What Is Pulmonary Fibrosis?

Pulmonary fibrosis is an interstitial lung disease in which scarring (fibrosis) of lung tissue makes it difficult for oxygen to get to the blood, resulting in shortness of breath that worsens over time. Many cases have no known cause, although exposure to certain toxins, medications, allergies, and other factors have been implicated. There's no cure for pulmonary fibrosis, but there are effective treatments for managing symptoms and improving quality of life.

Pulmonary Fibrosis Symptoms

The first symptom of pulmonary fibrosis is usually shortness of breath with physical exertion that over time progresses to difficulty breathing while at rest. Additional symptoms of the condition include:

• A dry cough
• Unintended weight loss
• Discomfort in the chest
• Fatigue
• Muscle and joint aches
• Enlarged fingertips and curved nails (clubbing) 

The progression of the disease can vary significantly. For some people, it can take many years for breathing to become difficult even without activity, while others may have a more rapid decline.

Causes

In pulmonary fibrosis, the walls of the alveoli (tiny air sacs in the lungs) become scarred, preventing them from expanding and contracting while breathing. For most people, the condition has no discernible cause, in which case it's regarded as idiopathic pulmonary fibrosis. About 50,000 people are diagnosed with idiopathic pulmonary fibrosis each year.

Among the known potential causes of pulmonary fibrosis are:

• Occupational exposure to toxins: Hazardous substances, such as asbestos, coal, and silica dust, have been implicated.
• Drug toxicity: Certain medications have been associated with pulmonary fibrosis.
• Other diseases: Conditions like rheumatoid arthritis, scleroderma, hypersensitivity pneumonitis, sarcoidosis, and Sjögren’s syndrome can cause the body’s immune system to attack the lungs. Gastroesophageal reflux disease (GERD) also can increase the risk of pulmonary fibrosis when drops of acid from the stomach are breathed in, damaging the lungs over time.
• Radiation therapy: When this treatment is used for lung cancer, breast cancer, or lymphoma it can injure the lungs and lead to pulmonary fibrosis.

Diagnosis

Your doctor will first perform a physical exam and listen to your lungs. A crackling sound can be a sign that you have pulmonary fibrosis. They’ll ask about any family history or exposure that you may have had to hazardous materials. 

You doctor will order a combination of imaging tests, such as an X-ray and CT (computerized tomography) scan of your chest to confirm the diagnosis. They may also order tests to evaluate how well your lungs are working, including pulmonary function tests, pulse oximetry, exercise stress tests, and an arterial blood gas test.

Treatment

There's no cure for pulmonary rehabilitation, but it can be managed. Treatment is focused on easing symptoms and slowing progression of the disease, and is based on the cause of the condition and how severe it is.

The first step in treatment is to address the underlying cause if it is known. That could mean taking medication for a medical condition like GERD or stopping exposure to a hazardous substance like asbestos.

Treatment strategies for pulmonary fibrosis include:

• Oxygen therapy: Your doctor may prescribe oxygen therapy if your oxygen level is 88% or lower. Using oxygen can help ease symptoms like shortness of breath and fatigue.
• Pulmonary rehabilitation: Pulmonary rehabilitation programs are designed to strengthen muscles involved in breathing, teach safe exercise practices, and improve quality of life. Such a program could include exercise training, breathing exercises, and health education. A pulmonary rehabilitation team usually is made up of doctors, nurses, respiratory therapists, exercise specialists, dietitians, and social workers.
• Medications: Ofev (nintedanib) and Esbriet (pirfenidone) are approved to treat idiopathic pulmonary fibrosis by slowing the progression of the disease. In addition, medications may be used to reduce lung inflammation in non-idiopathic pulmonary fibrosis. These medications include prednisone and CellCept (mycophenolate mofetil/mycophenolic acid).
• Surgery: In some cases, a lung transplant may be an option. In 2015, of about 2,000 people who had a lung transplant, half had pulmonary fibrosis.

Coping

When diagnosed with a chronic condition like pulmonary fibrosis, it’s normal to feel vulnerable, anxious, and stressed about what to expect. Fatigue may make it hard to do normal activities, which can affect mood as well. You can find support by reaching out to others for help. Some ways to do this:

• Talk to your healthcare provider, not just about your physical symptoms but your emotions as well. They can provide help and suggestions in dealing with stress and anxiety. 
• Connect with others who have pulmonary fibrosis. You can join in-person support groups, like the American Lung Association’s Better Breathers Club, or find supportive online communities.
• Ask your doctor about palliative care to help manage your symptoms. A palliative care team includes specialists like doctors, nurses, social workers, and nutritionists who work together to help you feel more comfortable and improve your quality of life.
• Make time for the things you enjoy, including visits with friends and favorite hobbies. Oxygen therapy may help you resume these activities with less shortness of breath.
• Allow yourself time to rest when your body needs to recover. Ask for help with chores from family, friends, and caregivers.
• Call the American Lung Association’s Lung HelpLine for free at 1-800-LUNGUSA. You can talk to a medical professional who can help you find the support you need and offer suggestions for your care.

A Word From Verywell

While pulmonary fibrosis doesn’t have a cure, there are options for treatments to slow the progression, reduce symptoms, and improve your quality of life. Tell your doctor about any symptoms or side effects you’re experiencing. Ask for help with any challenges you’re facing in your daily activities. Being your own advocate can help you feel more in control of your disease, and in some cases, it can even improve outcomes.