Abstract
BACKGROUND: Idiopathic pulmonary fibrosis (IPF), also called cryptogenic fibrosing alveolitis (CFA), is a lethal form of diffuse lung disorder of unknown origin; the mean survival being two to four years. Currently recommended and most prescribed therapy for IPF is based on the use of systemic corticosteroids, even if no formal demonstration of efficacy of this treatment of IPF is available. Furthermore, new insights from pathological studies have produced a new hypothesis, based upon the central role played by aberrant wound healing following repeated lung injury, weakening the rationale basis of the use of corticosteroids in IPF, previously considered simply a chronic inflammatory disease. OBJECTIVES: The objective of the review was to determine the efficacy of corticosteroids in the treatment of adults with IPF. SEARCH STRATEGY: We searched the Cochrane Central Register of Controlled Trials (The Cochrane Library Issue 2, 2002), MEDLINE (January 1966 to May 2002) and EMBASE (January 1985 to December 2002) and reference lists of articles. We searched reference lists of published articles to identify trials. SELECTION CRITERIA: Randomised controlled trials (RCT) and controlled clinical trials (CCT) using corticosteroids alone for the treatment of adults with IPF. DATA COLLECTION AND ANALYSIS: Abstracts of identified articles were retrieved and articles possibly fulfilling inclusion criteria were retrieved in full. Two reviewers would have independently assessed trial quality if there had been any included studies. MAIN RESULTS: Fifteen studies were selected as potentially eligible for meta-analysis. After further analysis of full text papers, no RCTs or CCTs were identified as suitable and therefore no data was available for inclusion in any meta-analysis. All studies were excluded due to inadequate methodologies. REVIEWER'S CONCLUSIONS: At present, there is no evidence for an effect of corticosteroid treatment in patients with Idiopathic pulmonary fibrosis(IPF)/usual interstitial pneumonia (UIP). Given developments in understanding of the pathogenesis of IPF, randomised controlled trials designed to test the efficacy of corticosteroids will probably never be designed. As other forms of pulmonary fibrosis such as non-specific interstitial pneumonia are reported to show a better response to corticosteroids, it is crucial to make an accurate diagnosis in each patient. Moreover, therapies with immunomodulatory rather than anti-inflammatory or immunosuppressive effects may be more promising for the effective treatment of IPF/UIP.