Idiopathic pulmonary fibrosis what. Idiopathic pulmonary fibrosis (IPF): a modern approach to classification and diagnosis. Idiopathic pulmonary fibrosis. Treatment

Idiopathic pulmonary fibrosis (IPF) is a very serious disease. It provokes thickening of the walls of the alveoli, and the appearance of damage there. When they are damaged, the lung tissue fibroses (thickens), which is why an irreversible process of decreasing the function of the respiratory organ begins, and it gradually only progresses.

It is easy to guess that the risk of IPF hangs primarily over:

• people working in hazardous industries;
• smokers;
• persons with a family history.

It is worth noting that according to medical statistics, in the age category from 50 to 70 years, IPF is diagnosed in men 2 times more often than in women. According to experts. The main factor causing this problem is smoking. This is true even for those who have given up a bad habit quite a long time ago - the negative consequences for the body eventually make themselves felt.

IPF is often called pneumonia, but many doctors argue that inflammation plays a very minor role in this situation. The basis of the clinical development of this disease is the proliferation of mesenchymal cells and interstitial fibroblasts (aberrant and specific) with fibrosis and collagen deposition. Although initially damage to the alveoli is indeed caused by smoking, genetic, environmental, or some other factors.

In the vast majority of cases, the presence of a particular health problem in our body is indicated by certain signs. Just after examining them, the doctor makes a preliminary diagnosis. In order to confirm or refute it, the patient may be referred for additional examination - tests, x-rays, MRI, and so on. Idiopathic pulmonary fibrosis is not an exception to the general rule here. This disease also has its own specific symptoms, which will immediately guide an experienced doctor along the right path.

However, the main problem here is that the signs of IPF do not develop and appear immediately, but gradually. In most cases, this takes from 6 months to several years.

Among the most typical symptoms of the disease is a nonproductive cough. Another characteristic symptom is shortness of breath, which appears even after minor physical exertion. In later stages it occurs even at rest. However, such general symptoms as myalgia and increased body temperature to the “febrile level” appear very rarely.

On the other hand, there is one sure sign of IPF: wheezing. In this case, they will be bilateral dry fine-bubble inspiratory basal ones. The wheeze of idiopathic pulmonary fibrosis is very similar to the sound of a fastener known as Velcro being undone. In approximately half of the cases, patients experience thickening of the terminal phalanges of the fingers. In fact, no further anomalies will be detected as a result of the external examination. They will not appear until the thermal stage of IPF begins to develop. Right ventricular systolic dysfunction and pulmonary hypertension may already appear here.

In order to make a correct diagnosis, anamnestic data alone is not enough. The doctor will also need the results of a biopsy and tests - both radiation and pulmonary function tests. It should be noted that misdiagnosis is quite common in idiopathic pulmonary fibrosis. Inexperienced doctors often confuse it with a number of other diseases that have similar clinical symptoms.

Diffuse enhancement of the pulmonary pattern in the peripheral and lower zones can be detected using radiography. This method also helps to identify another sign of the disease. We are talking about “honeycomb lung” - small cystic clearings and dilated upper respiratory tracts.

Pulmonary function tests help to show the restrictive nature of the changes occurring and the reduced diffusion capacity for DI_CO (carbon monoxide). High-resolution computed tomography can detect subpleural pulmonary enhancement and traction bronchiectasis. However, laboratory tests play a secondary role in diagnosing IPF.

No effective methods have yet been found to combat idiopathic pulmonary fibrosis.

One important point should be emphasized. Unfortunately, medicine has not yet learned how to effectively combat idiopathic pulmonary fibrosis. In other words, none of the therapy methods used today gave the expected results. Therefore, patients can only rely on supportive treatment. It consists, in particular, in prescribing antibiotics if pneumonia develops. In case of hypoxemia, patients are referred to oxygen inhalation. In case of the terminal stage of the disease, the option of a lung transplant is not excluded.

In order to stop the development of inflammation, patients are prescribed cytotoxic drugs (azathioprine and cyclophosphamide), as well as glucocorticoids, but this method is not considered very effective. The common practice here is a 6-9 month course of oral Prednisolone with a gradual reduction in dosage. In this case, a combination with azathioprine or cyclophosphamide and N-acetylcysteine ​​is used - the latter plays the role of an antioxidant.

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Dr Toby Maher, Research Fellow at the UK National Institute for Health Research, Consultant Physician at the Royal Brompton Hospital in London

Idiopathic pulmonary fibrosis is a progressive disease of unknown origin, which is characterized by gradual scarring, replacement of healthy lung tissue with the inevitable outcome of pulmonary failure.

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 UK National Institute for Health Research and a 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 studying the pathogenesis of idiopathic pulmonary fibrosis (IPF).

Dr. Maher previously served as Editor-in-Chief of Respirology and was 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 severe, fatal disease that affects 3 million people worldwide.

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

With IPF, 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 shortness of breath occurs only during exertion, then over time the life of patients with IPF becomes a daily struggle. Even the simplest tasks, such as taking a shower or getting dressed, require superhuman effort from them.

The rate of progression of IPF varies. On average, every year 1 in 20 patients experiences a catastrophic worsening of the disease. Episodes of exacerbation require hospitalization and intensive treatment: in 50% of cases of exacerbation of IPF, patients are killed within 30 days.

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

- Does early diagnosis of IPF help 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 between the symptoms of IPF and other, more common lung diseases (asthma, COPD) makes diagnosis very difficult. In half of IPF cases, 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 about 1-2 years.

Two wasted years!

All this time, patients are unsuccessfully struggling with a non-existent disease until they turn to a specialized center that has experience in diagnosing interstitial lung diseases.

Rapid access to such centers and specialists is critical for accurate diagnosis and early initiation of correct medical treatment of IPF.

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 affect life decisions, so professional help for such patients is necessary.

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

- Although pulmonary fibrosis has no cure, various options are available to slow IPF, relieve symptoms, and improve quality of life.

These include antifibrotics, oxygen, antitussives and bronchodilators, rehabilitation measures and palliative care at the end of life.

Until recently, no new drugs were available to treat IPF. The situation changed with the advent of the antifibrotic drugs pirfenidone and nintedanib in the US and EU. These medications may 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 physical 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 IPF, what they can and cannot do, and support them in difficult times.

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

As I said, 1 in 20 IPF patients experience severe worsening of symptoms each year, leading to a hospital bed. Currently, there are no reliable therapeutic options that reliably 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 made great progress in understanding the pathogenesis, clinical picture and promising targets for the treatment of IPF.

I hope the future brings good news to millions of patients and their families.

Most importantly, there is a growing understanding 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 IPF are studying. In many countries, a coherent system of care for such patients is being formed.

The patients themselves are aware of the positive changes and the importance of scientific research.

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

Today, clinical trials of new drugs are being conducted everywhere, extending a hand of hope to seriously ill patients. We have a range of ongoing and planned trials: new drugs, combinations of existing drugs, diagnostic and therapeutic biomarkers.

: Master of Pharmacy and professional medical translator

Idiopathic pulmonary fibrosis (IPF) is one of the most common diseases from the IIP group. The picture of IPF was described by Scadding in 1960, and he was the first to coin the term “fibrosing alveolitis.” It is possible that the earliest description of IPF was by Rindfleisch, who in 1897 described “cystic cirrhosis of the lungs,” a lung disease characterized by thickening and shrinking of the lung parenchyma and the formation of a “honeycomb lung.”

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

In our country, synonyms for IPF are “idiopathic fibrosing alveolitis” (IFA) and “cryptogenic fibrosing alveolitis”, which has become more widespread in the UK. The concepts “idiopathic” and “cryptogenic”, despite a slight semantic difference, are currently considered synonyms, denoting the hidden, unclear nature of the disease.

ELISA (synonyms: Hamman-Rich disease or syndrome, Skedding syndrome, diffuse progressive interstitial fibrosis of the lungs, fibrous dysplasia of the lungs, etc.) is a unique 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 the structural and functional units of the parenchyma, which leads to the development of restrictive changes in the lungs and impaired gas exchange.

Etiology unknown. Possible etiological factors include smoking and 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 decrease in the diffusion capacity of the lungs and, accordingly, the severity of arterial hypoxemia, respiratory failure and their clinical manifestations largely depend on the degree of its severity.

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 caused by a decrease in the contact surface of the alveolar air with the blood of the alveolar capillaries and a reduction in contact time. The listed mechanisms, as well as reflex constriction of pulmonary vessels due to endocapillary hypoxia, contribute to an increase in pressure in the pulmonary artery (Euler-Lillestrand reflex) and the development of cor pulmonale. The share of venoarterial shunt is relatively small - about 6%.

It is assumed that in the interstitial tissue of the lungs, collagen breakdown is reduced and its synthesis by fibroblasts and alveolar macrophages is increased. 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 inhibits collagen synthesis under normal conditions. Many authors classify Hamman-Rich syndrome as an autoimmune disease in which the functional activity of T-suppressors is inhibited, which leads to overproduction of various classes of immunoglobulins by B-lymphocytes. Antigen-antibody complexes (AIC) formed in the blood are deposited in the walls of the small vessels of the lungs. The main reason for the long-term persistence of CEC is a defect in the functional activity of the Fc fragments of IgG. Under the influence of the CEC, lysosomal fragments of alveolar macrophages and neutrophils, damage to the lung tissue occurs, compaction, 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 epithelial cell injury and mesenchyamal cells leads to dysregulation of repair mechanisms with excess production of profibrotic cytokines, extracellular matrix, and impaired angiogenesis.

Pathological anatomy. Histological changes in 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, cellular infiltration, capillary tortuosity.

II degree: exudation of serous fibrous fluid (rich in protein and stained with eosin) and cellular exudation into the alveoli, which leads to obliteration of the alveolar space (intra-alveolar 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 of bronchioles in the process with the formation of small cysts and 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 “cellular (or cellular) lung.” Cysts reach 1 cm in diameter.

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

There are no pathognomonic signs of the disease characteristic only of ELISA. The onset may be imperceptible or is associated by patients with an acute respiratory infection, influenza, and is manifested by the occurrence of shortness of breath during moderate physical exertion. Steadily progressive shortness of breath- one of the most characteristic and constant signs of ELISA. Sometimes, as the first sign of the disease, patients note a cough (dry or with scanty mucous sputum), which is then accompanied by progressive shortness of breath. As the disease progresses, the cough may intensify and be accompanied by neurological chest pain. 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 to 38-39°C, only then shortness of breath and cough occur. About 5% of patients report 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 joint stiffness), muscle pain, intermittent increases in body temperature to subfebrile or febrile levels, and Raynaud's syndrome are observed in half of patients with IFA. Such a high incidence of joint damage is an additional argument for the participation of autoimmune disorders in the pathogenesis of this pathology. All patients experience weakness and fatigue.

When examining the patient, attention is drawn to cyanosis of varying degrees of severity (from acrocyanosis to diffuse). 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 during physical activity, but as the disease progresses, it intensifies. In acute forms of the disease, cyanosis is one of the early signs.

Patients notice changes in the nail phalanges associated with chronic hypoxia (symptom of “drumsticks” and “watch glasses”). The rate of formation of these symptoms depends on the activity, duration of the pathological process and the severity of respiratory failure.

When percussing the lungs over the affected area, a dullness of the percussion tone is noted (usually in the lower parts of the lungs).

During auscultation, crepitus is heard during inspiration (usually at the height of inspiration). This sound phenomenon is called “cellophane crackling” in the literature. Often this is a bilateral crepitus, best heard along the posterior and middle axillary lines, as well as between the shoulder blades. Crepitation is not always a constant 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 present in a chronic course and changes in the x-ray; it may disappear with adequate therapy.

A characteristic auscultatory sign of IFA is weakened vesicular breathing (shortened inhalation and exhalation phases). Hard breathing and dry wheezing may occur when endobronchitis is associated. In the presence of pulmonary hypertension, an accentuation of tone II over the pulmonary artery is observed.

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.

Fatigue and decreased oxygen levels in the blood. Sometimes pulmonary fibrosis is caused by substances from the external environment that can be identified. But in many cases the cause of the disease remains unclear. If the cause of pulmonary fibrosis is unknown, the condition is called idiopathic pulmonary fibrosis (IPF); the disease used to be called idiopathic fibrosing alveolitis (IFA), but this term is no longer used.

Figures and facts

• There have been no large-scale studies on the incidence and incidence of IPF.
• According to various sources, from 2 to 29 people per 100 thousand of the population suffer from IPF.
• It is unknown whether geographic, ethnic, cultural, or racial factors influence the incidence and incidence of IPF.
• Most patients with IPF develop symptoms such as cough and shortness of breath between the ages of 50 and 70. IPF is uncommon in people under 50 years of age.
• It has long been thought that IPF occurs more often in men than women, but in recent years there has been an increase in the incidence of IPF in women.
• In some cases, IPF develops in more than one person from the same family. When this happens, the disease is called familial pulmonary fibrosis. The fact that pulmonary fibrosis is sometimes inherited has led many experts to believe that having certain genes may lead to the development of the disease.

When to see a doctor

• For a dry cough or difficulty breathing that does not improve over time.
• If your condition suddenly worsens and your symptoms worsen, you should seek help immediately.

Diagnosis of the disease

A doctor may suspect IPF based on symptoms such as cough and difficulty breathing. Pathological sounds in the lungs, called crepitus, can be heard by the doctor at the moment of deep inspiration. The patient and the attending physician may notice thickening of the fingers at the very tips and a characteristic change in their shape, the so-called drumsticks. The presence of these signs gives grounds to refer the patient to a specialist in lung diseases.

The pulmonologist will perform a complete examination and may order several tests, such as a chest x-ray, a respiratory function test (spirometry), or a blood oxygen level measurement. In addition, a high-resolution computed tomography (HRCT) scan of the chest, an echocardiogram (ultrasound of the heart), and sometimes a lung biopsy may be needed.

A lung biopsy is usually performed using video-assisted thoracoscopic surgery (VATS - video assisted thoracoscopic surgery) under general anesthesia. During this procedure, the surgeon makes two or three small holes in the chest wall through which he inserts a video camera on a flexible base. The device allows you to look inside the chest cavity and take a piece of lung tissue for examination.

Treatment of the disease

Once diagnosed with IPF, patients should see a pulmonologist regularly. Treatment of IPF is mainly symptomatic, aimed at relieving cough and shortness of breath. Two new specific drugs for the treatment of IPF that slow the progression of fibrosis have been approved for use in the United States. These drugs are also available in Russia, although, unfortunately, the cost of the drugs is very high.

Before the advent of specific drugs, glucocorticosteroid hormones (corticosteroids) and immunosuppressants were used to treat IPF, but they were not very effective and caused many unwanted side effects. Pulmonary rehabilitation, oxygen therapy, and treatment of pulmonary hypertension are also used to relieve the symptoms of IPF and related conditions.

Many specialists should be involved in working with a patient with IPF: pulmonologists, physical therapists, palliative care specialists, physical therapists. Many of them are just beginning to appear in our country. Talk to your doctor about possible medications and therapies that may help in your particular case.

Lung transplantation for IPF

Today, lung transplantation is the only way to increase life expectancy in patients with IPF. A transplant is a major surgery that requires lifelong treatment with drugs that prevent the immune system from rejecting the donor lung. Not all patients with IPF are eligible for lung transplantation. The treating pulmonologist can evaluate the condition to determine whether transplantation is possible in a particular case. This evaluation can take months, so the doctor may consider a lung transplant before the condition begins to worsen.

The leading institutions performing lung transplantation in Russia are the Federal Scientific Center for Transplantology named after. Academician V.I. Shumakov and Research Institute of SP named after. N.V. Sklifosovsky.

Pulmonary rehabilitation

Involvement in a pulmonary rehabilitation program and participation in support groups is necessary to learn more about the disease and treatment options. Pulmonary rehabilitation programs can invigorate and improve overall tone, reduce shortness of breath, provide better understanding of IPF and oxygen use, and teach self-care skills.

Blood oxygen saturation should always be maintained above 89%, regardless of what the person is doing: sitting, walking, exercising or sleeping. But as the disease progresses, the need for additional oxygen may change. Therefore, it is important to regularly assess oxygen levels to understand how much oxygen is sufficient at this stage at rest, during physical activity or during sleep.

It is very important for smokers to quit this habit. Tobacco smoke worsens breathing problems.

Precautionary measures

If you have chronic lung disease, it is very important to avoid situations in which you can become infected with ARVI and influenza. It is necessary to get vaccinated against influenza annually. A small percentage of patients with IPF develop a sudden exacerbation of the condition, and the shortness of breath due to IPF worsens rapidly. No one knows why sudden exacerbations occur and in which patients they are more likely to occur. If you notice a sudden worsening of your shortness of breath, contact your healthcare provider or seek emergency medical help.

Participation in clinical trials on IPF

If you are interested in participating in research, ask your pulmonologist. As new treatments become available, clinical studies are conducted to understand how each treatment works. These studies can only be conducted in volunteers with IPF. It may be worth checking to see if there is research into IPF at any of the research centers near where you live. Even if you don't intend to be a research participant, getting help from a center that specializes in IPF can be helpful.

In 2017, the first Regional Center for Diagnostics of Patients with IPF opened in Yekaterinburg.

How to prepare for your visit

Make a list of your symptoms and questions you would like to discuss with your doctor in advance. It's also important to remember (and write down) when you first noticed symptoms and how they changed over time. It’s good if your relatives come to the appointment to help you ask additional questions or remember important information.

In which, due to fibrous changes (scars) and compaction of the lung tissue, the normal functioning of the lungs is disrupted. As the disease progresses, the lung tissue becomes increasingly scarred, which leads to a decrease in the amount of oxygen entering the blood.

The five-year survival rate of patients with IPF does not exceed 30%, and only the recent advent of antifibrotic therapy has made it possible to slow down the progression of the disease and prolong the life of patients. Unfortunately, such therapy is not available to every Russian patient: this is due to the low level of awareness about the disease - often not only patients, but also medical specialists do not know about it. Currently, only a few hundred cases of the disease have been officially registered in Russia, but according to experts, there are more than 10 thousand such patients in the country.

Difficulties in diagnosing IPF are due to the fact that the symptoms of the disease also occur in other diseases - patients are misdiagnosed and prescribed therapy that does not alleviate their condition. About 60% of patients with IPF do not receive treatment on time.

“Diagnosis of IPF is objectively difficult,” says Alexander Averyanov, director of the Federal State Budgetary Institution Research Institute of Pulmonology of the Federal Medical and Biological Agency of Russia, Doctor of Medical Sciences, Professor. – On the one hand, its symptoms: dry cough, shortness of breath during exercise and lung sounds on auscultation, reminiscent of the crunching of cellophane, are characteristic of many other respiratory and cardiovascular diseases. However, due to the rarity and little-studied nature of the disease, most therapists and even pulmonologists do not have sufficient experience in diagnosing and treating this disease. As a result, in more than 50% of cases, patients with idiopathic pulmonary fibrosis are initially given a completely different diagnosis: COPD, heart failure - and are prescribed therapy that does not and cannot help, and in some cases even harms. From the moment you consult a doctor until the correct diagnosis is made, on average, more than a year passes, and during this time the disease progresses, fibrotic processes in the lungs increase, making breathing more and more difficult, leading to disability and early death.”

In some cases, a correct diagnosis cannot be made throughout the patient's life, leading to the disease being considered a rarer disease than it actually is. As a result, the funding available for the treatment of patients with IPF is insufficient to provide effective therapy for all patients. Traditional regimens using glucocorticosteroids and cytostatics do not give the expected results, and innovative drugs that can actually prolong life are not paid for by the state.

To raise awareness about idiopathic pulmonary fibrosis and provide care to patients, International IPF Week is being held from September 16 to 23 around the world, including Russia. Representatives of the medical community and patient organizations are interested in ensuring that as many people as possible know about the symptoms of the disease, its dangers and possible treatment. Timely administration of therapy soon after diagnosis will help prevent rapid progression of the disease and prolong the period of active life.

Men suffer from idiopathic pulmonary fibrosis much more often than women, and the mortality rate from this disease exceeds the mortality rate from many types of cancer. Men over 60 years of age, smokers (as well as those who have quit), if shortness of breath and cough occur, should contact a specialized pulmonology center for an examination to rule out IPF.