Ann Am Thorac Soc. 2022 May 2. doi: 10.1513/AnnalsATS.202103-342OC. Online ahead of print.
ABSTRACT
Background: To inform an American Thoracic Society, European Respiratory Society, Japanese Respiratory Society, and Asociación Latinoamericana del Tórax clinical practice guideline, this systematic review evaluated existing interstitial lung disease literature to determine whether patients with progressive pulmonary fibrosis (PPF) should be treated with the antifibrotic pirfenidone. Methods: A literature search was conducted across MEDLINE, EMBASE, and Cochrane databases through December 2020 for studies using pirfenidone to treat patients with PPF. Mortality, disease progression, lung function, and adverse event data were extracted, and meta-analyses performed when possible. The Grading of Recommendations, Assessment, Development and Evaluation (GRADE) Working Group approach was used to assess the quality of evidence. Results: Two studies met inclusion criteria. Meta-analyses revealed changes in FVC % predicted [mean difference (MD) 2.3% (95% CI 0.5-4.1)], FVC in mL [MD 100.0 mL (95% CI 98.1-101.9)], and 6MWD in meters [MD 25.2 m (95% CI 8.3-42.1)] all favored pirfenidone to placebo. Change in DLCO in mmol/kPa/min [MD 0.40 mmol/kPa/min (95% CI 0.10-0.70)] and risk of DLCO declining more than 15% [relative risk (RR) 0.27 (95% CI 0.08-0.95)] also favored pirfenidone. The risks of gastrointestinal discomfort [RR 1.83 (95% CI 1.29-2.60)] and photosensitivity [RR 4.88 (95% CI 1.09-21.83)] were higher with pirfenidone. The quality of evidence was low or very low GRADE, depending on the outcome. Conclusions: Pirfenidone use in patients with PPF is associated with statistically significant decrease in disease progression and protection in lung function. However, there is very low certainty in the estimated effects due to limitations in the available evidence.
PMID:35499847 | DOI:10.1513/AnnalsATS.202103-342OC
