Idiopathic Pulmonary Fibrosis

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 have a family history of lung scarring.

Symptoms of IPF often appear gradually and include:

  • Shortness of breath, particularly during or after physical activity
  • Chronic, dry hacking cough
  • Crackle sound 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.

Diagnosing idiopathic pulmonary fibrosis requires input from pulmonologists, radiologists and, in many cases, pathologists experienced in evaluating patients with interstitial lung disease. A face-to-face discussion among these various specialists is often necessary to make an accurate diagnosis.

To determine if you have IPF, your doctor will start by conducting a thorough medical history and physical examination. The medical history will include discussing other medical problems you have that could be related to lung scarring, such as connective tissue diseases, and reviewing any medications you're taking. The physical exam will include listening to your chest with a stethoscope to check for a crackling sound and carefully examining your skin and joints.

In addition to a thorough medical history and physical exam, your doctor may conduct the following tests:

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Two antifibrotic medications — nintedanib and pirfenidone — were approved in the fall of 2014 for use in idiopathic pulmonary fibrosis. While these medications are not a cure, they have both been shown to slow the decline of lung funciton over time.

Ongoing studies of other medications for IPF have shown initial promise, but need more research.

Pulmonary rehabilitation is a structured exercise and education program designed to increase endurance, strength, and exercise capacity, as well as to decrease fatigue and shortness of breath, for people with chronic lung disease, including people with IPF.

Additionally, it is important that any other medical problems that are associated with IPF are treated, such as gastroesophageal reflux disease (GERD) and pulmonary hypertension.

Finally, lung transplant may be an effective treatment option for some people with IPF.

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Reviewed by health care specialists at UCSF Medical Center.