Randolph J. Lipchik, MD
Which treatments are effective for idiopathic pulmonary fibrosis (IPF)?
Included studies evaluated treatments for IPF. Reported outcomes varied across studies.
MEDLINE (1996 to May 2010), EMBASE/Excerpta Medica, CINAHL, Cochrane Central Register of Controlled Trials, Cochrane Database of Systematic Reviews, and files of committee members were searched for randomized controlled trials (RCTs), cohort studies, case–control studies, and cross-sectional studies published in English or with English-language abstracts. Studies with preliminary data were excluded. 46 studies, including 20 RCTs, met the selection criteria, evaluated drugs, and could be graded for quality (high, moderate, low, or very low) using the Grading of Recommendations Assessment, Development and Evaluation methodology. Only the results of placebo-controlled RCTs that studied drugs are presented here.
Results for mortality and exacerbation are in the Table. Sildenafil improved quality of life (St. George’s Respiratory Questionnaire mean difference −4.09, 95% CI −0.87 to −7.31, moderate-quality evidence, 1 RCT, n = 180). Based on high-quality evidence (2 RCTs, n = 1156), interferon increased influenza-like illness (relative risk [RR] 2.31, CI 1.78 to 3.01), fever (RR 3.22, CI 2.35 to 4.41), and fatigue (RR 1.35, CI 1.10 to 1.67). Based on moderate- to high-quality evidence (4 RCTs, n = 1015), pirfenidone increased photosensitivity (RR 5.3, CI 1.46 to 19.24), anorexia (RR 3.57, CI 2.15 to 5.93), and stomach discomfort (RR 4.2, CI 2.17 to 8.11). Evidence was insufficient (no RCTs or low or very low-quality RCTs) to evaluate corticosteroid monotherapy, cyclophosphamide, colchicine, cyclosporin A, azathioprine, anticoagulants, and acetylcysteine alone or in combination with corticosteroids and azathioprine.
Current drug treatments do not reduce mortality in idiopathic pulmonary fibrosis, and some increase risk for adverse effects.
Drug treatment vs placebo in idiopathic pulmonary fibrosis*
*NR = not reported; NS = not significant; other abbreviations defined in Glossary. RRR, RRI, and CI calculated from relative risk and control event rates in article. Only drugs with pooled data or recommendations based on moderate- or high-quality evidence using Grading of Recommendations Assessment, Development and Evaluation (GRADE) methodology are reported here.
†Outcome rated as low quality using GRADE methodology.
Lipchik RJ. Review: Current drug treatments do not reduce mortality in idiopathic pulmonary fibrosis. Ann Intern Med. ;154:JC6–8. doi: 10.7326/0003-4819-154-12-201106210-02008
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Published: Ann Intern Med. 2011;154(12):JC6-8.
Interstitial Lung Disease, Pulmonary/Critical Care.
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Print ISSN: 0003-4819 | Online ISSN: 1539-3704
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