Symptoms, Diagnosis and Treatment

What Are the Symptoms of Pulmonary Fibrosis?

  • Shortness of breath, particularly during exercise
  • Dry, hacking cough
  • Fast, shallow breathing
  • Gradual unintended weight loss
  • Tiredness
  • Aching joints and muscles
  • Clubbing (widening and rounding of the tips of the fingers or toes)

How Is Pulmonary Fibrosis Detected?

Your doctor will do a physical exam. Your doctor may ask about your history of smoking, things in the air at your job that could irritate your lungs, your history of drug use, other medical conditions, your family's medical history, and your hobbies.

Other tests for pulmonary fibrosis may include:

  • Chest X-ray
  • Lung function tests to find out how much lung damage you have
  • Blood tests to check for oxygen levels in your bloodstream and for possible infections
  • Bronchoalveolar lavage, a test that removes and examines cells from the lung.  The test is done during bronchoscopy, a special examination of the lung.
  • Lung biopsy, done during bronchoscopy or as a surgical procedure. The biopsy removes a sample of lung tissue for your doctor to study.
  • CT scan
  • Exercise testing to find out how well your lungs move oxygen and carbon dioxide in and out of your bloodstream.

How Is Pulmonary Fibrosis Treated?

There is no cure for pulmonary fibrosis. Current treatments are aimed at preventing more lung scarring, relieving symptoms and helping you stay active and healthy. Treatment cannot fix lung scarring that has already occurred.

Treatments include:

Medicine. Your doctor may prescribe prednisone to reduce inflammation. Prednisone is a corticosteroid. It can have serious side effects. Other medicines sometimes given for pulmonary fibrosis are azathioprine and cyclophosphamide, which suppress the immune system. They also can cause serious side effects.

The FDA has approved two other drugs for pulmonary fibrosis, nintedanib (Ofev®) and pirfenidone (Esbriet®, Pirfenex®, Pirespa®). Both have been shown to slow worsening of pulmonary fibrosis in mild to moderate cases.

These treatments may help prevent further scarring and increase survival time in some patients, but they don't work for everyone with pulmonary fibrosis.

Oxygen therapy may be prescribed if the amount of oxygen in your blood gets low. Supplemental oxygen therapy may help reduce your shortness of breath and make it easier for you to stay active.

Pulmonary rehabilitation is a program that teaches you about your lung disease, how to exercise and how to manage your disease, and provides support and counseling.

Lung transplant may be recommended for you if you are younger than 65, have no other medical problems and are not being helped by medicines.

You may be a candidate for a clinical trial of a new pulmonary fibrosis treatment. Information on clinical trials can be found at

Learn about living with pulmonary fibrosis