title: Non-steroid agents for idiopathic pulmonary fibrosis.
abstract: BACKGROUND
  Idiopathic pulmonary fibrosis is a chronic progressive lung disease with poor outcome and no effective treatment to date. This is an update of a Cochrane Review first published in 2003.
  
  
  OBJECTIVE
  To assess the efficacy of non-steroid agents in adults with idiopathic pulmonary fibrosis.
  
  
  METHODS
  We searched the Cochrane Airways Group Register (30 March 2010), the Cochrane Central Register of Controlled Trials (CENTRAL) (The Cochrane Library, Issue 1, 2010), Ovid MEDLINE to March week 5, 2010, EMBASE to week 13, 2010 and PubMed to April 2010, with additional handsearching, including abstracts of international conferences. We also contacted pharmaceutical companies and researchers in the field.
  
  
  METHODS
  Randomised studies comparing non-steroid drugs with placebo or steroids in adults with idiopathic pulmonary fibrosis.
  
  
  METHODS
  Two authors independently assessed trial quality, extracted data and assessed risk of bias. We contacted pharmaceutical companies to obtain missing information, if any. We combined survival outcomes using Peto odds ratios or hazard ratios (HR).
  
  
  RESULTS
  Fifteen trials involving 10 different drugs were included. Two trials enrolling 1156 patients compared interferon gamma-1beta with placebo: interferon gamma-1beta did not significantly improve survival (HR 0.88, 95% CI 0.47 to 1.64; P = 0.68). Four trials involving 1155 patients compared pirfenidone with placebo. Three trials, conducted in 1046 patients, provided data on progression-free survival: pirfenidone significantly reduced the risk of disease progression by 30% (HR 0.70, 95% CI 0.56 to 0.88, P = 0.002). Data on the effect of pirfenidone on pulmonary function could only be assessed for two studies analysing 314 patients. Forced vital capacity or vital capacity was significantly improved by pirfenidone (mean difference 0.08 L, 95% CI 0.03 to 0.13, P = 0.0006).
  
  
  CONCLUSIONS
  Based on available data, partly still unpublished, pirfenidone appears to improve progression-free survival and, to a lesser extent, pulmonary function in patients with idiopathic pulmonary fibrosis. More data are needed on overall survival and quality of life on treatment. From the studies in this review, interferon gamma-1beta has not been shown to affect survival. Other agents evaluated in single studies either failed to provide evidence for a benefit or need to be assessed in larger randomised controlled trials.
authors.affiliation: Center for Rare Lung Disease, University of Modena and Reggio Emilia, Modena Italy and Respiratory Disease Section, Department of Oncology, Hematology and Respiratory Disease, University of Modena and Reggio Emilia, Modena, Italy.
authors.forename: Paolo
authors.initials: P
authors.lastname: Spagnolo
authors.forename: Cinzia
authors.initials: C
authors.lastname: Del Giovane
authors.forename: Fabrizio
authors.initials: F
authors.lastname: Luppi
authors.forename: Stefania
authors.initials: S
authors.lastname: Cerri
authors.forename: Sara
authors.initials: S
authors.lastname: Balduzzi
authors.forename: E Haydn
authors.initials: EH
authors.lastname: Walters
authors.forename: Roberto
authors.initials: R
authors.lastname: D'Amico
authors.forename: Luca
authors.initials: L
authors.lastname: Richeldi
mesh_terms: D000894:Anti-Inflammatory Agents, Non-Steroidal / Q000627:therapeutic use; D001379:Azathioprine / Q000627:therapeutic use; D003078:Colchicine / Q000627:therapeutic use; D003520:Cyclophosphamide / Q000627:therapeutic use; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D007371:Interferon-gamma / Q000627:therapeutic use; D011241:Prednisone / Q000627:therapeutic use; D011658:Pulmonary Fibrosis / Q000188:drug therapy*; D011728:Pyridones / Q000627:therapeutic use; D016032:Randomized Controlled Trials as Topic; D011994:Recombinant Proteins
chemical_list: D000894:Anti-Inflammatory Agents, Non-Steroidal; D007166:Immunosuppressive Agents; D011728:Pyridones; D011994:Recombinant Proteins; D007371:Interferon-gamma; D003520:Cyclophosphamide; C093844:pirfenidone; D001379:Azathioprine; D003078:Colchicine; D011241:Prednisone