Pulmonary Fibrosis

What is pulmonary fibrosis?

Pulmonary fibrosis is a lung disease that makes it hard to breathe. The disease is progressive, which means it will keep getting worse over time. 

Healthy lungs are made up of air sacs that expand like balloons so that the body can take in oxygen. In lungs with pulmonary fibrosis, the air sacs develop scar tissue (fibrosis). Over time, the scar tissue destroys the normal lung tissue and makes it hard for oxygen to get into your blood. Fibrosis causes shortness of breath and often a cough that never goes away.

It’s important to seek treatment to help improve your breathing and slow the progression of the disease.

Most common symptoms

Symptoms of pulmonary fibrosis usually come gradually over a number of months. They include:

• Dry cough (not very much phlegm).
• Shortness of breath, especially with any kind of activity or exercise
• Weight loss without dieting. People spend more energy trying to breathe. This can lead to weight loss.

What is the main cause of pulmonary fibrosis?

In many cases, the cause of pulmonary fibrosis is not known. But cigarette smoking is a big risk factor. Some people have inherited a predisposition to get it.

In rare instances, a more reversible form of the disease can be caused by long-standing environmental exposure (mold, animals, or other triggers), some medicines (like chemotherapy), radiation treatment, occupational exposures, and some connective tissue diseases (like rheumatoid arthritis and scleroderma). These don’t usually progress to permanent pulmonary fibrosis.

These factors may increase your risk:

• Cigarette smoker.
• Male, over 50 years old.
• Acid reflux disease.
• You have rheumatoid arthritis or lupus.
• Family history of pulmonary fibrosis. About 20% of people with pulmonary fibrosis have a family member with it, according to the Pulmonary Fibrosis Foundation. Hair turning gray early might be a sign of genetic pulmonary fibrosis.
• Work history involves metalworking, woodworking, sandblasting, or time spent in chemical factories.

Next steps

If you have pulmonary fibrosis, you should see a doctor. It is usually not an emergency unless your symptoms get worse quickly over hours or a few days. Talk with your doctor about seeing a pulmonologist.

Various tests may be ordered to make a diagnosis including a CT scan of the lungs and blood work. There is no single blood test to diagnose pulmonary fibrosis. Results of certain tests can still be helpful.

You may be given a 6-minute walk test to measure your oxygen during exercise. You may take a pulmonary function test to check how well your lungs are working.

Sometimes, even with these tests, the diagnosis is not clear. You may need a lung biopsy—a small sample of tissue from the lungs is removed and tested. It’s typically done as an outpatient procedure.

Pulmonary fibrosis treatment

Right now there is no cure for pulmonary fibrosis. While there aren’t any medications to reverse it, some can slow the progression of mild to moderate disease. Treatments can also make symptoms better and help you breathe. They can also minimize flare ups that can lead to hospital visits.

These medications and therapies can improve your breathing:

• Pirfenidone (Esbriet) and Nintedanib (Ofev): These medications help prevent new scarring and may slow down progression of the disease from months to years.
• Oxygen: Some patients with severe fibrosis will need to breathe oxygen from a tank to get relief.
• Pulmonary rehabilitation: a special form of physical therapy specifically for patients with lung diseases to improve their breathing. It’s done several times a week.
• Your doctor may prescribe other medications such as antibiotics, steroids, rituximab (Rituxan), and mycophenolate mofetil.

It’s important to try to avoid getting sick from colds and pneumonias. Stay up-to-date on all vaccines, especially the flu shot and pneumonia vaccine. If you smoke or vape, quit.

If the disease is severe or progressing rapidly, your doctor may suggest a lung transplant. Transplantation is a very long process. It requires a huge commitment from you and your family.

The transplant requires a long recovery and a lifetime of medications. This prevents your body from rejecting the new lung. Discuss transplantation with a pulmonologist at an accredited transplant center.