Advertisement

How is idiopathic pulmonary fibrosis (IPF) treated?

Howard E. Lewine, MD
Hospitalist
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.

Continue Learning about Restrictive Lung Diseases

What is the rate of incidence for restrictive lung disease?
Honor Society of Nursing (STTI)Honor Society of Nursing (STTI)
Restrictive lung disease is a term that can be applied to several types of specific lung conditions,...
More Answers
Is restrictive lung disease serious?
Honor Society of Nursing (STTI)Honor Society of Nursing (STTI)
Once restrictive lung disease develops, it is irreversible in most cases. Pulmonary fibrosis, which ...
More Answers
What is restrictive lung disease?
Honor Society of Nursing (STTI)Honor Society of Nursing (STTI)
Restrictive lung disease is any condition in which the elasticity of the lungs has been compromised....
More Answers
What are some examples of common restrictive lung diseases?
Honor Society of Nursing (STTI)Honor Society of Nursing (STTI)
Pulmonary fibrosis is a common restrictive lung disease often resulting from overexposure to radiati...
More Answers

Important: This content reflects information from various individuals and organizations and may offer alternative or opposing points of view. It should not be used for medical advice, diagnosis or treatment. As always, you should consult with your healthcare provider about your specific health needs.