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Idiopathic pulmonary fibrosis. Idiopathic pulmonary fibrosis (IPF): a modern approach to classification and diagnosis Participation in clinical trials on IPF

Idiopathic pulmonary fibrosis (IPF) is one of the most common IIP diseases. The picture of IPF in 1960 was described by Scadding, and he also introduced the term "fibrosing alveolitis" for the first time. It is possible that the very first description of IPF belongs to Rindfleisch, who described in 1897 "cystic cirrhosis of the lungs" - a lung disease characterized by thickening and wrinkling of the lung parenchyma and the formation of a "honeycomb lung".

The ATS/ERS International Conciliation Document (2000) proposes the following definition of ILF: IPF is a specific form of chronic interstitial fibrosing pneumonia that is limited to the lungs and is associated with the histological pattern of ordinary interstitial pneumonia on surgical (thoracoscopic or open) lung biopsy.

In our country, synonyms for IPF are "idiopathic fibrosing alveolitis" (ELISA) and "cryptogenic fibrosing alveolitis", which has become more common in the UK. The concepts of "idiopathic" and "cryptogenic", despite a slight semantic difference, are currently considered to be synonymous, denoting the hidden, unclear nature of the disease.

ELISA (synonyms: Hamman-Rich disease or syndrome, Skedding syndrome, diffuse progressive interstitial pulmonary fibrosis, fibrous dysplasia of the lungs, etc.) is a peculiar pathological process characterized by progressive damage to the interstitial tissue of the lungs, inflammation and fibrosis of the pulmonary interstitium and air spaces, disorganization of structural and functional units of the parenchyma, which leads to the development of restrictive changes in the lungs, impaired gas exchange.

Etiology unknown. Among the possible etiological factors are smoking, certain types of silicate dust. The viral nature of the disease and genetic predisposition are discussed.

Pathogenesis remains unclear. The main pathogenetic mechanism that determines the clinical picture is the development of the alveolar-capillary block. The degree of its severity largely depends on the degree of decrease in the diffusion capacity of the lungs and, accordingly, the severity of arterial hypoxemia, respiratory failure and their clinical manifestations.

The decrease in the diffusion capacity of the alveolar-capillary membrane is associated primarily with fibrosis of the interalveolar septa and the loss of respiratory functions by the alveolar epithelium due to its metaplasia into cubic. However, the resistance of the alveolar-capillary membrane to gas exchange is only half of the total diffusion resistance. The decrease in the diffusion capacity of the lungs largely depends on the degree of perfusion impairment, which is due to a decrease in the contact surface of the alveolar air with the blood of the alveolar capillaries and a reduction in the contact time. These mechanisms, as well as reflex vasoconstriction of the lungs due to endocapillary hypoxia, contribute to an increase in pressure in the pulmonary artery (Euler-Liljestrand reflex) and the development of cor pulmonale. The proportion of veno-arterial bypass is relatively small - about 6%.

It is assumed that in the interstitial tissue of the lungs, the breakdown of collagen decreases and its synthesis by fibroblasts and alveolar macrophages increases. An increase in collagen synthesis is facilitated by an increase in the number of individual subpopulations of lymphocytes that react to lung tissue collagen as a foreign protein and produce lymphokines that stimulate collagen formation. It is also important to reduce the production of "inhibitory factor" by lymphocytes, which under normal conditions inhibits the synthesis of collagen. Many authors attribute Hamman-Rich syndrome to autoimmune diseases in which the functional activity of T-suppressors is inhibited, which leads to hyperproduction of various classes of immunoglobulins by B-lymphocytes. The antigen-antibody complexes (CEC) formed in the blood are deposited in the walls of small vessels of the lungs. The main reason for the long-term persistence of the CIC is a defect in the functional activity of IgG Fc fragments. Under the influence of the CEC, lysosomal fragments of alveolar macrophages and neutrophils, damage to the lung tissue occurs, thickening of the interalveolar septa, obliteration of the alveoli and capillaries by fibrous tissue.

Currently, the most attractive hypothesis is that IPF is an “epithelial-fibroblastic” disease. According to this model, the complex interaction between damage to epithelial cells and mesenchymal cells leads to dysregulation of repair mechanisms with excessive production of profibrotic cytokines, extracellular matrix, and impaired angiogenesis.

Pathological anatomy. Histological changes in the lung tissue vary, which depends not only on the characteristics of the process itself in a particular patient, but also on the phase (stage) of the disease.

There are 5 degrees of pathomorphological changes in the lung tissue in patients with IPF:

I degree: swelling of the interalveolar septa, cell infiltration, capillary tortuosity.

II degree: exudation of serous fibrous fluid (rich in protein and stained with eosin) and cellular exudation into the alveoli, resulting in obliteration of the alveolar space (intraalveolar fibrosis). Another way of organizing alveolar exudate is its resorption into the interalveolar septa with compaction and fibrosis of the latter. Both of these options can coexist.

III degree: involvement in the process of bronchioles with the formation of small cysts and the destruction of the structure of the alveoli.

IV degree: the normal structure of the lung tissue is completely disrupted, the cystic cavities gradually increase.

V degree: the formation of the so-called "honeycomb (or cellular) lung." The cysts are up to 1 cm in diameter.

Clinical symptoms: most often ELISA occurs in the age range from 40 to 49 years. Male to female ratio 2:1

There are no pathognomonic, characteristic only for ELISA, signs of the disease. The onset may be imperceptible or associated by patients with an acute respiratory infection, influenza, and is manifested by the occurrence of shortness of breath with moderate physical exertion. Steadily progressive dyspnea- one of the most characteristic and permanent signs of ELISA. Sometimes, as the first sign of the disease, patients note a cough (dry or with scanty mucous sputum), which is then joined by progressive shortness of breath. As the disease progresses, the cough may intensify and be accompanied by pain in the chest neurological nature. A typical complaint is the inability to take a deep breath.

In some patients, the first manifestation of the disease may be an increase in body temperature up to 38-39 ° C, only then there is shortness of breath and cough. About 5% of patients note periodic hemoptysis.

One of the signs of the disease, indicating (along with others) the progression of the pathological process in the lungs, is weight loss.

Arthralgia (including morning stiffness of the joints), muscle pain, intermittent increase in body temperature to subfebrile or febrile figures, Raynaud's syndrome are observed in half of patients with ELISA. Such a high frequency of joint damage is an additional argument for the involvement of autoimmune disorders in the pathogenesis of this pathology. All patients have weakness and fatigue.

When examining a patient, cyanosis of varying severity (from acrocyanosis to diffuse) attracts attention. The degree of its severity depends on the severity of the disease. In the early stages of the chronic course of the disease, cyanosis can appear only with physical activity but it gets worse as the disease progresses. In acute forms of the disease, cyanosis is one of the early signs.

In patients, the change in the nail phalanges associated with chronic hypoxia (a symptom of "drum sticks" and "watch glasses") attracts attention. The rate of formation of these symptoms depends on the activity, duration of the pathological process and the severity of respiratory failure.

With percussion of the lungs over the affected area, dullness of the percussion tone is noted (more often these are the lower sections of the lungs).

On auscultation, inspiratory crepitus is heard (usually at the height of inhalation). This sound phenomenon is referred to in the literature as "cellophane crackling". Often this is a bilateral crepitus, it is better heard along the posterior and middle axillary lines, as well as between the shoulder blades. Crepitus is not always a permanent symptom of ELISA. At acute form diseases crepitus can be heard even with a normal x-ray picture, at the same time it may not be in a chronic course and changes in the x-ray; it can disappear with adequate therapy.

A characteristic auscultatory sign of ELISA is weakened vesicular breathing (shortening of the inspiratory and expiratory phases). Harsh breathing, dry wheezing can occur when endobronchitis is attached. In the presence of pulmonary hypertension there is an accent of II tone over the pulmonary artery.

As the disease progresses, signs of respiratory failure and cor pulmonale appear: diffuse gray-ash cyanosis, accent II tone over the pulmonary artery, tachycardia, gallop rhythm, swelling of the jugular veins, peripheral edema (all signs of circulatory failure of the right ventricular type appear). A decrease in body weight of patients up to the development of cachexia is a characteristic sign of the terminal stage of IPF.

Pulmonary fibrosis is a pathology characterized by the appearance of fibrous tissue in the organ. The latter is otherwise called fibrous. It is quite strong and in the course of the disease replaces the lung tissue. The disease is also characterized by the obstructed movement of oxygen through the alveoli. A patient with fibrosis has serious respiratory failures.

The consequences of this disease are quite unpredictable. The patient should carefully understand what pulmonary fibrosis is, what are the symptoms of the disease and how to treat it.

Varieties of the disease

The classification of pathology in the lungs is extensive.

Judging by the reasons for its formation, two types of ailment can be distinguished:

Idiopathic.
Interstitial.

The idiopathic form of the disease is the most common. Moreover, the exact reasons for its occurrence remain a mystery. According to statistical data, one can only judge the influence of genetics and adverse environmental conditions.

Typically, idiopathic fibrosis affects the lungs of males. The age category of the latter is 50-60 years. Treatment is based on nintedanib (Vargatef), which only a doctor can prescribe.

Negative factors serve as a catalyst for the development of interstitial fibrosis. This form of the disease has its subspecies. You can see the reasons for their occurrence in the table below.

Name of the disease	Reasons for development
Post-radiation fibrosis of the lungs	The impact of radiation therapy
dusty	Regular intake of dust in the respiratory tract
connective tissue	Pathology connective tissue
Infectious	Transferred infectious disease
Drug	Regular intake of medications
peribronchial	Inflammatory processes in the chronic stage

By the amount of pronounced fibrous tissue, the pathology is as follows:

Pneumofibrosis. The disease is characterized by a moderate alternation of connective tissue with lung tissue.
Pneumosclerosis. In addition to explicit substitution, organ compaction is diagnosed.
Cirrhosis of the lung. Fibrous tissue completely replaced the lung tissue. Vessels and bronchi are damaged.

Pneumofibrosis of the lungs can be local or diffuse types. Local pneumofibrosis is characterized by the presence of individual foci in the affected organ. With a diffuse variety of pathology, a violation of lung ventilation occurs, the density of the latter increases, and their shape and structure change.

Another classification of the disease is linear and basal. Linear pulmonary fibrosis develops as a result of past infections, tuberculosis, etc.

Why fibrosis of the roots of the lungs occurs is not completely understood. It is usually diagnosed against the background of pneumonia or bronchitis. As an independent disease, it is quite rare.

Note: regardless of the type of disease, transform fibrous tissue back to the lung is impossible.

Signs of pathology

With pulmonary fibrosis, symptoms appear gradually as the disease progresses. If fibrosis affects a specific area (left or right), the pathology proceeds without any signs. For other cases, the main symptom that can be diagnosed almost immediately is shortness of breath. Difficulty breathing at first after exercise. Over time, even at rest, it is difficult for a patient with pulmonary fibrosis to breathe.

The following symptoms gradually develop:

Cough (at first dry, then secretion begins).
Blueness of the skin (most of all on the fingers and oral mucosa).
Deformity of the fingers (occasionally).

With a prolonged course of the disease from the heart and blood vessels, some symptoms also occur.

Here they are:

Swelling of the lower extremities.
Increased heartbeat.
Bulging of the veins in the neck.
Pain in the chest.
Listening to pulmonary rales.
Excessive sweating.
Excessive fatigue, increased fatigue.

At an early stage, lung fibrosis is diagnosed in only 20% of patients. If you find any of these symptoms, you should get the advice of a competent specialist.

Diagnostic methods

To identify the pathology, the doctor will need to perform the following actions:

Collect and analyze history.
Check the available signs.
Assess the current state of the patient.
Conduct a full inspection.

Not the last role is played by laboratory research. To check the amount of oxygen contained in the patient's blood, the specialist will perform oximetry. General analysis blood will help to identify the leukocytosis that has developed due to a secondary infection.

The clinical picture of the disease will become more apparent when using various diagnostic methods.

These are:

Radiography.
Taking a biopsy of the affected organ.
CT scan.

The doctor also performs auscultation thoracic. Conducting fluorography allows you to identify focal or diffuse enhancement of the pulmonary pattern. Sometimes there are cystic lucencies on the pictures. It is possible to identify pulmonary hypertension by means of ECHO KG.

Video

Video - idiopathic pulmonary fibrosis

Getting rid of pathology

Treatment of pulmonary fibrosis should be carried out under the supervision of a physician. At the first stage, an integrated approach is needed, consisting of medicines, physiotherapy exercises, diet. All activities are carried out in order to stop the signs of pneumofibrosis and make life easier for the patient. If the disease is detected at a late stage, the prognosis is disappointing. It is important to understand that it is impossible to completely cure this type of pathology.

Only a specialist can explain how to treat pneumofibrosis. If the problem arose as a result of inflammatory processes, the patient is prescribed antibiotics and drugs that stop inflammation. To reduce the rate of formation of connective tissue, Veroshpiron is prescribed.. Its reception is designed for a long period. Careful study of the instructions for use is necessary.

Treatment of pneumofibrosis sometimes comes down to surgical intervention, namely, to a lung transplant.

Be sure to reduce the impact of factors that can provoke deterioration. Workplace the patient should be equipped with high-quality ventilation.

Breathing exercises

Physical activity within reasonable limits contributes to the speedy relief of the symptoms of the disease.

regular cycling;
morning run;
hiking in the open air;
breathing exercises.

The latter saturates the blood with oxygen and normalizes the work. respiratory system. A set of exercises helps to improve sputum discharge and relax the muscles that are involved in breathing.

Respiratory gymnastics for pulmonary fibrosis is performed in a standing position.

The main exercises of this warm-up are:

It is necessary to inhale slowly, while sticking out the stomach. On exhalation, it should be pulled in as much as possible. The chest is calm.
Inhale deeply and smoothly so that the stomach remains motionless. On exhalation, the sternum descends, on inhalation it rises.
The full breathing exercise is the final one. Inhalation begins with the involvement of the peritoneum. With the abdomen fully bulging, continue to inhale using the chest. Exhalation occurs in the same order: first the abdominal region, then the sternum. All transitions are slow and smooth.

Reach desired effect succeed with a daily repetition of each exercise 5-6 times.

Folk remedies

In addition to drug therapy, it makes sense to try home methods. Treatment folk remedies effective in many diseases. It is important not to forget about the need for parallel reception medicines.

Only a doctor can designate the means of adjuvant therapy. Otherwise, it is possible that allergic reactions which will only make pulmonary fibrosis even more dangerous.

Treatment is based on the maximum removal of sputum and mucus from the respiratory system. This can help infusions and decoctions of medicinal herbs.

It is advisable to get rid of pneumosclerosis with the help of wild rose and elecampane roots, which will help cleanse the lungs and restore lung tissue. To prepare a decoction, mix herbs in equal proportions (1 tablespoon each) and pour water (about 1.5 cups). Boil the resulting mixture for 10-20 minutes, cool and strain. Take the decoction daily for at least 2 months.

Normalize lung function and prevent the development of cancer in a state of rosemary. It is necessary to grind the branches and fill them with clean water (in equal amounts). Hold the resulting mixture in the oven for about 45-60 minutes, then add a little honey. Take twice a day for 1 tsp.

You can get rid of shortness of breath and minimize coughing with the help of flax seeds. It is necessary to mix the main ingredient and boiling water in equal amounts, let it brew for 30 minutes. Take 1/3 cup three times a day for a week.

The effectiveness of alternative therapy has been proven exclusively on early stage lung pathology. In more advanced cases, only medicines can alleviate the situation.

How many people live with the disease

By how severe the disease is, one can judge the overall prognosis. In a patient with pulmonary fibrosis, life expectancy depends on the stage at which the pathology was detected.

The acute form of the disease is almost not amenable to any conservative therapy.The lethal outcome occurs literally after a couple of months.

Pathology in a child

Pulmonary fibrosis in children under 3 years of age is quite rare. The causes of the development of the disease are similar to those that provoke the disease in adults.

It is possible to diagnose pathology in the lungs by using the usual methods ( x-ray, biopsy, MRI, CT). Sometimes you may additionally need to consult a pediatric pulmonologist.

Self-treatment can be based only on the elimination of the main irritants (dust, sand, etc.). The doctor will recommend antibiotic therapy to prevent infections in the lungs.

Inhalation procedures that increase the oxygen content in the patient's blood are also important. Only the doctor decides which inhalers can be used.

Candidiasis of the lungs

This is the name of the defeat of the organ tissue by yeast-like fungi. The disease often occurs against the background of lung cancer.

The disease is characterized by the formation of small foci of inflammation. Their main localization is the middle and lower parts of the body. There is a lesion of the lumen of the small bronchi, the accumulation of leukocyte inflammatory fluid in them.

The patient is concerned about:

Severe cough (usually dry).
Dyspnea.
Increase in basal body temperature.
Soreness of the chest.

Getting rid of the disease is based on the use of antimycotic drugs.

More about medicines

Treatment of idiopathic pulmonary fibrosis is usually based on the use of a drug such as Vargatef. This drug is a triple angiokinase inhibitor. A number of clinical studies have confirmed the effectiveness of this tool.

Some time later, Vargatef successfully established itself as a drug to combat lung cancer. However, nintedanib must be used in conjunction with docetaxel. Evaluation of the effectiveness of Vargatef was carried out on the basis of many studies in which about 1.5 thousand patients participated. According to the results, life expectancy and overall survival rate among patients increased significantly.

Of course, only a competent specialist is entitled to prescribe such a drug, based on the results of the examination.

Note: Vargatef is quite expensive. medicines. It is not always possible to find it in pharmacies of our country.

With pulmonary fibrosis, treatment can only be prescribed by a qualified doctor, taking into account the patient's age and symptoms. Self-medication is extremely unacceptable! The key to successful therapy is timely access to a doctor.

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Dr. Toby Maher, Research Fellow, UK National Institute for Medical Research, Consultant Physician, Royal Brompton Hospital, London

Idiopathic pulmonary fibrosis is a progressive disease of unknown origin, which is characterized by gradual scarring, replacement of healthy lung tissue with an inevitable final, pulmonary insufficiency.

In our article today, we will talk about idiopathic pulmonary fibrosis, its diagnosis and treatment, as well as the prospects for combating the disease.

Dr. Toby Maher is a Research Fellow at the National Institute for Medical Research in the UK and Consultant Physician at the Royal Brompton Hospital (London). Lecturer at Imperial College London.

Dr. Maher is a specialist in interstitial lung disease and sarcoidosis.

His research interests include the development of new biomarkers for pulmonary diseases, clinical trials of new drugs, and the study of the pathogenesis of idiopathic pulmonary fibrosis (IPF).

Previously, Dr. Maher was Editor-in-Chief of Respirology and Editor of PLOS One. He is on the board of editors of the prestigious journal Lancet Respiratory Medicine. Author of more than hundreds of articles and publications.

- Dr. Maher, what is idiopathic pulmonary fibrosis?

- Idiopathic pulmonary fibrosis (IPF) is a serious fatal disease that affects 3 million people worldwide.

Although pulmonary fibrosis kills more people each year than some types of cancer, the disease is often overlooked even by physicians, and scientists know surprisingly little about IPF.

With IFL, gradual scarring occurs, and the gas exchange function of the lungs decreases. As the disease progresses, organs and tissues receive less and less oxygen, and respiratory failure develops.

If at first there is shortness of breath only during exertion, then over time, the life of patients with IFL becomes a daily struggle. Even the simplest things, such as taking a shower or getting dressed, require superhuman efforts from them.

The rate of progression of IFL is not the same. On average, every year, 1 in 20 patients experience a catastrophic worsening of the disease. Episodes of exacerbation require hospitalization and intensive treatment: in 50% of cases of exacerbation, IFL kills patients within 30 days.

In general, the prognosis for idiopathic pulmonary fibrosis is poor. The average life expectancy without treatment is 2-3 years from the time of diagnosis. Five-year survival does not exceed 20%; this figure is comparable to lung adenocarcinoma.

- Does early diagnosis of IFL improve prognosis?

- Indeed, early accurate diagnosis of idiopathic pulmonary fibrosis is very important: patients receive adequate treatment in a timely manner and maintain a high quality of life longer.

Unfortunately, the similarity of symptoms of IFL and other more common lung diseases (asthma, COPD) makes diagnosis very difficult. In half of the cases of IFL, patients are initially misdiagnosed.

As a result, the average time between the onset of the first symptoms of idiopathic pulmonary fibrosis and the diagnosis of IPF is on the order of 1-2 years.

Two wasted years!

All this time, patients unsuccessfully struggle with a non-existent disease until they turn to a specialized center where there is experience in diagnosing interstitial lung diseases.

Rapid access to such centers and specialists is critical for accurate diagnosis and early onset of correct drug treatment IFL.

We must understand that idiopathic pulmonary fibrosis is an incurable disease, so psychologists are needed to solve the emotional problems that arise after hearing the diagnosis.

The latest global survey on idiopathic pulmonary fibrosis (IPF) published by Boehringer Ingelheim found that 49% of patients experience "anxiety" and 45% "fear" after diagnosis. Their feelings can influence life decisions, so professional help is needed for such patients.

What is the treatment for idiopathic pulmonary fibrosis? How can modern medicine help patients if IFL is incurable?

- Although there is no cure for pulmonary fibrosis, various options are offered to slow IPF, relieve symptoms, and improve quality of life.

This includes antifibrotic drugs, oxygen, antitussives and bronchodilators, rehabilitation interventions, and end-of-life palliative care.

Until recently, new drugs for the treatment of IPF have not appeared. This has changed with the introduction of the antifibrotic drugs pirfenidone and nintedanib in the US and EU. These medicines can slow the progression of the disease.

Non-drug options help improve the well-being and quality of life of patients. The pulmonary rehabilitation program is built around exercise and includes a whole team of specialized specialists, physiotherapists.

In addition to improving physical fitness and exercise tolerance, we inform patients how to live with IFL, what can and cannot be done, and support them in difficult times.

Several large studies have confirmed that pulmonary rehabilitation achieves its goals and allows patients to lead more fulfilling lives.

As I said, 1 out of 20 patients with IFL annually has a severe worsening of symptoms, leading to a hospital bed. Currently, there are no reliable therapeutic options that significantly improve outcomes in such crises (we usually give corticosteroids and antibiotics).

- How do you see the future of treatment of idiopathic pulmonary fibrosis?

- Over the past couple of years, science has advanced a lot in understanding the pathogenesis, clinical picture and promising targets for the treatment of IPF.

I hope that the future will bring good news to millions of patients and their families.

Most importantly, there is growing awareness of the importance of early diagnosis and treatment of pulmonary fibrosis. New specialized centers are being created, a new generation of doctors who understand the intricacies of IFL are studying. In many countries, a well-coordinated system of care for such patients is being formed.

Patients themselves realize the positive shifts and the importance of scientific research.

That same Boehringer Ingelheim global survey shows that 20% of patients with idiopathic pulmonary fibrosis (IPF) continue to live in the hope of future advances in the fight against their disease. Indeed, research funding is gradually increasing, and the success of this policy is now evident.

Today, clinical trials of new drugs are being conducted everywhere, which offer a hand of hope to seriously ill patients. We have a number of ongoing and planned trials: new drugs, combinations of already known drugs, diagnostic and therapeutic biomarkers.

: Master of Pharmacy and Professional Medical Translator

Idiopathic pulmonary fibrosis (cryptogenic fibrosing alveolitis) is the most common form of idiopathic interstitial pneumonia, corresponding to progressive pulmonary fibrosis and predominant in male smokers. Symptoms of idiopathic pulmonary fibrosis develop over months to years and include dyspnoea on exertion, cough, and wheezing.

The diagnosis is established by analysis of the history, physical examination, chest x-ray, and pulmonary function tests and is confirmed by HRCT, lung biopsy, or both, if necessary. No specific treatment for idiopathic pulmonary fibrosis has been shown to be effective, but glucocorticoids, cyclophosphamide, azathioprine, or combinations of these are often prescribed. In most patients, deterioration occurs even during treatment; median survival is less than 3 years from the time of diagnosis.

ICD-10 code

J84.1 Other interstitial lung diseases with mention of fibrosis

Causes of idiopathic pulmonary fibrosis

Idiopathic pulmonary fibrosis, histologically defined as ordinary interstitial pneumonia, accounts for 50% of cases of idiopathic interstitial pneumonia and occurs in both men and women aged 50 to 60 years in a ratio of 2:1. Continued or past smoking is highly correlated with the disease. There is some genetic predisposition: a burdened family history is noted in 3% of cases.

Although idiopathic pulmonary fibrosis is referred to as pneumonia, inflammation probably plays a relatively minor role. Environmental, genetic, or other unknown factors are thought to initially cause damage to the alveolar epithelium, but the proliferation of specific and aberrant interstitial fibroblasts and mesenchymal cells (with collagen deposition and fibrosis) likely underlies the clinical course of the disease. The key histological criteria are subpleural fibrosis with foci of fibroblast proliferation and areas of marked fibrosis interspersed with areas of normal lung tissue. Widespread interstitial inflammation is accompanied by lymphocytic, plasmacytic and histiocytic infiltration. Cystic dilatation of the peripheral alveoli ("honeycomb lung") is found in all patients and increases as the disease progresses. This histological pattern is uncommon in IBLARB of known etiology; The term usual interstitial pneumonia is used for idiopathic lesions with no apparent cause.

Symptoms of idiopathic pulmonary fibrosis

Symptoms of idiopathic pulmonary fibrosis usually develop over 6 months to several years and include dyspnea on exertion and a non-productive cough. General symptoms(increased body temperature to subfebrile figures and myalgia) are rare. The classic sign of idiopathic pulmonary fibrosis is sonorous, dry bilateral basal inspiratory fine bubbling rales (reminiscent of the sound of opening a Velcro fastener). Thickening of the terminal phalanges of the fingers is present in approximately 50% of cases. The rest of the examination remains normal until the development of the terminal stage of the disease, when manifestations of pulmonary hypertension and right ventricular systolic dysfunction may develop.

Diagnosis of idiopathic pulmonary fibrosis

The diagnosis is based on the analysis of anamnestic data, the results of radiation methods of research, studies of lung function and biopsy. Idiopathic pulmonary fibrosis is commonly misdiagnosed as other diseases that have similar clinical manifestations such as bronchitis, bronchial asthma or heart failure.

Treatment of idiopathic pulmonary fibrosis

None of the specific treatment options has been shown to be effective. Supportive treatment for idiopathic pulmonary fibrosis is reduced to oxygen inhalation for hypoxemia and antibiotics for the development of pneumonia. End-stage disease may require lung transplantation in some patients. Glucocorticoids and cytotoxic agents (cyclophosphamide, azathioprine) have traditionally been given empirically to patients with idiopathic pulmonary fibrosis in an attempt to stop the progression of inflammation, but there is limited evidence to support their effectiveness. However, it is common practice to try prednisolone (orally, at a dose of 0.5 mg/kg to 1.0 mg/kg, once a day for 3 months, followed by a dose reduction to 0.25 mg/kg, once per day for the next 3-6 months) in combination with cyclophosphamide or azathioprine (oral, at a dose of 1 mg/kg to 2 mg/kg, once a day and N-acetylcysteine 600 mg 3 times a day orally as an antioxidant ). With a frequency of every 3 months to 1 time per year, clinical, radiological and physical assessment of the condition and correction of doses of drugs are performed. Treatment of idiopathic pulmonary fibrosis is stopped in the absence of an objective response.

Pirfenidone, which is a collagen synthesis inhibitor, may stabilize lung function and reduce the risk of exacerbations. The effectiveness of other antifibrotic agents, in particular, inhibiting the synthesis of collagen (relaxin), profibrotic growth factors (suramin) and endothelin-1 (angiotensin receptor blocker) has been demonstrated only in vitro.

Interferon-y-lb showed a good effect when administered together with prednisolone in a small study, but a large double-blind multinational randomized trial showed no effect on disease-free survival, lung function or quality of life.

Etiology unknown. Among the possible etiological factors are smoking, certain types of silicate dust. The viral nature of the disease and genetic predisposition are discussed.

There are 5 degrees of pathomorphological changes in the lung tissue in patients with IPF:

I degree: swelling of the interalveolar septa, cell infiltration, capillary tortuosity.

III degree: involvement in the process of bronchioles with the formation of small cysts and the destruction of the structure of the alveoli.

IV degree: the normal structure of the lung tissue is completely disrupted, the cystic cavities gradually increase.

V degree: the formation of the so-called "honeycomb (or cellular) lung." The cysts are up to 1 cm in diameter.

Clinical symptoms: most often ELISA occurs in the age range from 40 to 49 years. Male to female ratio 2:1

There are no pathognomonic, characteristic only for ELISA, signs of the disease. The onset may be imperceptible or associated by patients with an acute respiratory infection, influenza, and is manifested by the occurrence of shortness of breath with moderate physical exertion. Steadily progressive dyspnea- one of the most characteristic and permanent signs of ELISA. Sometimes, as the first sign of the disease, patients note a cough (dry or with scanty mucous sputum), which is then joined by progressive shortness of breath. As the disease progresses, the cough may intensify and be accompanied by pain in the chest of a neurological nature. A typical complaint is the inability to take a deep breath.

One of the signs of the disease, indicating (along with others) the progression of the pathological process in the lungs, is weight loss.

When examining a patient, cyanosis of varying severity (from acrocyanosis to diffuse) attracts attention. The degree of its severity depends on the severity of the disease. In the early stages of the chronic course of the disease, cyanosis may appear only with physical exertion, but as the disease progresses, it intensifies. In acute forms of the disease, cyanosis is one of the early signs.

With percussion of the lungs over the affected area, dullness of the percussion tone is noted (more often these are the lower sections of the lungs).

On auscultation, inspiratory crepitus is heard (usually at the height of inhalation). This sound phenomenon is referred to in the literature as "cellophane crackling". Often this is a bilateral crepitus, it is better heard along the posterior and middle axillary lines, as well as between the shoulder blades. Crepitus is not always a permanent symptom of ELISA. In the acute form of the disease, crepitus can be heard even with a normal x-ray picture, while at the same time it may not be in the chronic course and changes in the x-ray; it can disappear with adequate therapy.

A characteristic auscultatory sign of ELISA is weakened vesicular breathing (shortening of the inspiratory and expiratory phases). Harsh breathing, dry wheezing can occur when endobronchitis is attached. In the presence of pulmonary hypertension, there is an accent of II tone over the pulmonary artery.