How is idiopathic pulmonary fibrosis (IPF) treated?

Howard E. Lewine, MD
Idiopathic pulmonary fibrosis is a poorly understood disease. In fact, any disease that is called "idiopathic" usually means that the cause is not known.

Early on in the disease, there is inflammation in the lungs. But the main problem in pulmonary fibrosis is that scar tissue replaces the normal breathing sacs (alveoli) in the lungs.

Inflammation may not be the reason scar tissue forms. (The inflammation might be an innocent bystander.) In fact, many strong anti-inflammatory drugs have been tried with minimal success.

Even so, lung specialists often recommend a trial of corticosteroids such as prednisone or methylprednisolone. This may be combined with another anti-inflammatory drug such as azathioprine.

Researchers are studying drugs that may be able to stop the fibrosis (the formation of scar tissue). One drug is pirfenidone. The Food and Drug Administration is reviewing this drug. It is approved for treating pulmonary fibrosis in other countries.

Another drug that shows some promise is bosentan (Tracleer). Animal studies show that bosentan can decrease scarring. The results of human studies so far are somewhat encouraging. Additional studies are under way.

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