Drug Therapy for Treatment of Idiopathic Pulmonary Fibrosis: Systematic Review and Network Meta-Analysis.
Chest. 2016 Jan 13;
Authors: Canestaro W, Forrester S, Raghu G, Ho L, Devine B
BACKGROUND: Idiopathic pulmonary fibrosis (IPF) is a form of chronic progressive fibrosing interstitial lung disease of unknown origin. Recently, nintedanib and pirfenidone demonstrated efficacy in slowing disease progression and were approved by the US Food and Drug Administration. Although numerous treatments have been evaluated in IPF, none have shown significant decreases in mortality. The objective of this study was to identify all pharmacological treatments evaluated as a treatment for IPF and analyze their efficacy via Bayesian network meta-analysis and pairwise indirect treatment comparisons. This review did not evaluate the effect of steroid therapy.
METHODS: We searched MEDLINE, EmBase and the Cochrane Library for studies published on or before August 2014. Studies were required to contain a randomized evaluation of non-steroidal drug therapy for treatment of IPF and be published in English. The key outcomes of interest for this analysis were pulmonary function as measured by forced vital capacity as well as all-cause and respiratory-specific death. All outcomes were analyzed via a Bayesian framework.
RESULTS: Our review identified 30 eligible studies that evaluated 16 unique treatments. Under both the fixed effect and random effect model for respiratory-specific mortality no treatments performed better than placebo. For all-cause mortality, pirfenidone and nintedanib had effects approaching significance with credible intervals slightly crossing the null under a fixed effect model. Notably, for respiratory-specific mortality, all-cause mortality, and decline in percent predicted forced vital capacity nintedanib and pirfenidone were virtually indistinguishable and no clear advantage was detected.
CONCLUSIONS: While two treatments have been approved for the treatment of IPF on the basis of reduced decline in pulmonary function, no treatment has a clear advantage on mortality outcomes.
PMID: 26836914 [PubMed - as supplied by publisher]