Pulmonary fibrosis in adults

Pneumofibrosis is a specific change in the lung tissue that interferes with breathing. The word “pneumo” means lung, and the word “fibrosis” means scar tissue. In fact, these are changes in the lungs, similar to scars that can be on the skin from an old injury or operation. These scars can’t oxygenate the blood, and ventilation suffers.

In its simplest sense, pulmonary fibrosis (or pneumophyrosis) means scarring in the lungs. Over time, the scar tissue can grow so large that it can destroy a normal lung and make it difficult for oxygen to get into the blood. Low oxygen levels (and the tough scar tissue itself) can cause shortness of breath, especially when walking and exercising.

Causes of pulmonary fibrosis in adults

Pulmonary fibrosis is not just a disease. This is a whole group of more than 200 different lung diseases that are very similar to each other. The family of lung diseases known as pulmonary fibrosis falls into an even larger group of diseases called interstitial lung diseases, which includes all diseases that cause inflammation or scarring of the lungs. Some interstitial lung diseases do not involve scar tissue. When interstitial lung disease involves scar tissue in the lung, doctors call it pulmonary fibrosis.

Nobody knows exactly how many people have pulmonary fibrosis (PF). One recent study found that idiopathic pulmonary fibrosis affects 1 in 200 adults over the age of 60. Approximately 50 new cases are diagnosed each year, and approximately 000 patients die of IPF each year.

It can be difficult for doctors to figure out what causes pulmonary fibrosis. Sometimes they can identify one or more causes of a disease. PF of unknown cause is called “idiopathic”.

There are five main categories of identifiable causes of pulmonary fibrosis:

• drug-induced, such as previous or current use of amiodarone, nitrofurantoin, chemotherapy, methotrexate, or other drugs known to affect the lungs;
• radiation-induced, the cause of which is radiation therapy, previous or current radiation therapy of the chest;
• environmental (called hypersensitivity pneumonitis) – this is exposure to mold, animals or other triggers (heard “squeaks” in the lungs);
• autoimmune (called connective tissue disease) – typical inflammation of the joints, skin changes (especially on the fingers and face), dry eyes or mouth, abnormal blood tests;
• occupational (so-called pneumoconiosis), the causes of which are previous or current exposure to dust, fibers, smoke or vapors that can cause PT (for example, asbestos, coal, silica, etc.).

In our country, environmental and autoimmune causes seem to be the most common types of pulmonary fibrosis with a known cause. In addition, idiopathic pulmonary fibrosis occurs when the cause cannot be determined.

Drugs that cause pulmonary fibrosis. Some medications can cause PF. Drugs used to treat cancer (chemotherapy), drugs used to treat heart rhythm disorders (eg amiodarone) used to treat inflammatory conditions (eg methotrexate), an antibiotic used to treat urinary tract infections (nitrofurantoin) are among the the most well-known agents that can cause injury, inflammation and scarring in the lungs. In some cases, many other drugs can be the cause of pulmonary fibrosis.

Pulmonary fibrosis caused by radiation. Radiation to the chest in lymphoma, Hodgkin’s disease, breast, lung, and other cancers can damage the lungs and cause fibrosis.

environmental reasons. The condition is commonly referred to as hypersensitivity pneumonitis (HP) or chronic hypersensitivity pneumonitis. It occurs when the lungs react with inflammation and scarring after inhaling mold spores, bacteria, animal proteins (especially poultry or caged birds), or other known triggers. No one knows why some people are so prone to developing pulmonary fibrosis, while others are not.

Pulmonary fibrosis and autoimmune diseases. Autoimmune diseases are also called connective tissue diseases, collagen vascular diseases, or rheumatological diseases. “Auto” means self, and “immunity” refers to the immune system. So, if you have an autoimmune disease that affects your lungs, it means that your body’s immune system is attacking your lungs. Examples of autoimmune diseases that can cause pulmonary fibrosis include:

• rheumatoid arthritis;
• scleroderma (also called systemic sclerosis);
• Sjogren’s syndrome;
• polymyositis, dermatomyositis and antisynthetase syndrome.

professional reasons. Occupational causes, also called pneumoconiosis, can develop after significant exposure to a wide range of inorganic dusts, including asbestos, silica, coal dust, beryllium, and hard metal dust.

Hereditary pulmonary fibrosis. The risk of developing most human diseases is influenced by genes inherited from parents. Several genes have been identified that seem to increase the risk of developing pulmonary fibrosis when the abnormal form of the gene is inherited. There is no official medical recommendation as to whether genetic testing should be done, but a doctor may want to test you for one or more abnormal forms of these genes.

Symptoms of pulmonary fibrosis in adults

The most common symptoms of pulmonary fibrosis are a dry, hacking cough and shortness of breath. Symptoms may be mild or even absent in the early stages of the disease. As more scar tissue forms in the lungs, the symptoms worsen.

Shortness of breath initially occurs with exercise, but as the disease progresses, patients may become short of breath when they take part in daily activities such as showering, dressing, talking on the phone, or even eating.

Due to a lack of oxygen in the blood, some people with idiopathic pulmonary fibrosis may also experience fingertip changes. A tubercle or drumstick is a thickening of the skin under the nails, causing the nails to bulge downward. It is not specific for fibrosis and occurs in other diseases of the lung, heart, and liver, and may also be present at birth. Other common symptoms of pulmonary fibrosis include:

• fatigue and weakness;
• chest discomfort;
• loss of appetite;
• unexplained weight loss.

Treatment of pulmonary fibrosis in adults

Pulmonary fibrosis is a serious disease, but there is ongoing research that allows people to live longer and have a better quality of life.

Diagnostics

Scar tissue and inflammation make the lungs stiff. Rigid lungs are difficult to stretch, so the breathing muscles have to work very hard to draw in air with each breath. Your brain senses this extra work and lets you know about the problem by causing you to feel short of breath (or “breathing difficulties”) when you exercise. Also, stiff lungs hold less air (they shrink a little). Doctors use this “squeezing” to diagnose and track the disease with breathing tests (called lung function tests), which measure how much air your lungs can hold. The more scar tissue in the lungs, the less air they will hold.

Scar tissue blocks the movement of oxygen from the air sacs into the bloodstream. For many people with pulmonary fibrosis, oxygen levels drop only slightly during rest, but their oxygen levels drop significantly during exercise. The brain can sense this low oxygen level causing shortness of breath. Doctors will check your oxygen levels to see if they drop after walking, which could indicate pulmonary fibrosis.

Doctors also often prescribe oxygen for use through a nasal cannula or face mask during exercise and sleep in patients with pulmonary fibrosis. As pulmonary fibrosis progresses, oxygen may be needed 24 hours a day.

You may have been told by your doctor that you hear “crackling” sounds in your lungs. Wheezes (also called “crepitations”) sound like Velcro being torn. They are heard in many lung conditions because any type of problem affecting the air sacs (such as PF, pneumonia, or fluid in the lungs due to heart failure) can cause wheezing. Some people with pulmonary fibrosis do not have wheezing, but most do.

Once your doctor recognizes that you may have pulmonary fibrosis, the next step is to try to diagnose the specific type you have – there are over 200 different types. Doctors usually start by asking a lot of questions, doing a thorough physical examination, and ordering a lot of blood tests.

The doctor will also use a special type of chest x-ray called a high-resolution computed tomography (HRCT) scan to see what your lung tissue looks like. The scan gives a close-up of the lungs and provides more detailed information than conventional CT scans. Healthy lung tissue appears almost black on CT. Scar tissue and inflammation appear gray or white.

Sometimes, even after all the examinations are completed, the doctor will still not get an answer, and he will have to take a lung biopsy to determine which of the 200 different types of pulmonary fibrosis the patient has. A lung biopsy can also help a doctor decide which treatment might be helpful.

Modern treatments

Once scar tissue has formed, it cannot be repaired or removed, so treatment is aimed at slowing the progression of the disease and improving symptoms. Unfortunately, the treatment we have is limited. For many people with pulmonary fibrosis, symptomatic relief, such as oxygen therapy, is the only treatment available. Candidates for lung transplantation may be relatively young (under 65) and healthy people with pulmonary fibrosis.

For idiopathic pulmonary fibrosis, there are 2 drugs that slow the progression of the disease. Treatment with nintedanib or pirfenidone has been shown to reduce the rate of scarring or fibrosis in the lungs. For pulmonary fibrosis caused by another disease, such as rheumatoid arthritis, treating the underlying disease is the best approach and usually reduces inflammation in the lungs.

Pulmonary rehabilitation is a standard part of the treatment for people with pulmonary fibrosis. This could be an exercise program, breathing exercises, and stress management. Exercise can improve the body’s ability to provide oxygen to tissues and maximize the efficiency of healthy lung tissue. People with severe pulmonary fibrosis may need oxygen equipment to keep enough oxygen in their circulation. Support groups can help reduce the stress caused by illness. People with pulmonary fibrosis should not smoke.

Prevention of pulmonary fibrosis in adults at home

It is difficult to prevent the disease if it is not known what is causing it, so there are no generally accepted recommendations for the prevention of pulmonary fibrosis. However, people in jobs that increase the risk of developing pulmonary fibrosis (eg, hay farmers, miners, welders, sandblasters, demolition workers) should do everything possible to minimize exposure to harmful substances (eg, wear masks).

Popular questions and answers

We asked questions about pneumofibrosis Pathologist, Junior Researcher, National Research Center for Pediatric Hematology, Oncology and Immunology named after N.N. Dmitry Rogachev to Dmitry Abramov.