What is pulmonary fibrosis?
Pulmonary fibrosis (PF) is a disease marked by scarring of the tissue inside and between the air sacs in the lungs. When the scar forms, the tissue becomes stiff and thicker. This makes it harder for oxygen to pass through the walls of the air sac into the bloodstream. Once the lung tissue becomes scarred, the damage cannot be reversed. In most cases, there is no known cause for the disease. There are many causes of pulmonary fibrosis, such as inhalation of toxic dust. However in many cases, there is no identifiable cause, this is usually called "idiopathic" pulmonary fibrosis, although your doctor may use other names such as "usual interstitial pneumonia."
Am I at risk for pulmonary fibrosis?
The list of substances and conditions that can lead to pulmonary fibrosis is long and includes environmental and occupational exposures such as tobacco smoke, autoimmune disorders, and certain medications. Idiopathic pulmonary fibrosis sometimes runs in families, so a genetic component is suspected. Talk to your doctor about your work and health history, and be sure to mention if you have family members with IPF.
What are the symptoms of pulmonary fibrosis?
The symptoms of pulmonary fibrosis can be similar to the symptoms of other lung diseases and can include shortness of breath, cough, shallow breathing, weight loss, fatigue and aching. Some people with PF develop widening and rounding of the tips of the fingers or toes known as clubbing. If you have any of these symptoms, talk to your doctor who will conduct tests to determine if you have pulmonary fibrosis.
How can I have a good quality of life with pulmonary fibrosis?
Ask your doctor if you are eligible for pulmonary rehabilitation. Pulmonary rehabilitation is a program of education and exercise classes that teach you about your lungs and your disease, and how to exercise and be more active with less shortness of breath. The classes take place in a group setting, giving you the chance to meet others with your condition, and both give and receive support. Work with your doctor to brainstorm other ways you can maintain your quality of life.
Am I eligible for any clinical trials?
Clinical trials are being conducted to better understand how pulmonary fibrosis develops and to advance the treatments available. Clinical trials may give you access to new types of treatment being studied. Your doctor can help determine if enrolling in a clinical trial is the right choice for you.
Page last updated: March 22, 2020