Idiopathic pulmonary fibrosis

N Engl J Med. 2001 Aug 16;345(7):517-25. doi: 10.1056/NEJMra003200.


Idiopathic pulmonary fibrosis is a rapidly progressive illness of unknown cause characterized by sequential acute lung injury with subsequent scarring and end-stage lung disease. Treatment at present remains largely supportive, with evidence that patients' satisfaction and survival may be improved by referral to centers specializing in the evaluation of interstitial lung diseases. Although no drug therapy has clearly been demonstrated to benefit patients with idiopathic pulmonary fibrosis, a number of novel investigational agents hold promise for future study. Given the poor prognosis associated with idiopathic pulmonary fibrosis, patients should be referred to regional centers of expertise for enrollment in therapeutic clinical trials or for lung transplantation.

Publication types

  • Research Support, U.S. Gov't, P.H.S.
  • Review

MeSH terms

  • Adjuvants, Immunologic / therapeutic use
  • Anti-Inflammatory Agents / therapeutic use
  • Disease Progression
  • Fibrinolytic Agents / therapeutic use
  • Humans
  • Lung / diagnostic imaging
  • Lung Transplantation
  • Pulmonary Fibrosis* / diagnosis
  • Pulmonary Fibrosis* / drug therapy
  • Pulmonary Fibrosis* / etiology
  • Pulmonary Fibrosis* / surgery
  • Radiography
  • Steroids


  • Adjuvants, Immunologic
  • Anti-Inflammatory Agents
  • Fibrinolytic Agents
  • Steroids