Interstitial Lung Disease: Pulmonary Fibrosis

What is interstitial lung disease?

Interstitial lung disease refers to a group of about 100 chronic lung disorders characterized by inflammation and scarring that make it hard for the lungs to get enough oxygen. The scarring is called pulmonary fibrosis.

The symptoms and course of these diseases may vary from person to person. The common link between the many forms of the disease is that they all begin with inflammation.

• Bronchiolitis: inflammation of the small airways (bronchioles).
• Alveolitis: inflammation of the air sacs where oxygen and carbon dioxide exchange in the blood takes places (alveoli).
• Vasculitis: inflammation that involves the small blood vessels (capillaries).

Fibrosis leads to permanent loss of your lung tissue’s ability to carry oxygen. The air sacs, as well as the lung tissue around the air sacs and the lung capillaries, are destroyed when the scar tissue forms.

The disease may run a gradual course or a rapid course. People who have it may notice variation in symptoms, from very mild to moderate to very severe. The condition may stay the same for a long time or it may change quickly. The course of the disease is unpredictable. If it progresses, the lung tissue thickens and becomes stiff, making breathing more difficult.

What causes interstitial lung diseases?

The cause of interstitial lung disease is not known. Major contributing factors include:

• Smoking
• Certain drugs or medicines
• Exposure to substances at work or in the environment such as organic or inorganic dusts
• Certain connective tissue or collagen diseases and sarcoidosis
• Family history
• Radiation treatment

What are the symptoms of interstitial lung diseases?

Each person may experience interstitial lung disease differently, but the most common symptoms include:

• Shortness of breath, especially with activity
• Dry, hacking cough that does not produce phlegm
• Extreme tiredness and weakness
• Loss of appetite
• Unexplained weight loss
• Discomfort in the chest
• Labored breathing, which may be fast and shallow
• Bleeding in the lungs

The symptoms of interstitial lung diseases may look like other lung conditions or medical problems. Talk with your healthcare provider for a diagnosis.

How are interstitial lungs diseases diagnosed?

In addition to a complete medical history and physical exam, the healthcare provider may also request pulmonary function tests. These tests help to measure the lungs’ ability to move air into and out of the lungs. They may include:

Spirometry

A spirometer is a device used to check lung function. Spirometry is one of the simplest, most common tests. It may be used to:

• Determine how well the lungs receive, hold, and move air
• Look for lung disease
• See how well treatment is working
• Determine the severity of a lung disease
• Find out whether the lung disease is restrictive (decreased airflow) or obstructive (disruption of airflow)

Peak flow monitoring

This device is used to measure the how fast you can blow air out of the lungs. Disease-related changes can cause the large airways in the lungs to slowly narrow. This will slow the speed of air leaving the lungs. This measurement is very important in evaluating how well or how poorly the disease is being controlled.

Chest X-rays

This test takes pictures of internal tissues, bones, and organs.

Blood tests

Arterial blood gas may be done to check the amount of carbon dioxide and oxygen in the blood. Other blood tests may be used to look for possible infections.

CT scan

This test uses a combination of X-rays and computer technology to produce horizontal, or axial, images (often called slices) of the body. CT scans are more detailed than regular X-rays.

Bronchoscopy

This is direct exam of the main airways of the lungs (bronchi) using a flexible tube called a bronchoscope. Bronchoscopy helps to evaluate and diagnose lung problems, check blockages, take out samples of tissue or fluid, and help remove a foreign body. Bronchoscopy may include a biopsy or bronchoalveolar lavage.

Bronchoalveolar lavage

Removing cells from the lower respiratory tract to help identify inflammation and exclude certain causes.

Lung biopsy

Removing a small piece of tissue from the lung so it can be examined under a microscope.

How are interstitial lung diseases treated?

Because there are so many causes, treatment will vary. Some interstitial lung diseases do not have a cure. Treatment is aimed at preventing more lung scarring, managing symptoms, and helping you stay active and healthy. Treatment can’t fix lung scarring that has already occurred.

Treatments may include:

• Lung transplant
• Oral medicine, including corticosteroids to reduce inflammation and cyclophosphamide (Cytoxan) to suppress the immune system
• Oxygen therapy, from portable containers
• Pulmonary rehab

Check with your healthcare provider about getting flu and pneumococcal shots. Getting a flu shot every year can help prevent both the flu and pneumonia. In addition, pneumococcal bacteria can cause minor problems, such as ear infections, but can also develop into life-threatening illnesses of the lungs (pneumonia), the covering of the brain and spinal cord (meningitis), and the blood (bacteremia).  Pneumococcal disease can be acquired by anyone, but children younger than age 2, adults ages 65 and older, people with certain medical problems, and smokers are at the highest risk.  

Key points about interstitial lung diseases

• Interstitial lung disease is the name for a group of 100 lung disorders that inflame or scar the lungs.
• The cause is not known. Major contributing factors are smoking and inhaling environmental or occupational pollutants.
• The most common symptoms are shortness of breath, especially with activity, and a dry, hacking cough.
• Tests that help measure the lungs’ ability to exchange oxygen and carbon dioxide are used to diagnose the condition. Blood tests and imaging tests may also be used to see how severe the problem is and monitor it over time.
• The goal of treatment for people with the disease is to prevent more scarring and manage symptoms.

Next steps

Tips to help you get the most from a visit to your healthcare provider:

• Know the reason for your visit and what you want to happen.
• Before your visit, write down questions you want answered.
• Bring someone with you to help you ask questions and remember what your healthcare provider tells you.
• At the visit, write down the name of a new diagnosis, and any new medicines, treatments, or tests. Also write down any new instructions your healthcare provider gives you.
• Know why a new medicine or treatment is prescribed, and how it will help you. Also know what the side effects are.
• Ask if your condition can be treated in other ways.
• Know why a test or procedure is recommended and what the results could mean.
• Know what to expect if you do not take the medicine or have the test or procedure.
• If you have a follow-up appointment, write down the date, time, and purpose for that visit.
• Know how you can contact your healthcare provider if you have questions.

Interstitial Lung Disease | Cedars-Sinai

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DIAGNOSIS

TREATMENT

NEXT STEPS

What are interstitial lung diseases?

Interstitial lung disease is the
name for a group of more than 200 lung disorders. Most of them are long lasting, or
chronic. These diseases inflame or scar the lungs. The inflammation and scarring make
it
hard to get enough oxygen. The scarring is called pulmonary fibrosis.

Fibrosis leads to long-term (permanent) loss of your lung tissue’s
ability to carry oxygen. When the scar tissue forms, it destroys the air sacs. It
also
destroys the lung tissue around the air sacs and the lung capillaries.

The symptoms and course of these
diseases may vary from person to person. The common link between the many forms of
the
disease is that they all often start with inflammation in the interstitium. The
interstitium is the supporting structure of the lungs. It’s almost like a scaffolding.
This inflammation then causes inflammation in other parts of the lung, leading to
conditions such as:

• Bronchiolitis.  This is inflammation
  of the small airways (bronchioles).
• Alveolitis. This is inflammation of
  the air sacs where oxygen and carbon dioxide exchange in the blood takes place
  (alveoli).
• Vasculitis. This is inflammation that
  affects the small blood vessels (capillaries).

What causes interstitial lung diseases?

Interstitial lung disease has been
linked to certain diseases, such as sarcoidsosis or rheumatoid arthritis. It can also
be
caused by factors in the environment. Often, its cause is not known.

Major contributing factors are
smoking and inhaling environmental or occupational pollutants, such as inorganic or
organic dusts.

Other factors include:

• Certain illegal drugs or
  medicines
• Certain connective tissue or collagen
  diseases
• Family history
• Radiation treatment

What are the symptoms of interstitial lung diseases?

Symptoms are a bit different for each person. The disease may run a
slow or rapid course. People with the disease may have symptoms that range from very
mild to moderate to very severe. The condition may stay the same for a long time.
Or it
may change quickly. The course of the disease is unpredictable. If it progresses,
the
lung tissue thickens and becomes stiff. Breathing becomes more difficult.

Here are the most common symptoms:

• Shortness of breath, especially with
  activity
• Dry, hacking cough that does not
  produce phlegm
• Extreme tiredness and weakness
• No appetite
• Unexplained weight loss
• Mild pain in the chest
• Labored breathing, which may be fast
  and shallow
• Bleeding in the lungs

These symptoms may look like other
lung conditions or health problems. Talk with your healthcare provider for a
diagnosis.

How are interstitial lungs diseases diagnosed?

A healthcare provider will ask
about your health history and do a physical exam. You may also need pulmonary function
tests. These tests help to measure the lungs’ ability to move air into and out of
the
lungs. The tests are often done with machines into which you breathe. They may include
the following:

Spirometry

A spirometer is a device used to check lung function. Spirometry is one of the simplest,
most common tests. It may be used to:

• Determine how well the lungs receive, hold, and move air
• Look for lung disease
• See how well treatment is working
• Determine the severity of a lung disease
• Find out if the lung disease is
  restrictive. This is when the lungs get stiff and can’t hold as much air. Or if
  the lung disease is obstructive. This is when airflow is disrupted in the
  airways.

Peak flow monitoring

This device is used to measure
how fast you can blow air out of the lungs. Disease-related changes can cause the
large airways in the lungs to slowly narrow. This will slow the speed of air leaving
the lungs. This measurement is very important in evaluating how well or how poorly
the disease is being controlled.

Chest X-rays

This test takes pictures of internal tissues, bones, and organs.

Blood tests

Arterial blood gas may be done
to check the amount of carbon dioxide and oxygen in the blood. Other blood tests may
be used to look for possible infections or other diseases that may cause interstitial
lung disease.

CT scan

This test uses a combination of
X-rays and a computer to make images of the body. CT scans are more detailed than
regular X-rays.

Bronchoscopy

This test is a direct exam of
the main airways of the lungs (bronchi). It uses a flexible tube called a
bronchoscope. Bronchoscopy helps to evaluate and diagnose lung problems, check
blockages, take out samples of tissue or fluid, and help remove a foreign body. It
may include a biopsy or bronchoalveolar lavage.

Bronchoalveolar lavage

This test removes cells from the
lower respiratory tract to help find inflammation and rule out certain causes.

Lung biopsy

This test removes a small piece
of tissue from the lung so it can be checked under a microscope.

How are interstitial lung diseases treated?

Because there are so many causes,
treatment will vary. Some interstitial lung diseases don’t have a cure. Treatment
is
aimed at preventing more lung scarring, managing symptoms, and helping you stay active
and healthy. Treatment can’t fix lung scarring that has already occurred.

Treatments may include:

• Lung transplant
• Medicine taken by mouth (oral),
  including corticosteroids to reduce inflammation and cyclophosphamide to suppress
  the
  immune system. Some medicines protect the lung from more damage and scarring. These
  are called antifibrotics and include nintedanib and pirfenidone.
• Oxygen therapy, from portable
  containers
• Pulmonary rehab

If you smoke or use vaping devices,
ask your healthcare provider for help to stop. Also check with your healthcare provider
about getting the flu and pneumococcal shots. Getting a flu shot every year can help
prevent both the flu and pneumonia. In addition, pneumococcal bacteria can cause minor
problems such as ear infections. But they can also develop into life-threatening
illnesses of the lungs (pneumonia), the covering of the brain and spinal cord
(meningitis), and the blood (bacteremia). Anyone can get pneumococcal disease. But
those
at highest risk include children younger than age 2, adults ages 65 and older, people
with certain health problems, and smokers.  

Talk with your healthcare provider about the pneumococcal vaccine.
The CDC recommends it for all children younger than 2 years old and all adults age
65 or
older.

Key points about interstitial lung diseases

• Interstitial lung disease is the name
  for a group of more than 200 lung disorders that inflame or scar the lungs.
• The cause is often not known. Major
  contributing factors are smoking and inhaling environmental or occupational
  pollutants.
• The most common symptoms are shortness
  of breath, especially with activity, and a dry, hacking cough.
• X-rays and other imaging studies are
  used to diagnose the condition. So are tests that help measure the lungs’ ability
  to
  exchange oxygen and carbon dioxide.
• The goal of treatment is to prevent
  more scarring, manage symptoms, and help you stay active and healthy.
• If you smoke, quitting is critical to help maintain as much lung
  health as possible.
• Ask your healthcare provider about getting the flu
  and pneumococcal vaccines.

Next steps

Tips to help you get the most from a visit to your healthcare provider:

• Know the reason for your visit and what you want to happen.
• Before your visit, write down questions you want answered.
• Bring someone with you to help you ask questions and remember what your healthcare
  provider tells you.
• At the visit, write down the name of a
  new diagnosis and any new medicines, treatments, or tests. Also write down any new
  instructions your healthcare provider gives you.
• Know why a new medicine or treatment
  is prescribed and how it will help you. Also know what the side effects are.
• Ask if your condition can be treated in other ways.
• Know why a test or procedure is recommended and what the results could mean.
• Know what to expect if you do not take the medicine or have the test or procedure.
• If you have a follow-up appointment, write down the date, time, and purpose for that
  visit.
• Know how you can contact your healthcare provider if you have questions.

Medical Reviewer: Allen J Blaivas DO

Medical Reviewer: Marianne Fraser MSN RN

Medical Reviewer: Daphne Pierce-Smith RN MSN

© 2000-2022 The StayWell Company, LLC. All rights reserved. This information is not intended as a substitute for professional medical care. Always follow your healthcare professional’s instructions.

Nonspecific granulomatous inflammation in Crohn’s disease

Crohn’s disease (CD) is a chronic disease affecting all parts of the gastrointestinal tract. Its etiology and pathogenesis are not well understood. Recent studies indicate a significant role of disruption of the antibacterial mechanisms of innate immunity: identification of association with genes responsible for recognition (NOD2) and intracellular processing (ATG16L1, IRGM) of bacterial components [5].

The dominant microscopic signs of the disease are chronic inflammation with the presence of sarcoid-type granulomas and an infiltrate with a predominance of lymphocytes and plasmocytes, often transmural. Granulomas are located in isolation, consist of epithelioid and giant cells of the Pirogov-Langhans type, surrounded by lymphocytes [1, 3]. E. Mooney et al. [10] showed that 46.1% (15.3–90.4%) of granulomas were located near the lymphatic vessels, and 10.1% were located near the blood vessels. The frequency of detection of sarcoid granulomas varies within 20-50%.

According to most authors [8], the leading role in the development of the inflammatory process in CD belongs to the subpopulation of CD4+ T-lymphocytes, which acquire the ability to persist for a long time in the focus of inflammation due to resistance to apoptosis. “Naive” CD4+ T-lymphocytes under the influence of polarizing cytokines IL-12, IL-18, IL-23 and Th27 (IL-17) differentiate in CD into Th2 CD4 T-cells, which give an immune response with predominant production of gamma-interferon ( γ-IFN) and tumor necrosis factor alpha (TNF-α), activation of monocytes and macrophages, leading to the development of an inflammatory process of the type of delayed-type hypersensitivity [7, 8].

The data accumulated in recent years suggest that the defective interaction of the bacterial flora with the innate immune system plays a decisive role in the pathogenesis of CD. It is known that the formation of infectious granulomas, including intestinal infections, occurs in the presence of a specific bacterial component. The frequency of detection of granulomas in surgical preparations of the small and large intestine of patients with CD increased in the distal direction as the spectrum and number of bacteria in the intestinal contents increased: 63% in the small intestine, 72% in the ascending colon, 88% in its descending department and 90% – in the rectum [11]. The presence of granulomas did not correlate with the genotypic features of CD — CARD/NOD2 and ASP 299 Gly in TLR4 (toll-like receptor 4), which are responsible for interaction with certain PAMPs (pathogen-associated molecular patterns) of microorganisms — muramyl peptide structures, LPS, etc. The authors believe that granulomas in CD develop as a result of a chronic immune response to persistent, as yet unidentified, bacterial components [11].

It has been established that macrophages developed from blood monocytes of patients with CD reduce the secretion of pro-inflammatory cytokines and chemokines that regulate the migration of granulocytes to the site of infection. The defect in cytokines is not associated with a violation of gene transcription and translation of the corresponding RNA. Cytokines were subjected to intracellular degradation, and the use of lysosomal inhibitors restored their secretion [12]. Therefore, it is assumed that a violation of granulocyte chemotaxis secondary to a defect in macrophages leads to a decrease in the intensity of inflammation in response to the invasion of the intestinal microflora of the mucous membrane, a decrease in the clearance of bacteria and tissue detritus, which in turn cause T-lymphocyte-mediated chronic granulomatous inflammation [4, 12 ]. The genetic determinism of the activity of the inflammatory process is shown in the model of granulomatous inflammation induced in C57B1/6 and Balb/c mice by the BCG-M vaccine. Inflammation in C57B1/6 mice mediated by Th2 lymphocytes was more active than in Balb/c mice with a Th3 type of immune response [2]

A number of publications [6, 9] are devoted to prospective studies of the clinical significance of granulomas in CD. T. Molnar et al. [9] presented the results of a survey of 56 primary patients with CD, in 25 (44.6%) of which granulomas were found in biopsies of the mucous membrane of the gastrointestinal tract (GIT). In the group of patients with the presence of granulomas, a higher activity of the disease and its more severe course were found: the number of patients with a penetrating form of CD with and without granulomas was 44 and 22.6%, respectively, with extraintestinal manifestations – 40 and 22.6%. However, the severe course of the disease and the occurrence of complications cannot be due to the presence of granulomas. Apparently, the formation of granulomas is observed more often with significantly pronounced inflammatory-destructive changes in the intestine.

The gradually formed notion that sarcoid granulomas should be regarded as the “gold standard” in the diagnosis of CD is not entirely correct. The Commission of Experts of the European Organization for the Treatment and Diagnosis of Crohn’s Disease (ECCO) [13] believes that for diagnosis it is necessary to identify at least one more morphological sign of CD. We share the opinion of A.I. Strukova and O.Ya. Kaufman [3] that granulomas in granulomatous diseases, having certain features, do not have specificity sufficient to be guided by it in morphological diagnosis.

Granulomatous inflammation is a special form of chronic inflammation characterized by the development of granulomas around the damaging agent. In the process of evolution, such inflammation has formed in invertebrates to delimit foreign structures that, due to the size or toxic properties of the agent, cannot be destroyed by phagocytosis. As in higher vertebrates, granulomatous inflammation occurs in response to the action of persistent antigens when they are massively introduced in order to delimit them, creating a barrier in the form of granulomas.

Detection of single granulomas in biopsy specimens or surgical preparations is not a reason to attribute these cases to the group of granulomatous diseases, since it is not granulomas that determine the nature of the disease. The latter are only one of the morphological signs of chronic inflammation. In addition, in CD, granulomas are found in less than half of the cases. This circumstance makes it possible to disagree with the opinion of individual researchers who call CD granulomatous ileitis or colitis.

The foreign body response consists of a series of successive steps including plasma protein adsorption, complement activation, thrombus formation, acute and chronic inflammation, granulation tissue development, foreign body giant cell formation, and fibrous capsule formation. Granulomatous inflammation is a variant of chronic inflammation, in which macrophages, epithelioid and giant multinucleated cells forming granulomas are determined in the cellular infiltrate [14].

In many granulomas in the cytoplasm of giant cells, various inclusions are found in the form of light stellate or rounded bodies or foreign bodies with clear boundaries (Schauman’s bodies).

Our experience in the study of surgical preparations removed for Crohn’s disease indicates that in CD there are various forms of macrophage inflammatory reaction – from the accumulation of macrophages or giant cells of foreign bodies to sarcoid-type granulomas, and foreign inclusions in the cytoplasm of giant cells can have a variety of forms. character.

In our observations, transparent vacuoles of round shape were sometimes found in giant cells (Fig. 1, a), Figure 1. Crohn’s disease. a — giant cells of foreign bodies with transparent vacuoles in the cytoplasm. ×400; b – in the cytoplasm of a giant cell, a crystalline inclusion. Nearby are the Pirogov-Langhans cells. ×200; c — formation of a granuloma around a giant cell of a foreign body. ×200; g – a group of giant cells of foreign bodies in the bottom of the ulcer. ×200. Stained with hematoxylin and eosin. less often, foreign bodies had an elongated shape with clear boundaries (see Fig. 1, b) . In single preparations, it was possible to observe the formation of granulomas around the giant cells of foreign bodies (see Fig. 1, c) . Quite often, giant cells of foreign bodies were found in the bottom of ulcers (see Fig. 1, d) , sometimes they took the form of Pirogov-Langhans cells (see Fig. 1, b) . More often giant cells of foreign bodies and granulomas are found in the subserous layer, less often in the submucosa or muscle layer. Sometimes they are found in the lymph nodes or in the tissue of the mesentery along the lymphatic vessels. Occasionally, accumulations of macrophages and giant cells can be found in the walls of lymphatic vessels. In addition to individual giant cells or their granulomas, sarcoid-type granulomas are found in the intestinal wall. It cannot be ruled out that their occurrence is a reaction to the introduction of particles with peculiar antigenic properties into the intestinal wall.

Detection of various foreign bodies in giant cells indicates the presence of numerous particles with antigenic properties in the intestinal contents. This diversity of foreign body cells is especially well seen in the example of other chronic inflammatory processes, in particular, those occurring in the walls of fistulous passages in chronic paraproctitis.

In these observations, among the inflammatory infiltrate, as a rule, giant cells of foreign bodies are found around particles of food masses penetrating through the internal opening of the fistula (Fig. 2, a). Figure 2. Chronic paraproctitis. a — giant cells around particles of intestinal contents. ×200; b — a giant cell of a foreign body with a transparent vacuole in the cytoplasm. ×400; c — giant cells of foreign bodies are located among the inflammatory infiltrate. One of them has the shape of a Pirogov-Langhans cell. ×200; d – foreign body granuloma with sclerosis symptoms (“sarcoid” granuloma). ×100. Stained with hematoxylin and eosin. Rarely, vacuolar inclusions are found in the cytoplasm of these cells (see Fig. 2, b) . Individual giant cells take the form of Pirogov-Langhans cells (see Fig. 2c) .

In isolated cases, giant cell granulomas with signs of sclerosis are found, which makes them similar to sarcoid (see Fig. 2, d) .

Granulomas in the walls of rectal fistulas, which are very similar to granulomas in CD, convincingly indicate that they are not specific and their detection in the intestinal wall cannot serve as a basis for the diagnosis, as also European CD experts write [13] . Nevertheless, in some cases, these findings can lead to overdiagnosis of CD due to stereotypes.

Changes in the wall of rectal fistulas indicate a significant role of infection and foreign particles coming with food masses in the development of chronic inflammation. This circumstance allows us to assume that similar changes can occur in ulcerative colitis, since food particles can penetrate into the tissues of the intestinal wall in the region of the edges of the ulcers, where deep pockets form. In these cases, conditions are created that are close to those that occur in the fistulous passages.

Conclusion

Comparative morphological study of the tissues of the intestinal wall in such chronic diseases of the colon as CD, ulcerative colitis (UC) and rectal fistulas allows us to consider the formation of giant cells of foreign bodies and their granulomas, as well as sarcoid-type granulomas, as nonspecific. The location and distribution of granulomas along the layers of the intestinal wall indicates that antigenic particles penetrate into it from the intestinal lumen when the mucous membrane is damaged, as evidenced by the presence of granulomas and giant cells in the bottom of ulcers. Their distribution through the layers of the intestine and beyond is due to the migration of foreign agents through the lymphatic vessels and, much less often, through small veins.

Diverse in shape and structure, foreign inclusions in the cytoplasm of giant cells indicate that the contents of the intestine contain numerous particles with different antigenic properties, causing a unified morphological reaction of the tissues of the intestinal wall, which greatly complicates the microscopic differential diagnosis of CD and UC, and in some cases makes it impossible.

Interstitial lung diseases. Causes, symptoms and treatment.!

content

1. What is interstitial lung disease?
2. Causes and symptoms of disease
3. Diagnosis of interstitial lung diseases
4. Treatment of diseases

1. What is interstitial lung disease?

Interstitial lung diseases is the common name for a whole group of lung diseases. What unites the diseases of this category is that they all affect the interstitium, part of the anatomical structure of the lungs.

Interstitium , or interstitial tissue is lung connective tissue . The interstitium provides support to the alveoli, the microscopic air sacs of the lungs. Tiny blood vessels pass through the interstitium and perform the function of gas exchange between blood and air in the lungs. The interstitium tissue is so thin that it is usually not visible on a chest x-ray or CT scan, although interstitial disease may be detected on these tests.

Any damage to the lung tissue causes thickening of the interstitium . Thickening may result from inflammation, scarring, or accumulation of extra fluid (edema). Some forms of lung tissue damage soon pass, while others are chronic and incurable.

Examples of interstitial lung disease would be:

• Interstitial pneumonia due to exposure to bacteria, viruses or fungus.
• Idiopathic pulmonary fibrosis . This is a chronic disease in which fibrosis (scarring) of the interstitium occurs. The causes of idiopathic pulmonary fibrosis are still not exactly known.
• Non-specific interstitial pneumonia is an interstitial lung disease that is often associated with autoimmune diseases such as rheumatoid arthritis or scleroderma.
• Allergic pneumonia is an interstitial lung disease caused by inhalation of dust, mold or other irritants.
• Cryptogenic organizing pneumonia is an interstitial lung disease similar to pneumonia, but without actual infection.
• Acute Interstitial Pneumonia , a severe and sudden interstitial lung disease often requiring life support.
• Desquamative interstitial pneumonia is a lung disease caused in part by smoking.
• Sarcoidosis – a condition that causes interstitial lung disease along with an increase in lymph nodes, and sometimes accompanied by damage to the heart, skin, nerves, organs of vision.
• Asbestosis is a disease caused by exposure of the lungs to asbestos.
• Fibrosing alveolitis .
• Hamman-Rich syndrome and other diseases.

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2. Causes and symptoms of diseases

Causes of interstitial lung diseases.

The causes of lung tissue damage can be various. So, interstitial pneumonia can be caused by bacteria, viruses or fungus. Other interstitial diseases may be associated with regular inhalation of irritants – asbestos, quartz dust, talc, coal and metal dust, grain dust. In rare cases, lung diseases in this group can develop due to exposure to certain narcotic substances .

The peculiarity of interstitial lung disease is that the above factors, in fact, cause only some of the diseases. In most cases the exact cause of lung disease remains unknown .

Symptoms of interstitial lung disease.

The most common symptom of all forms of the disease is shortness of breath, which may worsen over time. In most diseases, shortness of breath develops rather slowly, over a period of about a month. In the case of interstitial pneumonia or acute interstitial pneumonia, symptoms can develop very quickly, in just a few days or even hours.

Other symptoms of illness may be

• Cough, usually dry and unproductive;
• Weight loss;
• Labored breathing.

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3. Diagnosis of Interstitial Lung Disease

Typically, people with interstitial lung disease come to see a pulmonologist for shortness of breath or cough. To make a diagnosis, the doctor usually uses special methods of examination of the lungs :

• X-ray of the chest. This study is usually done primarily to assess the general condition of the lungs. Interstitium lesions may appear on x-ray as thin lines in the lungs.
• Computed tomography (CT). Tomography allows you to create a detailed image of the lungs and structures adjacent to them. Interstitial lung disease is usually seen on CT.
• High resolution CT. Special settings of the tomograph in case of suspected interstitium disease increase the diagnostic efficiency.
• Assessment of respiratory function using special lung tests, including body plethysmography, spirometry, and some others.
• Lung biopsy and examination of the obtained samples under a microscope. This is often the only way to determine what type of lung tissue disease a patient has. Biopsy tissue samples can be taken using bronchoscopy, video-assisted thoracoscopic surgery, or open lung biopsy (thoractomy).

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4.Treatment of diseases

The treatment regimen for interstitial lung diseases is selected by a pulmonologist depending on the type of damage to the lung tissue and its causes. In general, antibiotics can be treated (they are especially effective for most types of bacterial interstitial pneumonia). Viral pneumonia usually resolves on its own and is not treated with antibiotics. Fungal pneumonia, which is extremely rare, is treated with special antifungal drugs.

Another type of medication is corticosteroids which reduce inflammation in the lungs and other parts of the body.