Currently there is no evidence to support the routine use of corticosteroids alone in the management of idiopathic pulmonary fibrosis.

Idiopathic pulmonary fibrosis (IPF) is a chronic disorder of unknown cause that is progressive and leads to death. Existing evidence about the treatment of IPF is difficult tointerpret because of evolving diagnostic criteria and classification schemes, grouping together diverse entities with markedly different natural histories and responses totherapy. Therefore, because the old studies did not include only IPF patients, their results cannot be extended to IPF as they are currently defined. In addition, methodologic problems mainly related to the lack of randomized placebo-controlled treatment trials have made evidence-basedconclusions difficult. A systematic search for high quality placebo-controlled randomised trials comparing corticosteroid treatment with placebo in subjects with a diagnosis of IPF was unable to identify any studies suitable for inclusion in the review.

Authors' conclusions: 

At present, there is no evidence for an effect of corticosteroid treatment in patients with IPF. On the other hand, other fibrotic lung diseases, such as non-specific interstitial pneumonia (NSIP), are reported to show a better response to corticosteroids. Making a clear distinction between IPF and other entities grouped under the umbrella term interstitial lung disease is, therefore, essential as this may have therapeutic and prognostic implications.

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Background: 

Idiopathic pulmonary fibrosis (IPF) is a disease with significant morbidity and mortality. Patients short survival time, high mortality and generally rapid decline raise the importance of early treatment. Current guidelines suggest a combination of corticosteroids and immunosuppressants as "gold standard" for IPF treatment, although the evidence for this recommendation is weak. Based on animal models, it has been hypothesized a central role for aberrant wound healing following repeated epithelial lung injury, weakening the rationale for using corticosteroids in IPF, previously thought to be a chronic inflammatory disease.

Objectives: 

The aim of the review is to determine the efficacy of corticosteroids in the treatment of adults with familial and sporadic IPF.

Search strategy: 

We searched the Cochrane Central Register of Controlled Trials (The Cochrane Library Issue 2, 2008), MEDLINE (January 1966 to June 2008) and EMBASE (January 1985 to June 2008) and reference lists of articles. We searched reference lists of published articles to identify clinical 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 study.

Main results: 

Seventeen articles 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.

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