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Pulmonary fibrosis

Medically reviewed by Drugs.com. Last updated on Feb 15, 2024.

Overview

Pulmonary fibrosis is a lung disease that occurs when lung tissue becomes damaged and scarred. This thickened, stiff tissue makes it harder for the lungs to work properly. Pulmonary fibrosis worsens over time. Some people can stay stable for a long time, but the condition gets worse faster in others. As it gets worse, people become more and more short of breath.

The scarring that happens in pulmonary fibrosis can be caused by many things. Often, doctors and other healthcare professionals cannot pinpoint what's causing the problem. When a cause cannot be found, the condition is called idiopathic pulmonary fibrosis.

Idiopathic pulmonary fibrosis usually occurs in middle-aged and older adults. Sometimes pulmonary fibrosis is diagnosed in children and infants, but this is not common.

The lung damage caused by pulmonary fibrosis cannot be repaired. Medicines and therapies can sometimes help slow down the rate of fibrosis, ease symptoms and improve quality of life. For some people, a lung transplant might be an option.

Pulmonary fibrosis

Pulmonary fibrosis is scarred and thickened tissue around and between the air sacs called alveoli in the lungs, as shown on the right. A healthy lung with healthy alveoli is shown on the left.

Symptoms

Symptoms of pulmonary fibrosis may include:

How fast pulmonary fibrosis worsens over time and how severe the symptoms are can vary greatly from person to person. Some people become ill very quickly with severe disease. Others have moderate symptoms that worsen more slowly, over months or years.

When symptoms suddenly get worse

In people with pulmonary fibrosis, especially idiopathic pulmonary fibrosis, shortness of breath can suddenly get worse over a few weeks or days. This is called an acute exacerbation. It can be life-threatening. The cause of an acute exacerbation may be another condition or an illness, such as a lung infection. But usually the cause is not known.

When to see a doctor

If you have symptoms of pulmonary fibrosis, contact your doctor or other healthcare professional as soon as possible. If your symptoms get worse, especially if they get worse fast, contact your healthcare team right away.

Causes

Pulmonary fibrosis is scarring and thickening of the tissue around and between the air sacs called alveoli in the lungs. These changes make it harder for oxygen to pass into the bloodstream.

Damage to the lungs that results in pulmonary fibrosis may be caused by many different things. Examples include long-term exposure to certain toxins, radiation therapy, some medicines and certain medical conditions. In some cases, the cause of pulmonary fibrosis is not known.

Your work and surroundings

The type of work you do and where you work or live could be the cause or part of the cause for pulmonary fibrosis. Having continuous or repeated contact with toxins or pollutants — substances that harm the quality of water, air or land — can damage your lungs, especially if you do not wear protective gear. Examples include:

Radiation treatments

Some people who receive radiation therapy to the chest, such as for lung or breast cancer, show signs of lung damage months or sometimes years after treatment. How severe the damage is may depend on:

Medicines

Many medicines can damage the lungs. Some examples include:

Medical conditions

Lung damage can also result from a number of conditions, including:

Idiopathic pulmonary fibrosis

Many substances and conditions can lead to pulmonary fibrosis. Even so, in many people, the cause is never found. But risk factors such as smoking or exposure to air pollution could be related to the condition, even if the cause cannot be confirmed. Pulmonary fibrosis with no known cause is called idiopathic pulmonary fibrosis.

Many people with idiopathic pulmonary fibrosis also may have gastroesophageal reflux disease, also called GERD. This condition occurs when acid from the stomach flows back into the esophagus. GERD may be a risk factor for idiopathic pulmonary fibrosis or cause the condition to worsen faster. But more studies are needed.

Risk factors

Pulmonary fibrosis has been found in children and infants, but this is not common. Idiopathic pulmonary fibrosis is much more likely to affect middle-aged and older adults. Other types of pulmonary fibrosis, such as that caused by connective tissue disease, can occur in younger people.

Factors that can raise your risk of pulmonary fibrosis include:

Complications

Complications of pulmonary fibrosis may include:

Diagnosis

To diagnose pulmonary fibrosis, your doctor or other healthcare professional reviews your medical and family history and does a physical exam. You can talk about your symptoms and review any medicines you take. You also will likely be asked about any continuous or repeated contact with dusts, gases, chemicals or similar substances, especially through work.

During the physical exam, your healthcare professional listens carefully to your lungs while you breathe. Pulmonary fibrosis often occurs along with a crackling sound at the base of the lungs.

You may have one or more of these tests.

Imaging tests

Lung function tests

Also called pulmonary function tests, these are done to find out how well your lungs are working:

In addition to showing whether you have pulmonary fibrosis, imaging and lung function tests can be used to check your condition over time and see how treatments are working.

Tissue sample

If other tests cannot find the cause of your condition, a small amount of lung tissue may need to be removed. This is called a biopsy. The biopsy sample is then examined in a laboratory to diagnose pulmonary fibrosis or rule out other conditions. One of these methods can be used to get a tissue sample:

Blood tests

You may have blood tests to look at your liver and kidney function. Blood tests also can check for and rule out other conditions.

Treatment

The lung scarring and thickening that occurs in pulmonary fibrosis cannot be repaired. And no current treatment has proved effective in stopping the disease from getting worse over time. Some treatments may improve symptoms for a time or slow how fast the disease worsens. Others may help improve quality of life.

Treatment depends on the cause of your pulmonary fibrosis. Doctors and other healthcare professionals evaluate how severe your condition is. Then together you can decide on the best treatment plan.

Medicines

If you have idiopathic pulmonary fibrosis, your healthcare professional may recommend the medicine pirfenidone (Esbriet) or nintedanib (Ofev). Both are approved by the U.S. Food and Drug Administration (FDA) for idiopathic pulmonary fibrosis. Nintedanib also is approved for other types of pulmonary fibrosis that get worse quickly. These medicines may help slow the worsening of pulmonary fibrosis and may prevent bouts when symptoms suddenly get worse.

Nintedanib can cause side effects such as diarrhea and nausea. Side effects of pirfenidone include nausea, loss of appetite and skin rash from sunlight. With either medicine, your healthcare professional uses regular blood tests to check how well the liver is working.

New medicines and therapies are being developed or tested in clinical trials but are not yet approved by the Food and Drug Administration (FDA). Researchers continue to study medicines to treat pulmonary fibrosis.

Doctors may recommend anti-acid medicines if you have symptoms of gastroesophageal reflux disease (GERD). GERD is a digestive condition that commonly occurs in people with idiopathic pulmonary fibrosis.

Oxygen therapy

Using extra oxygen, called supplemental oxygen, cannot stop lung damage, but it can:

You may use oxygen when you sleep or exercise. But some people need oxygen all the time. Carrying a small tank of oxygen or using a portable oxygen concentrator can help you be more mobile.

Pulmonary rehabilitation

Pulmonary rehabilitation can help manage your symptoms and improve your ability to do daily tasks. Pulmonary rehabilitation programs focus on:

Symptoms

When symptoms suddenly get worse

When symptoms suddenly get worse, called an acute exacerbation, you may need more supplemental oxygen. In some cases, you may need mechanical ventilation in the hospital. In this treatment, a tube is guided into the lungs and attached to a machine that helps with breathing. Your healthcare professional may recommend antibiotics, corticosteroid medicines or other medicines when symptoms suddenly get worse.

Treatment

Lung transplant

A lung transplant may be an option for some people with pulmonary fibrosis. Having a lung transplant can improve your quality of life and allow you to live a longer life. But a lung transplant can involve complications such as rejection and infection. After a lung transplant, you take medicines for the rest of your life. You and your healthcare team may discuss a lung transplant if it's thought to be the right treatment option for your condition.

Lifestyle and home remedies

Being actively involved in your treatment and staying as healthy as possible are essential to living with pulmonary fibrosis. It's important to:

Coping and support

Pulmonary fibrosis is a life-long lung condition that worsens over time. Having pulmonary fibrosis can cause fear, depression and stress. Here are some tips that may help you cope.

Preparing for an appointment

If your primary care doctor or other healthcare professional suspects a serious lung problem, you are likely to be referred to a pulmonologist. This is a doctor with training and skills in diagnosing and treating lung disorders.

Pulmonary fibrosis is a serious and complex disease. Take a friend or family member with you to your appointment. That person can take notes while you talk with your healthcare team, provide emotional support and help remember information that you may forget or miss.

What you can do

To get ready for your appointment, make a list of:

Some basic questions to ask include:

Feel free to ask other questions during your appointment.

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