughing, is removed from the bronchi.
Methods of postural drainage, breathing exercises and breathing simulators can be recommended by a pulmonologist

Modern methods of treatment of bronchiectasis | Useful articles

Bronchiectasis – irreversible morphological changes (expansion, deformation) and functional inferiority of the bronchi, leading to chronic suppurative lung disease.The whole complex of pulmonary and extrapulmonary changes in the presence of bronchiectasis is called bronchiectasis.

Congenital and acquired bronchiectasis

Congenital bronchiectasis are relatively rare and develop in connection with violations of the formation of the bronchial tree. The histological sign of congenital bronchiectasis is the disordered arrangement of the structural elements of the bronchus in their wall.

The main etiological factor of acquired bronchiectasis is a genetically determined inferiority of the bronchial tree (underdevelopment of the elements of the bronchial wall), which, in combination with impaired bronchial patency and the appearance of inflammation, leads to persistent deformation of the bronchi.

The formation of bronchiectasis is largely promoted by whooping cough, acute respiratory infections, measles, bronchitis, pneumonia, lung abscesses, tuberculosis, foreign bodies in the tracheobronchial tree.

Main complaints: cough with a lot of purulent sputum, hemoptysis, chest pain, shortness of breath, fever, sweating, loss of body weight and work capacity. The amount and nature of sputum depends on the degree of bronchial damage. It may contain impurities of blood and pus, an unpleasant odor.

The disease is characterized by exacerbations and remissions.During exacerbations, the temperature rises, shortness of breath appears, wheezing in the chest, blue lips. Against the background of a long course, the patient’s fingers acquire the characteristic shape of drumsticks, and the nails – an hour glass. Gradually, the patient’s general condition worsens.

Bronchiectasis is often complicated by pulmonary hemorrhage, abscess formation, development of pulmonary fibrosis and emphysema, cor pulmonale, amyloidosis.

Modern treatment tactics

In bronchiectasis, modern antibiotics of the macrolide class are prescribed to suppress pathogenic microflora, and β2-agonists to eliminate reflex spasms of small bronchi.Mucolytics are also effective, thinning mucus and making it easier to cough up. To stop inflammation, hormonal agents are indicated in the treatment of bronchiectasis. To activate the body’s own defenses, immunostimulants are included in the therapeutic scheme.

The key procedure for the conservative treatment of bronchiectasis is the sanitation of the bronchial tree (cleaning the lumen of the bronchi from purulent sputum followed by the introduction of antibiotics). With signs of oxygen deficiency, oxygen therapy is prescribed.The patient is also prescribed a set of exercises that promote the evacuation of bronchial sputum, and vibration massage of the chest. For general strengthening of the body are shown:

• high-calorie diet – 3000 kcal per day;
• Diet Therapy – Nutrient-rich functional food;
• vitamin therapy.

Given the irreversibility of the process, and, consequently, the futility of conservative therapy, the only radical method of treating bronchiectasis should be considered a surgical one, the volume of which depends on the spread of bronchiectasis.

Rehabilitation, prevention, possible risks

An important component of complex rehabilitation for bronchiectasis is lifestyle correction. The patient needs to walk in the fresh air, quit smoking and avoid secondhand smoke, eat a balanced diet, exercise, and regularly do breathing exercises.

It is important to be registered with a pulmonologist, at the frequency prescribed by the doctor, to attend preventive appointments, if necessary, take courses of physiotherapy.Comprehensive prevention provides for timely treatment of respiratory diseases and hardening.

Without adequate treatment of bronchiectasis, chronic bronchitis, pulmonary and heart failure, cor pulmonale, bronchial asthma develop. Patients experience reduced efficiency and poor quality of life. It is extremely important to see a doctor in a timely manner in order to achieve long-term sustained remission.

Two antibiotic therapy for bronchiectasis

Relevance

Bronchiectasis (bronchiectasis) is a lung disease that causes pathological changes in the airways that lead to repeated chest infections and is associated with more than double mortality compared to the general population.Although previously considered relatively rare, the number of people with the disease appears to be on the rise, especially among those over 75 in low / middle income countries. Antibiotics are the main treatment for chest infections, but their use must be weighed against possible side effects and the risk of increased resistance to antibiotic therapy. One strategy to improve response and / or reduce antibiotic resistance involves the use of two antibacterial agents simultaneously: dual antibiotic therapy.Therefore, the aim of this review was to evaluate the effects of dual antibiotic therapy in the treatment of bronchiectasis in adults and children.

Research characteristics

In October 2017, we found two relevant studies comparing therapy with two antibiotics (oral antibiotic and inhaled antibiotic) with oral antibiotic alone. The studies included 118 adults (mean age 62.8 years). One study compared the combination of inhaled tobramycin and oral ciprofloxacin with oral ciprofloxacin monotherapy, and in the second study inhaled gentamicin combined with a systemic (which affects the whole body, not just the lungs) antibiotic was compared with only one systemic antibiotic.For the last study, only a summary was available. The sources of research funding were not presented in the publications.

Main Findings

Results from one small clinical trial in 53 adults showed no evidence of benefit from dual antibiotics (oral antibiotic combined with inhaled antibiotic) for successful treatment of exacerbations, serious adverse events, sputum count, lung function scores, and antibiotic resistance …However, we did not find sufficient evidence to draw confident conclusions about their use.

Quality of evidence

Overall, the quality of the evidence was very low, mainly because one of the studies was not well described and included a small number of participants. No information was provided on exacerbations, exercise tolerance and quality of life. We did not find any clinical trials that compared other therapies with the two antibiotics, and we did not find trials in children.Thus, uncertainty remains regarding the use of the two antibiotics, and further high-quality research is needed to investigate the role of the simultaneous use of two antibiotics in the treatment of adults and children with bronchiectasis.

1.6. Classification / ConsultantPlus

According to the Russian Classification of clinical forms of bronchopulmonary diseases in children (2009), bronchiectasis and bronchiectasis, which are a manifestation of another pathology, are distinguished: a purulent-inflammatory process in the dilated deformed bronchi with infiltrative and sclerotic changes in the peribronchial space.

Bronchiectasis is usually subdivided into cylindrical (Appendix D1, Fig. 3), saccular (Appendix D1, Fig. 4) and mixed (Appendix D1, Fig. 5). Also described are cystic, fusiform (Appendix D1, Fig. 6) and varicose (Appendix D1, Fig. 7) BE.

Due to the fact that different types of EB can occur in one patient, the prevalence and localization of changes within specific bronchopulmonary segments is of greater importance.

– Cylindrical EB occur mainly with sclerosis of the bronchial walls.In this case, the lumen of the bronchus expands evenly over a sufficiently large extent. Most often this occurs against the background of other lung diseases (secondary bronchiectasis). The cylindrical shape does not contribute to the accumulation of a large volume of pus, therefore, the general condition of patients, as a rule, is not too severe; in some cases, such BEs can regress when the cause that caused them is eliminated (aspiration of a foreign body, atelectasis, infection).

– Saccular BE – single spherical or oval expansion on one side of the bronchus.Often, this form occurs with congenital defects in the development of lung tissue. Sacks are blind bulges of the wall that can be large. A significant amount of sputum and pus accumulates here. The course of the disease in such patients is usually severe.

A common variant of the development of EB is partial obstruction of a large bronchus by a tumor, foreign body, scar, or its compression from the outside by enlarged lymph nodes. Such BEs occur in the area of ​​partial or complete atelectasis and are referred to as atelectatic.

Traction of the bronchial wall by fibrous cords from the surrounding fibrously altered tissue plays a certain role in the mechanism of EB development, in connection with which the concept of traction bronchiectasis has been established in scientific terminology [5, 6, 7, 12, 13].

During bronchiectasis, two phases are distinguished:

– Exacerbation phase – an active inflammatory process with the accumulation of pus. During this period, the symptoms of the disease are most striking. In some cases, in the absence of adequate treatment, a rapid deterioration of the patient’s condition may occur: the inflammatory process goes beyond the dilated bronchus, pneumonia develops.The frequency of exacerbations can vary – from a few episodes per year to several within a month.

– The remission phase is characterized by the absence of acute symptoms. BEs are preserved at the same time. In the presence of multiple dilatations of the bronchi and concomitant pneumosclerosis in the remission phase, dry or wet cough, signs of respiratory failure may be observed.

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Bronchiectasis.The ABC of antibiotic therapy. Vidal reference book of medicines

Bronchiectasis – Radiation diagnostics in pediatrics

Bronchiectasis

a condition characterized by the appearance in the lungs of abnormal dilated areas of the bronchi with a change in their structure and functioning.

Bronchiectasis can be congenital or acquired. Congenital bronchiectasis occurs in children and is usually associated with impaired fetal lung formation. The pathogenesis of acquired bronchiectasis is associated with inflammatory changes in the bronchial wall, usually affecting small bronchi. In this case, the infiltration of the parenchyma by inflammatory cells occurs. The subsequent destruction of the surrounding structures leads to the development of dilatation. [1]

Currently, bronchiectasis is most often caused by diseases that are characterized by damage to the bronchial wall or difficulty in clearing the bronchus from mucus (eg.: cystic fibrosis, primary ciliary dyskinesia). In adults, bronchiectasis most often develops for a long time, usually in the presence of chronic obstructive pulmonary disease. However, the cause of bronchiectasis in a particular patient is often not clear. [3.4]

Etiological factors are different, they include:

• Post-infectious processes (after pneumonia, whooping cough, measles, mycobacterial infection)
• Mucociliary Disorders
• Obstruction, aspiration
• Immune disorders (hypogammalbuminemia, HIV, cancer, GVHD)
• Rheumatic lesions
• COPD [1]

Depending on the form of changes in the bronchi, types of bronchiectasis are distinguished :

• Cylindrical
• Saccoon
• Spindle
• Mixed

Symptoms include cough, often with mucopurulent sputum, most pronounced in the morning, shortness of breath, bronchial obstruction syndrome, chest pain, rarely hemoptysis.

To diagnose bronchiectasis, radiation diagnostic methods are used, for the initial examination, radiography is usually used. On the X-ray, thickened and abnormally dilated bronchial walls are identified, in the form of parallel linear enlightenments, a symptom of “tramways”, or ring-shaped shadows.

Computed tomography is the gold standard for diagnosing bronchiectasis. The main signs of bronchiectasis are distinguished:

• The inner diameter of the bronchus is wider than the adjacent pulmonary artery
• Conical deformation of the bronchus
• Visualization of the bronchi on the outer 1-2 cm of the pulmonary field [1,4, 5]

Bronchiectasis can affect part or all of the lung.Bilateral damage is also possible. Occurrence in one lobe / segment of the lung is usually caused by an obstruction of the airway. [2]

Clinical case

Patient M., 17 years old. Sick from birth. At the place of residence until the age of 15, she was observed with a diagnosis of Daria’s disease. Repeatedly underwent inpatient treatment at the place of residence, periodically received antibacterial therapy, glucocorticosteroids, physiotherapy. Was sent to the FNKTS DGOI them.D. Rogachev, where he was diagnosed with primary combined immunodeficiency.

On the X-ray image, ring-shaped shadows of reduced density are visualized, with clear boundaries in the basal parts of the right lung.

During MSCT, pronounced widespread cylindrical bronchiectasis is visualized in the middle lobe on the right, in both lower lobes and in the reed segments of the upper lobe on the left. Uneven pneumatization of the lung tissue in the form of alternating areas of increased airiness of the lung parenchyma and areas of interstitial compaction by the type of frosted glass.

References:

1. Jessica Rademacher, Tobias Welte. Bronchiectasis — Diagnosis and Treatment. Dtsch Arztebl Int. 2011 Dec; 108 (48): 809-815
2. Paul T King. The pathophysiology of bronchiectasis. Int J Chron Obstruct Pulmon Dis. 2009; 4: 411-419.
3. http://www.who.int/respiratory/other/bronchiectasis/en/
4. O’Donnell AE. Bronchiectasis. Chest. 2008 Oct; 134 (4): 815-23. doi: 10.1378 / chest.08-0776.
5. Kim Bird; Jawedulhadi Memon.Treasure Island (FL): StatPearls Publishing; 2018 Jan. Bookshelf ID: NBK430810PMID: 28613561

Questions and Answers | page 1

Hello! On CT scan of 12/16/2013 I was diagnosed with SIGNS OF COPD, CYLINDRICAL BRONCHEECTASES OF THE LOWER LOCATE OF THE RIGHT LUNG AND S4-S5 OF BOTH LUNGS. I was in various hospitals in the city, undergoing antibiotic treatment – there was no improvement.I have constant exacerbations, subfibrality lasts for the 4th year, cough with purulent sputum (sometimes with a full mouth, streaked with blood). The operation was denied to me due to the bilateral nature of the disease. On March 7, 2014, in another hospital, I underwent a repeated CT scan, and was diagnosed with MIXED BRONCHEECTASES OF THE LOWER LOCATE OF THE RIGHT LUNG. CHRONIC DEFORMING BRONCHITIS. PARASEPTAL EMFYSEMA OF THE LOWER LOCKS OF BOTH LUNGS. Once again, the question arose about the operation, the surgeon insists on a right-sided lower lobe resection, but I’m worried, won’t emphysema at the bottom of the left lung remain? Will there be an improvement after the operation if the cause of the process is removed only on the right? Is the right lobe resection rational in my case? Please help with an answer.

Question # 26125 | Topic: Bronchiectasis, bronchiectasis of the lungs | 04/03/2014 | Anastasia | Ulyanovsk, Russia
The operation will most likely save you from the purulent process. The alternative is only regular lung debridement.

Hello! I am 18 years old. In July, she fell ill with pneumonia of the left lung S3. She was treated for a very long time, both in the hospital and on an outpatient basis. I was consulted by a phthisiatrician, there was no tuberculosis. Sent for CT of the chest, in the conclusion (from November 15) – pneumofibrosis in S3 and the formation of cylindrical bronchiectasis.I drank 2 courses of Erespal tablets, it got a little better. In the morning, a cough with purulent sputum, wheezing in the lung, and sometimes a cough during the day worries – this interferes with life. Is surgery to remove a segment / part of the lung indicated for me? And how can I contact you? Thank you in advance.

Question # 26070 | Topic: Bronchiectasis, bronchiectasis of the lungs | 02/10/2014 | Katya | Moscow
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Hello, I have Bronchostatic disease. I have recently undergone a course of treatment, but for two days now I have had severe hemoptysis.

Question # 25138 | Topic: Bronchiectasis, bronchiectasis of the lungs | 10.08.2011 | Natalia | PMR Tiraspol
Hemoptysis is one of the manifestations of bronchiectasis in which surgical treatment is indicated

Hello! diagnosed with bronchiectasis (S5) of the left lung. It was recommended to have a removal operation in a year and a half. Is it possible to do without surgical intervention?

Question # 24825 | Topic: Bronchiectasis, bronchiectasis of the lungs | 08.12.2010 | elena | Moscow
It all depends on the frequency of exacerbations – if it is only an X-ray finding – it is not worth operating – it can be observed.If exacerbations with purulent sputum occur quite regularly (at least once a year), the operation is necessary and should not be postponed for another year.

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