Ask An Expert: Idiopathic Pulmonary Fibrosis

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Ask An Expert: Idiopathic Pulmonary Fibrosis

Question:

What is "idiopathic pulmonary fibrosis?"

Answer:

Idiopathic pulmonary fibrosis (or IPF) is a lung disease of unknown cause that leads to shortness of breath and cough.

Its name tells a lot about it:

• Idiopathic: Cause is unknown

• Pulmonary: Associated with the lungs

• Fibrosis: A scarring process in which the tissue becomes thickened and unable to function normally

The main problem in this condition is that the lungs become filled with scar tissue, preventing the passage of oxygen into the red blood cells; lack of oxygen in the blood causes shortness of breath, especially during exercise when extra oxygen is needed. In severe cases, right heart failure may develop. That's because this condition increases the pressure in blood vessels near the lungs, causing the right side of the heart to work harder than normal to pump blood to the lungs. Right heart failure causes marked leg swelling as fluid backs up in the veins leading to the heart.

A similar condition may develop due to infections, certain occupational exposures (such as asbestos or silica), allergic reactions to drugs, radiation therapy, fumes or moldy hay, autoimmune diseases (such as scleroderma and sarcoidosis), and genetic disease. However, IPF is diagnosed when pulmonary fibrosis is detected and extensive evaluation fails to identify any of these causes. It may take a number of tests to establish the diagnosis, including chest X-ray, CT scan, pulmonary function tests and lung biopsy — these tests are particularly helpful not only to diagnose IPF but also to exclude other causes of the condition.

Because inflammation is thought to trigger the scarring in its early stages, treatment may include anti-inflammatory medicines, such as corticosteroids and other immune-suppressing treatments. Other important measures include avoidance of cigarette smoke and vaccinations to prevent influenza and bacterial pneumonia, because any additional lung damage or inflammation can cause a dangerous deterioration in breathing. In severe cases, or when IPF worsens despite treatment, oxygen therapy and lung transplant may be recommended.

Researchers are actively investigating the causes and best treatments for IPF; progress in these areas should lead to better ways to prevent this condition and its complications.

Robert Shmerling, M.D., is associate physician and clinical chief of rheumatology at Beth Israel Deaconess Medical Center and an associate professor in medicine at Harvard Medical School. He is an active teacher in the Internal Medicine Residency Program, serving as the Robinson Firm Chief. He is also a teacher in the Rheumatology Fellowship Program and has been a practicing rheumatologist for over 25 years.

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Last updated:	January 24, 2007