[Drug treatments for idiopathic pulmonary fibrosis]

Idiopathic pulmonary fibrosis is a disease of unknown cause characterized by cough, progressive dyspnea, restrictive respiratory disorder, a typical honeycomb aspect on the high-resolution CT-scan, and usual interstitial pneumonia at histological examination of the lung biopsy. Most patients die 3 to 8 years after diagnosis. Current treatment is based on a combination of corticosteroids and immunosuppressants, but the efficacy of treatment remains a matter of debate. New therapeutics currently under evaluation in controlled clinical trials include interferon-gamma, pirfenidone, N-acetylcysteine, etanercept (anti-TNFalpha), bosentan (endothelin receptor antagonist), imatinib (tyrosine-kinases inhibitor of the PDGF receptor), etc. At the same time, new compounds showing efficacy in experimental models of fibrosis and the development of new pathophysiological concepts open new perspectives both in terms of concept and clinical practice.