Interstitial Fibrosis

Content provided by the Faculty of the Harvard Medical School

Interstitial Fibrosis

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Question:

What is interstitial fibrosis? What causes it? Is it associated with asbestosis?

Answer:

Many lung disorders are grouped together under the category "interstitial lung disease," which is also called pulmonary fibrosis, interstitial fibrosis and interstitial pulmonary fibrosis.

In interstitial lung disease, something causes damage to lung tissue. The walls of the air sacs become inflamed, and then scarring (fibrosis) takes place. The lungs become "stiff" and people experience shortness of breath. Shortness of breath may first appear only when exercising, and later get worse. They may also have a dry cough. Heart failure, with associated leg swelling, may occur later.

The main scarring occurs in the interstitium of the lung. This is the lung tissue that sits between the air sacs. That is why the problem is called interstitial lung disease. Scarring leads to a permanent loss of the lung's ability to deliver oxygen to the body. The more scarring, the more breathlessness and disability people have.

There are many different causes of interstitial lung disease. Sometimes doctors can identify a specific reason why someone gets it. Other cases have no known cause (idiopathic).

Asbestos exposure has definitely been associated with the development of interstitial lung disease. Other known causes include exposure to metal dusts and to some farm products like moldy hay.

Certain diseases, including sarcoidosis, scleroderma and rheumatoid arthritis, can also lead to pulmonary fibrosis. So can certain medications (like bleomycin and cocaine) and radiation treatments. There is also a genetic version of the disease.

If no other associated problem is identified, the interstitial lung disease is referred to as "idiopathic" pulmonary fibrosis.

Diana Post, M.D., is an assistant professor of medicine at Harvard Medical School and a member of the Department of Medicine at Brigham and Women's Hospital.

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Last updated:	July 20, 2009

Medical content reviewed by the Faculty of the Harvard Medical School. Harvard Health Publications, Copyright © 2007 by President and Fellows of Harvard College. All rights reserved. Used with permission of StayWell.

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