PT - JOURNAL ARTICLE AU - Evans R. Fernández Pérez AU - James L. Crooks AU - Jeffrey J. Swigris AU - Joshua J. Solomon AU - Michael P. Mohning AU - Tristan J. Huie AU - Matthew Koslow AU - David A. Lynch AU - Steve D. Groshong AU - Kaitlin Fier TI - Design and rationale of randomized, double-blind trial of the efficacy and safety of pirfenidone in patients with fibrotic hypersensitivity pneumonitis AID - 10.1183/23120541.00054-2021 DP - 2021 Jan 01 TA - ERJ Open Research PG - 00054-2021 4099 - http://openres.ersjournals.com/content/early/2021/04/15/23120541.00054-2021.short 4100 - http://openres.ersjournals.com/content/early/2021/04/15/23120541.00054-2021.full AB - Hypersensitivity pneumonitis (HP) is an immunologically mediated form of lung disease resulting from inhalational exposure to any of a large variety of antigens. A subgroup of patients with HP develops pulmonary fibrosis (fibrotic HP, FHP), a significant cause of morbidity and mortality. This study will evaluate the safety and efficacy of the antifibrotic pirfenidone in treating FHP.This single-center, randomized, double-blind, placebo-controlled trial is enrolling adults with FHP (ClinicalTrials.gov: NCT02958917). Study participants must have fibrotic abnormalities involving ≥5% of the lung parenchyma on high-resolution CT scan, forced vital capacity ≥40% and diffusing capacity of the lung for carbon monoxide ≥30% of predicted values. Study participants will be randomized in a 2:1 ratio to receive pirfenidone 2403 mg·d−1 or placebo. The primary efficacy endpoint is the mean change in %FVC from baseline to week 52. A number of secondary endpoints have been chosen to evaluate the safety and efficacy in different domains.FootnotesThis manuscript has recently been accepted for publication in the ERJ Open Research. It is published here in its accepted form prior to copyediting and typesetting by our production team. After these production processes are complete and the authors have approved the resulting proofs, the article will move to the latest issue of the ERJOR online. Please open or download the PDF to view this article.Conflict of interest: Dr. Fernandez Perez reports grants from NHLBI, grants from Boehringer Ingelheim, outside the submitted work.Conflict of interest: Dr. Crooks has nothing to disclose.Conflict of interest: Dr. Swigris has nothing to disclose.Conflict of interest: Dr. Solomon has nothing to disclose.Conflict of interest: Dr. Mohning has nothing to disclose.Conflict of interest: Dr. Huie has nothing to disclose.Conflict of interest: Dr. Koslow has nothing to disclose.Conflict of interest: Dr. Lynch has nothing to disclose.Conflict of interest: Dr. Groshong has nothing to disclose.Conflict of interest: Dr. Fier has nothing to disclose.