Pulmonary Fibrosis Types and Causes

Pulmonary fibrosis (PF) is a form of interstitial lung disease that indicates scarring in the lungs. There are over 200 different types of interstitial lung disease.

Idiopathic Pulmonary Fibrosis (IPF)

The most common type of PF is IPF, which stands for idiopathic pulmonary fibrosis. Idiopathic means no known cause. Approximately 35,000 new cases of IPF are diagnosed each year. Most patients with IPF start noticing symptoms between the ages of 50 and 70 years old. It is more common in men, but the number of cases of IPF in women is on the rise.

PF from Autoimmune Diseases

  • Sometimes PF is caused by autoimmune diseases like rheumatoid arthritis, scleroderma or Sjogren's syndrome. In this case, your body’s immune system mistakenly attacks the lung tissue (in addition to other tissues), leading to chronic inflammation and scarring.  

Familial PF

PF that runs in a family is rare. PF is considered familial when two or more members within the same family have idiopathic pulmonary fibrosis (IPF) or a form of idiopathic interstitial pneumonia (IIP). There are genes that have been linked to PF, but much is still unknown about this field. If someone in your family has had any type of interstitial lung disease, be sure to speak with your healthcare provider and a genetic counselor about your risk .

PF from Exposure

PF can be caused by exposure to hazardous materials.  

  • Occupational exposures include breathing in fibers, fumes, vapors and dust which may include asbestos, coal or silica. 
  • Breathing in organic materials such as mold, bird feathers and droppings or animal dander may cause an allergic reaction that rarely progresses to PF. 
  • Radiation treatment to the chest and certain types of medications can cause PF.
  • Smoking increases a person's risk of developing PF.

Other Causes of PF

  • Viral infections can sometimes lead to abnormal healing and scarring in the lungs. 
  • Gastroesophageal reflux disease (GERD), a condition in which acid from your stomach backs up into your throat, can also put you at risk for developing PF. Some people with GERD may breathe in tiny drops of acid from their stomachs, which can injure their lungs.

Page last updated: August 21, 2025

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