Therapeutic approaches to idiopathic pulmonary fibrosis

Idiopathic pulmonary fibrosis (IPF) is a progressive pulmonary disease leading to death within a few years of diagnosis despite medical therapy. Based upon the methodologies of the systematic reviews of the Cochrane collaboration, this article describes the available evidence for the therapy of IPF. Good-quality studies on oral corticosteroids, the most commonly used medical therapy for IPF, are lacking. A few small studies have been carried out on the efficacy of many non-steroid immunosuppressive agents, and the results have been generally disappointing. The most extensively studied medical therapy, γ interferon, showed a significant effect in a first small randomized study, but its effect on survival has not been confirmed in a larger randomized, controlled trial. Preliminary positive data on high doses N-acetylcysteine have also been reported. The long-awaited good news for patients affected by this deadly disease and for their physicians could come in the near future from large randomized, controlled trials using gamma interferon and/or other immunomodulatory agents.