Pulmonary Fibrosis Types and Causes

Idiopathic Pulmonary Fibrosis (IPF)

The most common type of PF is IPF, which stands for idiopathic pulmonary fibrosis. Idiopathic means no known cause. There were 25,192 new cases of IPF and 80,614 total cases based on the total population in 2024. Most patients with IPF start noticing symptoms between the ages of 50 and 70 years old. It is more common in men, however, more women may have the disease than was once thought.

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 (FPF)

PF that runs in the family is rare. PF is considered familial when two or more members within the same family have idiopathic pulmonary fibrosis (IPF) or another form of fibrotic interstitial lung disease. Researchers continue to learn more about the factors that contribute to familial pulmonary fibrosis. While several genes have been linked to FPF, no single gene is responsible for all cases. If someone in your family has had any type of interstitial lung disease, talk to your healthcare provider and a genetic counselor about your possible risk.

PF from Exposure

PF can be caused by environmental or occupational 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  can cause PF.
• Smoking increases a person's risk of developing PF.

Medications

Some medications can cause damage to the lungs, leading to pulmonary fibrosis.

• Chemotherapy
• Some antibiotics
• Some heart medications
• Certain anti-inflammatory medications

 Risk Factors

• Some viral infections may increase the risk of developing pulmonary fibrosis. 
• 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.