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:
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:
Unfortunately, there is currently no cure for idiopathic pulmonary fibrosis, and there is no proven medication therapy to treat it. Medical studies suggest a potential role for several therapies, listed below, but further study is needed.
Reviewed by health care specialists at UCSF Medical Center.