Pulmonary fibrosis

2021-07-23 04:32 PM

The most common symptoms of pulmonary fibrosis are shortness of breath, especially during or after physical activity, and a dry cough.


Pulmonary fibrosis is a serious disease that causes progressive scarring of lung tissue.

According to current thinking, pulmonary fibrosis begins with repeated trauma to the tissues in and between the small air sacs (alveoli) in the lungs. The damage eventually leads to scarring (fibrosis) and makes breathing difficult. The most common symptoms are shortness of breath and a dry cough.

Current treatments for pulmonary fibrosis include medications and treatments to improve lung function and quality of life. Several new treatments for pulmonary fibrosis are now in clinical trials. Meanwhile, a lung transplant may be an option for some people with pulmonary fibrosis.


Signs and symptoms of pulmonary fibrosis include:

Shortness of breath.

Dry cough.


Unexplained weight loss.

Muscle and joint pain.

The most common symptoms of pulmonary fibrosis are shortness of breath, especially during or after physical activity, and a dry cough. Usually does not appear until the disease is severe, and irreversible lung damage has already occurred. Even then, it is possible to alleviate symptoms.

Breathing problems often get worse gradually, and can eventually be seen in regular activities - dressing, talking, even eating. At this point, the symptoms cannot be ignored.

The development of pulmonary fibrosis and the severity of symptoms can vary considerably from person to person. Some people get sick very quickly with severe symptoms. Others have moderate symptoms and get worse over a period of several months or a year.


The process of respiration

With each inhalation, air moves to the lungs through large airways called bronchi. Inside the lungs, the bronchi divide like twigs into a million small airways (bronchioles) that eventually end in clusters of air sacs (alveoli). There are about 300 million alveoli in each lung. The walls of the alveoli are small blood vessels (capillaries) where oxygen is added to the blood, and carbon dioxide, a waste product of metabolism, is removed.

What happens in pulmonary fibrosis?

In pulmonary fibrosis, the alveoli are damaged by irreversible scarring. Normally, the alveolar sacs are highly elastic and expand and deflate like bubbles with each breath. But scarring makes the interstitial tissue stiff and thickens making the air less flexible. Instead of being soft and elastic, the alveolar sacs have a dry sponge-like structure, making breathing much more difficult.

Cell accumulation in scar tissue is not normal, the body only makes enough scar tissue to repair the damage. But in pulmonary fibrosis, the repair process goes wrong.
For people with pulmonary fibrosis, healing may be hindered in another way. Normally, blood vessels help carry oxygen and nutrients to injured cells. For example, if a finger is cut, the body sends nutrients to the finger to help promote healing. But in people with pulmonary fibrosis, some new blood vessels grow and become blocked.

What causes damage to the lungs?

Hundreds of factors can cause lung damage that eventually leads to pulmonary fibrosis. Some of the most popular include:

Working environment

Long-term exposure to certain toxins and pollutants can damage the lungs. Among them are silica dust and asbestos fibers. Chronic exposure to certain organic matter, including grain dust, sugar cane, bird and animal droppings, can also cause fibrosis.


A small percentage of people who receive radiation therapy for their lungs or breast cancer show signs of lung damage months or sometimes years after initial treatment. The severity of the damage depends on the lung exposure to radiation, the total amount of radiation, chemotherapy used, and the presence of pre-existing lung disease.


Many drugs can harm the lungs, especially chemotherapy drugs (methotrexate, cyclophosphamide); medicines used to treat irregular heartbeats and other heart problems (amiodarone, propranolol); some psychiatric drugs, and some antibiotics (nitrofurantoin, sulfasalazine).

Gastroesophageal reflux disease (GERD)

Acid reflux occurs, stomach acid into the esophageal tube appears to play an important role in pulmonary fibrosis. Although people with pulmonary fibrosis frequently have GERD, they may not have the typical symptoms of GERD, such as heartburn and belching.

Health and other issues

Serious lung infections such as tuberculosis and pneumonia can cause permanent lung damage. Thus, the disorder can affect tissues in the body, not just the lungs, such as systemic lupus erythematosus, rheumatoid arthritis, dermatomyositis, polymyositis, Sjogren's syndrome, and sarcoidosis. In rare cases, scleroderma is associated with a particularly severe form of pulmonary fibrosis.

Idiopathic Pulmonary Fibrosis: Unknown causes

The list of substances and conditions that can lead to pulmonary fibrosis is long. In many cases, though, the cause is never found. Pulmonary fibrosis of unknown cause is called idiopathic pulmonary fibrosis.

Researchers have several theories about what may cause idiopathic pulmonary fibrosis, including viruses and exposure to cigarette smoke. And because the type of idiopathic pulmonary fibrosis runs in families, genetics are also thought to play a role, even in people who don't directly inherit the disease.

Risk factor

Factors that predispose you to pulmonary fibrosis include:

Age. Although pulmonary fibrosis has been diagnosed in children and infants, the disorder is most likely to affect middle age and seniors.

Sex. In general, men are more likely to have pulmonary fibrosis than women.

Labor and environmental toxins. There is an increased risk of developing pulmonary fibrosis if farming, mining, construction or exposure to pollutants known to damage the lungs.

Radiation and chemotherapy. Radiation treatments to the chest or the use of certain chemotherapy drugs make lung fibrosis more likely.

Risk factors for idiopathic pulmonary fibrosis

Although the causes of idiopathic pulmonary fibrosis are not known, researchers have identified several factors that appear to increase the risk:

Smoke. Heavy smokers, and former smokers, develop idiopathic pulmonary fibrosis more than those who have never smoked.

Genetic factors. A rare type of idiopathic pulmonary fibrosis runs in families. The researchers have yet to identify the specific genes involved, but they have detected genetic changes in proteins in the airways and alveoli of people with idiopathic pulmonary fibrosis.

Viruses. Many people report developing symptoms of pulmonary fibrosis following a viral illness, especially those caused by viruses such as herpes, Epstein-Barr, mononucleosis virus. Researchers are investigating viruses that may play a role in lung disease.


Complications of pulmonary fibrosis can include:

Low blood oxygen level

Because pulmonary fibrosis reduces the amount of oxygen that enters the blood, there is a chance that lower than normal blood oxygen levels develop. Lack of oxygen can disrupt bodily functioning, and in severe cases can be life-threatening.

Pulmonary arterial hypertension

Unlike systemic hypertension, this condition affects only the arteries in the lungs. It begins when small arteries and capillaries are compressed by scar tissue, causing increased vascular resistance in the lungs. This will increase the pressure in the pulmonary artery. Pulmonary hypertension is a serious disease that gets worse gradually and can eventually be fatal.

Right ventricle failure

This serious condition occurs when the right ventricle has to pump harder than usual to move blood through a blocked pulmonary artery.

Respiratory failure

This is often the end-stage of chronic lung disease. It occurs when the oxygen level in the blood drops dangerously low. Low blood oxygen can lead to arrhythmias and unconsciousness.

Tests and diagnostics

Diagnosing pulmonary fibrosis can be extremely difficult. The distinction between idiopathic and non-idiopathic forms of the disease is not always clear-cut, and the naming and classification systems for both have historically been confusing and controversial. In addition, many medical conditions, including chronic obstructive pulmonary disease (COPD), asthma, and even heart failure, can mimic pulmonary fibrosis, so doctors must consider these before giving them treatment. make a definite diagnosis.

A medical history, physical exam, and even an X-ray are not enough to diagnose pulmonary fibrosis but can help rule out other conditions. For that reason, could have tested like:


This will often show the typical scar tissue of pulmonary fibrosis and is useful for the disease process and treatment. Occasionally, chest radiographs are normal and further testing is required to explain the unknown cause of dyspnea.

High resolution computed tomography (HRCT) scan

This provides much sharper detailed images of the lungs than conventional CT or routine chest X-rays.

Lung function test

The tests determine how well the lungs are working. Assess how much air the lungs can hold, and how quickly air can move in and out of the lungs.

Method of measuring oxygen

This simple test uses a small device placed on the finger to measure the oxygen saturation in the blood. Oximetry is an easy way to monitor the course of a disease, which can sometimes be more accurate than an X-ray.

Stress test

Test performed on a treadmill or bicycle to monitor lung function while active.

Usually, though, pulmonary fibrosis can be definitively diagnosed by examining a small amount of lung tissue (biopsy) in a laboratory. Tissue samples can be obtained in one of the following ways:

Bronchoscopy (biopsy)

In this part, the doctor removes small samples of tissue - using a small tube (bronchoscope) that is passed through the nose or mouth into the lungs. The risks of bronchoscopy are often minor - often with temporary sore throat and hoarseness - tissue samples are sometimes too small for an accurate diagnosis.

Bronchoalveolar lavage

In this procedure, the doctor injects saline through the bronchoscope, and then immediately aspirates it. The recovered substance contains cells from the alveoli. Although the bronchoalveolar lavage sample is more informative than other procedures, it may not provide enough information to diagnose pulmonary fibrosis.

Biopsy surgery

In some cases, your doctor may recommend a surgical biopsy. Although this is a more invasive procedure with potential complications, it is often the only way to obtain a large enough tissue sample to make an accurate diagnosis. During the procedure, surgical instruments and a small camera are inserted through two or three small incisions between the ribs. The camera allows the surgeon to view the lung on a video monitor while removing tissue samples from the lung. Due to the surgical video support, there is no need to cut ribs, less pain, and faster healing compared to traditional open lung surgery.

Treatments and drugs

The lung scarring that occurs in pulmonary fibrosis is irreversible, and no current treatment regimen has proven effective in preventing the eventual development of the disease. Some treatments, though, can temporarily improve symptoms or slow disease progression. Others help improve quality of life.


Many people with a diagnosis of pulmonary fibrosis are initially treated with corticosteroids (prednisone), sometimes in combination with other immunosuppressants, such as methotrexate or cyclosporin. These combinations have not proven effective. Adding N-acetylcysteine, a derivative of a naturally occurring amino acid, to prednisone may reduce disease in some people. And in clinical trials, the drug pirfenidone has been shown to improve lung function and prevent damage to lung tissue.

Prednisone and other immunosuppressants can also cause serious side effects, including diabetes, glaucoma, decreased red blood cell production, skin cancer, and lymphoma. For that reason, treatment is usually discontinued if there is no improvement after six months. Although some people improve temporarily with immunosuppressive drugs, it is not clear why some people respond and others do not.

Lung transplant

A lung transplant may be the last resort option for young people with severe pulmonary fibrosis who have not benefited from other treatment options. To be considered for a transplant, you must agree to quit if you smoke, be healthy enough to undergo surgery and post-transplant treatment, and be willing and able to follow the medical plans outlined by the rehabilitation team function and transplant, patience and mental strength, and support to go through the wait for a donor agency. The last request is particularly important because the organs are provided by the donor.

Treatments for pulmonary fibrosis focus on improving quality of life. These include:

Oxygen therapy. Using oxygen cannot stop lung damage, but it can make breathing and exercise easier, prevent or reduce complications from low oxygen in the blood, and improve sleep and feeling good. . It can also lower blood pressure on the right side of the heart. They are more likely to get oxygen while sleeping or exercising, although some people may use it around the clock.

Pulmonary rehabilitation. This is an official program for people with chronic lung disease. The purpose of pulmonary rehabilitation is not only to treat a disease or even improve daily functioning but also to help people with pulmonary fibrosis live full, satisfying lives. Finally, the pulmonary rehabilitation program focuses on exercise, teaching how to breathe more efficiently, education, emotional support, and nutritional counseling.

Often, a multifaceted approach requires a team of healthcare professionals, which may include doctors, nurses, rehabilitation specialists, dietitians, and social workers. Programs can be very different, however. Your doctor can often tell you about pulmonary rehabilitation programs in your area. Or contact the Lung Association for more information.

Treatment method. Several treatments for pulmonary fibrosis are under development or in clinical trials. A list of clinical trials can be found.

Lifestyle and Remedies

Actively participating in treatment and resting, as healthy as possible, are essential to living with pulmonary fibrosis. For that reason, it is important to:

Stop smoking

If you have lung disease, the best thing you can do for yourself is to stop smoking. Talk to your doctor about options for stopping, using a variety of proven techniques to help quit. And because secondhand smoke can harm the lungs, do not allow others to smoke around.

Exercise regularly

Exercise is a double-edged sword for people with lung disease. It can make breathing difficult temporarily, but it helps maintain lung function, reduce stress, and improve overall health and well-being. If done, don't stop. And if currently inactive, talk to your doctor about an exercise program that may be best.

Good diet

People with lung disease may lose weight because it is uncomfortable to eat and because of the extra energy it takes to breathe. However, a nutrient-dense diet contains the necessary amount of calories. A dietitian can provide additional guidance for healthy eating.

Rest much

At least eight hours of good-quality rest each night can boost your immune system and feelings of well-being.

Controlling GERD

If GERD has been diagnosed, it is essential to keep it under control. May help reduce acid reflux, wait at least 3-4 hours after eating before lying down, eat small meals, avoid any foods that cause symptoms and maintain a healthy weight.