Corticosteroid and immunomodulatory agents in idiopathic pulmonary fibrosis

Respir Med. 2004 Nov;98(11):1035-44. doi: 10.1016/j.rmed.2004.07.019.


Idiopathic pulmonary fibrosis (IPF) is a progressive pulmonary disease leading to death within a few years of diagnosis despite medical therapy. On the basis of methodologies of the Cochrane collaboration, this overview discusses the evidence for IPF therapy. Good-quality studies on oral corticosteroids, the most common medical therapy in use 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, gamma interferon, showed a significant effect in a small randomized study, but its efficacy was not confirmed in a larger randomized-controlled trial. 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 with gamma interferon or other immunomodulatory agents.

Publication types

  • Review

MeSH terms

  • Adrenal Cortex Hormones / therapeutic use*
  • Evidence-Based Medicine
  • Humans
  • Immunosuppressive Agents / therapeutic use*
  • Practice Guidelines as Topic
  • Pulmonary Fibrosis / diagnosis
  • Pulmonary Fibrosis / drug therapy*


  • Adrenal Cortex Hormones
  • Immunosuppressive Agents