Interstitial pneumonia: symptoms, diagnosis and treatment. Idiopathic interstitial pneumonia How idiopathic and other forms of the disease develop

Interstitial pneumonia is often the result of viral diseases, influenza, that provoke the destruction of lung tissue. Inflammation disrupts the functionality of the lungs, and the exchange of carbon dioxide and oxygen changes. Interstitial pneumonia has an unclear etymology, which is characterized by shortness of breath, dry cough with a small amount of sputum, and general malaise.

Interstitial pneumonia is considered a serious and dangerous lung disease, which is difficult to determine for the pathogen. Inflammation is difficult to treat and often ends unfavorably.

Important! Incorrect therapy leads to patient disability or death.

Forecast

The outcome of the disease directly depends on the manifestation of pulmonary fibrosis and the type of disease. According to statistics, patient survival does not exceed 5 years, and due to the manifestation of pneumosclerosis or cardiopulmonary failure, life expectancy is no more than 3 years. Interstitial pneumonia is a serious disease that, even with proper therapy, has maximum mortality rates are 50-70%.

Improvement and normalization of the general well-being of the patient with interstitial pneumonia occurs only in 75% of cases, the remaining 35% of patients have a 10-year survival rate. In the case of desquamative pneumonia, improvement occurs in 2/3 of cases, 5-year survival is observed in 93%, and 10-year survival in 69%. You can get rid of RB-ILD by completely giving up cigarettes; in certain cases, progression of the disease with subsequent relapses may be observed. Patients are systematically vaccinated against influenza and pneumococcal infection.

Complications

Due to the advanced stage of the disease and with improper treatment of the pathology, against the background of interstitial pneumonia in adults and children, dangerous consequences appear that can be life-threatening. The most common consequences include:

  • Fibrosis – changes in gas exchange occur, irreversible sclerotic processes are observed in the affected area.
  • Respiratory failure
  • Heart failure.

In addition, a bacterial infection may be added to pneumonia, and at a late stage of inflammation, a serious pathology such as lung cancer may occur.

Qualification of pneumonia

There are several forms of interstitial pneumonia:

Additional Information. Smoking is the main cause of respiratory bronchiolitis and desquamative pneumonia.

Causes

Interstitial pneumonia in children and adults is a rare phenomenon, so it has not been thoroughly studied by specialists. The disease can manifest itself as a result of changes in homeostasis on the part of the immune system, and the provoking factor is an antigen, for which the body produces certain antibodies.

Pneumonia occurs under the influence of infectious agents, as well as certain types of dust. In addition, smokers, people who quit smoking, and people with AIDS or HIV are prone to the disease. Lymphoid pneumonia can be caused by immunopathies or autoimmune diseases. The peculiarity of the inflammatory process in interstitial pneumonia is that it is non-infectious in nature. Most often, the connective tissue of the lungs and the walls of the alveoli are destroyed, in which fluid can re-emerge. This type of pneumonia is marked by primary inflammation of the interstitial tissue with the accumulation of cells in it that are immunocomponent. It is these cells that contribute to the release of a damaging mediator at the initial stage of the disease. Fibriogenic factors provoke fibroproliferative reactions at the last stage.

Symptoms of interstitial pneumonia

In the initial stage of the disease the following symptoms are expressed:

  • Febrile fever
  • Significant shortness of breath
  • Cough with a small amount of sputum and streaks of blood.

When listening, harsh breathing is felt, but there is no wheezing. In most cases, rhinitis, conjunctivitis, arthralgia, myalgia, acute headache are manifested, and sometimes kidney or liver damage is observed.

Each form of interstitial pneumonia has different symptoms:

  • Lymphoid pneumonia - shortness of breath, non-productive cough, fever, increased sweating, weight loss.
  • Cryptogenic pneumonia - cough, fever, fatigue.
  • Desquamative pneumonia - gradual onset of cough, loss of appetite, heavy breathing.
  • Idiopathic pneumonia - shortness of breath, cough that does not disappear after taking antitussives, and when listening, crackling and noise are felt.
  • Acute pneumonia – pain in joints and muscles, high temperature, respiratory failure.
  • Nonspecific pneumonia - increasing cough, weight loss, shortness of breath, constant fatigue.

Features of the disease in children

Interstitial pneumonia in children implies an inflammatory process of the connecting areas of the lung and the likelihood of subsequent involvement of bronchioles and alveoli. Viruses and mycoplasmas are the causative agents of the disease. Pneumonia often begins with the manifestation of slight shortness of breath, and not with catarrhal symptoms.

Pneumonia occurs in severe form in children, since the high temperature can persist for more than 10 days. This condition helps to reduce blood pressure, arrhythmia, heart failure occurs, shortness of breath and cyanosis appear, which rapidly

are progressing. Often the cough is mild and produces little sputum. The prognosis may be worsened by the possibility of pulmonary edema and emphysema.

In the case of intermediate pneumonia, it is difficult to determine any significant changes by auscultation and percussion. On the radiograph, children have no lesions, the transparency of the lung tissue increases and the broncho-vascular pattern is significantly pronounced.

Important! Treatment of interstitial pneumonia in a child is always carried out in a hospital with the use of oxygen support.

For infants, the inhalation method of gas administration is used. Antibiotics are selected depending on the pathogen present in the body. Complex treatment consists of restorative techniques and physiotherapy.

Diagnostics

Interstitial pneumonia is characterized by imprecise and vague symptoms, so diagnosing the disease is difficult. To determine the presence of inflammation, perform the following procedures:

  • Measurement of external respiration functions - allows you to determine existing pulmonary ventilation disorders.
  • Chest X-ray – provides information about noticeable changes in the lung area.
  • Blood gas analysis
  • EchoCG, ECG – carried out in case of suspected heart failure
  • Lung biopsy - this procedure allows you to determine the morphological form of pneumonia. A biopsy is performed only if it is impossible to perform other studies, and the disease is rapidly progressing.

Treatment of interstitial pneumonia

Treatment of interstitial pneumonia is based on anti-inflammatory therapy, which involves the use of cytostatics, glucocorticoids, and hormonal agents. The duration of drug treatment varies from 3 months to several years. Radiological and clinical improvements occur in a third of patients.

Determining the effectiveness of therapy is based on combining several indicators with a frequency of 3-6 months:

  1. The strength of cough decreases, shortness of breath disappears
  2. X-ray features are reduced
  3. Blood gas exchange is stabilized.

If there is no dynamics in any direction during the treatment process, this indicates a normalization of the process. If the general condition worsens over six months, the X-ray results do not change - this indicates the ineffectiveness of the chosen technique.

When sclerosis of lung tissue develops, experts recommend the use of oxygen therapy, which can significantly prolong the patient’s life.

Important. Severe cases of the disease require a lung transplant .

When a patient is diagnosed with interstitial pneumonia, many people wonder what it is, what differences does the disease imply from the standard manifestation of pneumonia? The differences are that in the presence of this disease, the interstitium becomes inflamed, then the alveoli of the lungs. In the absence of timely treatment, connective tissue grows, which leads to disappointing prognosis. In our article we will look at the symptoms and treatment of interstitial pneumonia in adults and children.

Since this form of pneumonia does not occur very often, the reasons for its development are not fully understood. According to medical practice, the main causes of the development of the disease are pathogenic bacteria and infections that may be contained in dust. The risk of developing interstitial pneumonia increases significantly in long-term smokers, as well as in people suffering from the human immunodeficiency virus.

Symptoms of pathology

Interstitial pneumonia is divided into several types, depending on which the patient may experience different symptoms. Common signs of the disease include constant drowsiness, severe cough, mostly wet, and general malaise. Let's consider what symptoms may indicate this disease, depending on its type:

  • Acute interstitial pneumonia. Like any other disease, interstitial pneumonia can occur in the acute stage. This suggests that the disease develops very quickly. First, the patient experiences chills due to a significant change in body temperature. Sometimes the mark rises to 40 degrees. Severe shortness of breath and paroxysmal cough occurs. If the acute stage of this disease develops, artificial ventilation may be required until the patient’s condition stabilizes. X-rays show a standard picture: darkening of the lungs on one or both sides (bilateral interstitial pneumonia).
  • Nonspecific interstitial pneumonia. This disease progresses at a very slow speed. In some cases, signs of the disease may persist for several years. There is a constant cough, mainly developing in the morning, shortness of breath occurs when climbing stairs, and general malaise is observed. It is worth noting that this type of disease is typical for older people, especially for those who have been smoking for more than 10 years. During diagnostic examination using an X-ray machine, “ground glass” is observed.
  • Lymphoid interstitial pneumonia. It is worth noting that this type of disease is very rare compared to the others. The chances of a full recovery are high, provided that the necessary treatment is prescribed in a timely manner. According to statistics, the majority of patients are women. Symptoms include general weakness, drowsiness and dry periodic cough.
  • Cryptogenic interstitial pneumonia. The onset of the disease is abrupt. The first symptom is an increase in body temperature. Often the cryptogenic form occurs as a complication after the flu. The disease has a favorable prognosis. With the help of antibacterial drugs, it is cured in 4-8 weeks.
  • Desquamative interstitial pneumonia. The type of illness is typical for experienced smokers. Signs of the disease are scanty: a rare cough, shortness of breath during heavy physical exertion. The symptoms are very easy to confuse with the banal consequences of smoking. In most cases, patients manage to get rid of the disease.
  • Ideopathic interstitial pneumonia. To date, there is not much information about this disease, since the disease occurs quite rarely. The disease begins with a common cold, symptoms such as shortness of breath, cough and chest pain increase gradually. X-rays show a “ground glass” appearance.

How is the disease diagnosed?

It is very difficult to diagnose the first signs of the disease because the characteristic symptoms are similar to other diseases and illnesses. To confirm the presence of usual interstitial pneumonia, you must undergo the following types of examination:

  • X-ray examination of the lungs. Despite the fact that this type of diagnosis is not very informative, you can visually notice changes that signal a pathological change in the organ.
  • Computed tomography (CT). A more informative diagnostic method that helps to identify the structure of an organ and examine changes in the organ in more detail.
  • Electrocardiogram. If interstitial pneumonia is not treated promptly, complications often arise that can affect the heart. An ECG is necessary if complications are suspected.
  • Sputum examination. If the cough is wet, laboratory testing of sputum may be required to determine sensitivity to antibacterial agents.
  • Biopsy. In some cases of particular severity, a histological examination of the organ may be prescribed.

Help: If you notice the first symptoms of the disease, be sure to consult a doctor for diagnosis and treatment.

What complications are possible?

As mentioned above, interstitial pneumonia can lead to undesirable consequences. Against the background of a general illness, the following may occur:

  • Lung fibrosis. The most common complication that occurs when connective tissue grows.
  • Secondary infection. Occurs if the disease is not detected in time. The mucus in the lungs begins to build up and rot, which can lead to sepsis or an abscess.
  • Heart problems. Diseases of the respiratory system often cause complications in the heart. Most often, heart failure or arrhythmias of various etiologies occur.
  • Oncological disease. The most dangerous moment is the development of organ cancer. It is more common in experienced smokers.

It is also worth mentioning that interstitial pneumonia in children and newborns occurs a little differently. High body temperature necessarily signals the development of the disease. Complications in children are quite common. In addition to the above, pulmonary edema or emphysema may occur.

Treatment of the disease

Interstitial pneumonia should be treated immediately after diagnosis. In this case, the chances of a full recovery and avoiding serious complications are significantly increased. It is worth noting that the disease is treated exclusively in inpatient settings.

  • The main therapy is medications from the group of glucocorticosteroids. The drug Prednisolone has proven itself to be excellent.
  • As a complementary therapy, adults and children are prescribed antibacterial agents according to age. For high efficiency, the medicine is administered intramuscularly. Typically, a broad-spectrum drug is used - Ceftriaxone.
  • It is mandatory to take medications that help thin and release sputum.
  • Oxygen therapy is prescribed, which stimulates the restoration of lung tissue and respiratory function.
  • Intramuscular vitamin courses are indicated. This is necessary to stimulate increased immunity. Vitamins of the main groups (A, B, C, D) are usually used.

In addition to the main treatment, adherence to a certain diet is indicated. Against the background of the disease, patients with interstitial pneumonia rapidly lose weight, which is extremely undesirable during treatment. For this reason, the maximum calorie intake is prescribed. Depending on the individual characteristics of patients, nutrition may vary.

Note: The course of treatment for interstitial pneumonia is strictly individual. The duration of therapy depends on the form of the disease, its severity and the individual characteristics of the body.

Folk remedies

Of course, traditional medicine recipes are not capable of ridding a patient of his illness. But still, doctors recommend using them as additional therapy. Some recipes help remove mucus and restore respiratory function. Let's take a closer look:

  • Licorice root must be placed in water and brought to a boil. The resulting liquid is cooled to room temperature. It is recommended to drink 1/3 cup of the decoction three times a day.
  • Peppermint and thyme are finely chopped with a knife and poured with vegetable oil. Leave in a dark place for about a week. Rub the oil on your back and chest every night.
  • St. John's wort leaves are steamed with boiling water. Leave for at least 3 hours. The decoction is taken in a glass throughout the day.
  • If the patient is bothered by a dry cough with a sore throat, warm milk with liquid honey (at the rate of 1 tablespoon per glass of liquid) helps.

Preventive measures

  • Get a flu shot once a year.
  • If symptoms of a cold occur, start treatment on time.
  • Protect yourself from hypothermia.
  • Do breathing exercises daily.
  • Carry out wet cleaning of the premises daily.
  • In crowded places, use a medical mask.
  • Stop smoking.
  • Eat properly.

It is important! Interstitial pneumonia is an insidious disease that can occur in a latent form. Only modern diagnostic methods and laboratory tests of certain tests can detect the disease. If symptoms characteristic of this type of pneumonia appear, do not self-medicate and consult a doctor as soon as possible.

Idiopathic interstitial pneumonia refers to a special type of pneumonia affecting morphological changes in the lungs without an obvious etiology. Pneumonia, popularly called pneumonia, can occur in various forms and manifest itself with mixed symptoms. To identify interstitial pneumonia, it will be necessary to carry out high-quality multilateral diagnostics of the body.

Characteristics of the disease

A specific type of lung disease, interstitial pneumonia is a group of diffuse diseases of the pulmonary system, which is characterized by the absence of visible causes.

The sphere of manifestation is the connective anatomical tissue of the interstitium, which, under the influence of pathogens, begins to thicken, the breathing process becomes rapid and difficult for a person, shortness of breath, dry cough, and sometimes high fever appear. Signs of the disease may vary. The concept of interstitial pneumonia, which is also called interstitial pneumonia, includes such types of lung disease as:

  1. IPF is idiopathic pulmonary fibrosis. Characterized by the formation of so-called “honeycombs” in the lungs, the process of scarring of the interstitium begins. Often observed in people over 50 years of age. The disease can develop over several years, making it difficult to detect and treat.
  2. NSIP - nonspecific interstitial pneumonia. May occur in older people. Accompanied by weight loss, lethargy, shortness of breath, and a slight increasing cough.
  3. Acute interstitial pneumonia develops very quickly and can lead to a sharp deterioration in the patient's condition. Requires immediate connection of devices that ensure lung viability. Body temperature rises, quality of life deteriorates, very severe shortness of breath and chills appear.
  4. Cryptogenic organizing pneumonia, or bronchiolitis. It is characterized by an inflammatory process in the lungs in the absence of infection. Bronchioles are involved along with the interstitium; upon diagnosis, the formation of polyp-like granules is observed in the lungs.
  5. Desquamative interstitial pneumonia is typical for males, especially for male smokers. The first thing to do in this case is to stop smoking and take appropriate medications. This disease is completely curable.
  6. Lymphoid interstitial pneumonia is more often observed in forty-year-old women. It can grow over a period of one to several years. Symptoms: shortness of breath, persistent cough, pain in the joints, chest and back.

And this is not the entire list of possible diseases of the interstitial pneumonia group.

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Childhood pneumonia

Interstitial pneumonia occurs frequently in children, especially in newborns. The cause may be early illnesses of ARVI, bronchitis in the mother, or the penetration of viruses, mycoplasma infections. Children have a very difficult time with this disease due to severe shortness of breath, high fever and weakness.

Parents may not know that a common cough can be not just bronchitis, but also interstitial pneumonia, so the usual drugs for treating a child for bronchitis do not work. If there is the slightest suspicion of pneumonia of any type, you should immediately call an ambulance and hospitalize. In hospital settings, mandatory oxygen therapy is prescribed to maintain lung function. Antibiotics and symptomatic treatment must be used.

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Symptoms and causes

Symptoms of interstitial lung disease:

  • shortness of breath (deep and severe);
  • dry cough, sometimes with a little sputum, often with pus;
  • weight loss;
  • possible increase in body temperature.

For different types of disease, the manifestation of symptoms is characterized by periodicity: symptoms can appear in one day, develop quickly and sharply worsen the condition, or they can last for years. Causes of lung disease:

There are cases when the cause of the disease simply cannot be identified. Also important causes of the disease can be the constant inhalation of chemicals: dust, talc, asbestos. The disease can also occur under the influence of taking antibiotics, such as allergic pneumonia, or while taking narcotic substances.

Nonspecific interstitial pneumonia is probably a separate nosological form. Its incidence and prevalence are unknown, but it is probably the second most common of all forms of idiopathic interstitial pneumonia (accounting for 14 to 36% of reported cases). Most cases are recorded in patients with systemic connective tissue diseases, drug-induced IBLAP or chronic hypersensitivity pneumonitis. In some cases, the etiology of the disease remains unknown.

Symptoms of nonspecific interstitial pneumonia

Symptoms of nonspecific interstitial pneumonia are similar to those of idiopathic pulmonary fibrosis. Most patients are between 40 and 60 years of age. Cough and shortness of breath are present for many months and years.

Diagnosis of nonspecific interstitial pneumonia

X-ray of the chest organs reveals mainly an increase in the pulmonary pattern in the lower sections. Bilateral infiltrates may also occur. HRCT reveals bilateral ground-glass opacities, bilateral foci of consolidation, irregularly shaped linear structures, and bronchial dilatation. Ground glass opacity is the predominant finding in most cases and the only sign of disease in approximately one third of cases.

The main feature of histological changes in nonspecific interstitial pneumonia is the development of homogeneous inflammation and fibrosis, which is the opposite of the heterogeneity of foci of inflammation in ordinary interstitial pneumonia. The lesions are usually identical, but the process can be focal, with separate areas of the intact lung. Cellularity is rare.

Treatment of nonspecific interstitial pneumonia

Most patients have a good prognosis after treatment with glucocorticoids.

What is the prognosis for nonspecific interstitial pneumonia?

Nonspecific interstitial pneumonia has a poor prognosis. Relapses may occur. In some patients the disease progresses; in this case, their average life expectancy is 5 to 10 years after diagnosis. The estimated ten-year mortality rate is less than 15-20%.

Idiopathic interstitial pneumonia is an inflammatory disease of the lungs that primarily affects the connective tissue around the alveoli (interstitium).

The group of nosologies united by this term includes many diffuse parenchymal lung diseases. All of them have a number of common features: unclear etiology, similar clinical picture and radiological signs. Therefore, their diagnosis is difficult, and only the results of histological examination help to distinguish these diseases from each other.

The interstitium, in which the inflammatory process occurs, is located in the partitions between the alveoli of the lung and the blood vessels.

Due to the action of the infectious agent, swelling occurs, which leads to disruption of gas exchange.

If the disease is protracted, the lung tissue is subject to irreversible fibrosis.

Kinds

The division of interstitial pneumonia into groups is based on the morphological changes that occur in the lung tissues. According to them, the following types are distinguished:

  • nonspecific interstitial pneumonia;
  • cryptogenic organizing pneumonia;
  • desquamative;
  • lymphoid;
  • respiratory bronchiolitis;
  • acute interstitial pneumonia;
  • idiopathic pulmonary fibrosis.

The correct morphological diagnosis can only be established with the help of a lung biopsy, which is done openly or during thoracoscopy. Most often, due to the severe general condition of patients, this diagnostic method is not used.

Symptoms

There are several common symptoms that unite all types of interstitial pneumonia:

  • cough - usually a paroxysmal cough, dry, unproductive, sometimes it is possible to release a small amount of mucous or mucopurulent sputum;
  • shortness of breath - worries all patients, but has different severity;
  • symptoms of general weakness and malaise.

Nonspecific

The onset of nonspecific interstitial pneumonia is gradual, with symptoms present for many years and progressing over time. Patients also complain of weight loss, constant physical weakness and poor exercise tolerance.

Diagnosis of the disease takes time, and it is often possible to establish the correct diagnosis only 3-5 years after the onset of the disease. Men and women over 40 years of age are more likely to get sick; smokers and people who already suffer from connective tissue diseases are more predisposed to NIP.

Using radiography, symmetrical lung lesions in the periphery (“ground glass”) are determined.

Acute

This type of pneumonia begins acutely, flu-like - the temperature rises, chills and muscle pain occur. Severe shortness of breath develops very quickly, which leads to respiratory failure and in severe cases requires mechanical ventilation. The course of the disease is similar to adult respiratory distress syndrome; X-rays show bilateral diffuse darkening of the lungs characteristic of this syndrome.

The prognosis is unfavorable, with a high mortality rate.

Cryptogenic organizing pulmonary inflammation

It also has a flu-like onset. The inflammatory process extends not only to the interstitium, but also to the alveolar tissue. On auscultation, you can hear crepitus, characteristic of lobar pneumonia, so the disease is often treated unsuccessfully with antibiotics.

The prognosis is favorable; with adequate treatment, recovery occurs within 3-4 months.

Desquamative

A rather rare type of pneumonia, it occurs more often in male smokers over 40 years of age. The disease progresses gradually, and the bronchioles and alveoli are involved in the inflammatory process.

Dry cough and shortness of breath progress very slowly, may not alarm the patient and are often explained by the consequences of smoking.

The appearance of “frosted glass” on an x-ray is characteristic. The prognosis is favorable, complete recovery is possible.

Lymphoid

A gradually developing disease that is rare and more common in older women. In addition to the typical symptoms of cough and shortness of breath, fever, joint pain, and weight loss may occur.

During the examination, alveolar-interstitial infiltrates are revealed. The prognosis is favorable.

Idiopathic pulmonary fibrosis

A disease with a gradual onset and prolonged increase in symptoms. On auscultation, bilateral crepitus is heard, and on x-ray there is a “ground glass” pattern.

Respiratory bronchiolitis

Associated with smokers and characterized by damage to the small airways (bronchioles). Symptoms are nonspecific.

Possible complications of interstitial pneumonia:

  • The development of pulmonary fibrosis is a pathological replacement of normal lung tissue with connective tissue that occurs during a long course of the disease. As a result, elasticity and gas exchange are disrupted.
  • The addition of a bacterial infection occurs due to congestion in the lungs and general exhaustion of the body. Abscesses, empyema may occur, and distant purulent metastases may develop.
  • Respiratory failure is a consequence of pulmonary fibrosis. It manifests itself as a decrease in tolerance to physical activity, cyanosis, and symptoms of “drumsticks” and “watch glasses”.
  • Heart failure - due to respiratory failure, hypertrophy of the right ventricle develops, the so-called “pulmonary heart”.
  • Lung cancer: occurs in the later stages of the disease as a consequence of prolonged action of inflammatory factors, especially often observed in patients who smoke.

Diagnostics

The symptoms are quite vague and nonspecific, so diagnosing the disease is difficult. In order to recognize the disease, use:

  • measurement of external respiration function - restrictive disorders of pulmonary ventilation are detected;
  • blood gas analysis - characterized by hypoxemia and hypocapnia, which lead to alkalosis;
  • X-ray of the chest organs - allows you to see characteristic changes in the lungs (infiltrates, areas of “ground glass”, darkening);
  • computed tomography is the most accurate method;
  • ECG and EchoCG – used if the development of heart failure is suspected;
  • Lung biopsy is an invasive diagnostic method that is rarely used, but allows you to establish the exact morphological type of pneumonia (used in cases where it is impossible to establish a diagnosis in another way, and the disease has a malignant course and requires quick selection of the correct therapy; a biopsy is also indicated if it is necessary to carry out a differential diagnosis of sarcoidosis or lymphoma).

Treatment methods

Idiopathic interstitial pneumonia is treated with the following methods:

Non-drug methods:

  • quitting smoking helps improve the prognosis, especially in patients with respiratory bronchiolitis and desquamative interstitial pneumonia;
  • Exercise therapy – breathing exercises are useful to improve lung ventilation and prevent the development of respiratory failure;
  • oxygen therapy, mechanical ventilation – used in the later stages of the disease with severe respiratory failure.

Medication methods:

  • Cytostatics: Used when patients do not respond to corticosteroid therapy. Methotrexate and cyclophosphamide are commonly used; second-line drugs are colchicine, glutathione and cyclosporine.
  • Combination treatment: to achieve the best effect, it is recommended to combine corticosteroids with cytostatics.
  • Mucolytics: there is evidence that supports the use of N-acetylcysteine ​​in combination with basic treatment.

Therapy lasts 6 months, after which its effectiveness is assessed (reduction of clinical symptoms, disappearance of radiological signs and improvement of blood gas composition are taken into account). If the results are positive, the same regimen is followed for another 12 months. If treatment does not have an effect, colchicine and interferon are prescribed in addition.