Interstitial Lung Disease

Idiopathic Pulmonary Fibrosis

Signs and Symptoms
Diagnosis
Treatment

Signs and Symptoms

Idiopathic pulmonary fibrosis, or IPF, is a condition that causes progressive scarring of the lungs. Fibrous scar tissue builds up in the lungs over time, affecting their ability to provide the body with enough oxygen. The cause of the condition is unknown.

IPF affects more than 100,000 people in the United States, with 30,000 to 40,000 new cases diagnosed each year. Typically the disease is found in people between the ages of 50 and 70 and affects men more frequently than women. Most patients are former smokers. There are no proven risk factors for IPF, but a minority of patients does have a family history of lung scarring.

Symptoms and signs of IPF often appear gradually and include:

• Shortness of breath, particularly during or after physical activity
• Chronic, dry hacking cough
• Crackles in the lungs heard through a stethoscope
• Rounding of the fingernails, a condition called clubbing

Symptoms of IPF may mimic those of other diseases that cause lung scarring, so diagnosing IPF often involves ruling out other conditions. Several visits with your doctor may be needed to finalize your diagnosis and treatment approach.

Reviewed by health care specialists at UCSF Medical Center.
Last updated January 13, 2010