Design and rationale of a randomised, double-blind trial of the efficacy and safety of pirfenidone in patients with fibrotic hypersensitivity pneumonitis

Evans R Fernández Pérez, James L Crooks, Jeffrey J Swigris, Joshua J Solomon, Michael P Mohning, Tristan J Huie, Matthew Koslow, David A Lynch, Steve D Groshong, Kaitlin Fier, Evans R Fernández Pérez, James L Crooks, Jeffrey J Swigris, Joshua J Solomon, Michael P Mohning, Tristan J Huie, Matthew Koslow, David A Lynch, Steve D Groshong, Kaitlin Fier

Abstract

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-centre, randomised, 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 computed tomography scan, forced vital capacity (FVC) ≥40% and diffusing capacity of the lung for carbon monoxide ≥30% of predicted values. Study participants will be randomised in a 2:1 ratio to receive pirfenidone 2403 mg·day-1 or placebo. The primary efficacy end-point is the mean change in FVC % predicted from baseline to week 52. A number of secondary end-points have been chosen to evaluate the safety and efficacy in different domains.

Conflict of interest statement

Conflict of interest: E.R. Fernández Pérez reports grants from the NHLBI and Boehringer Ingelheim outside the submitted work. Conflict of interest: J.L. Crooks has nothing to disclose. Conflict of interest: J.J. Swigris has nothing to disclose. Conflict of interest: J.J. Solomon has nothing to disclose. Conflict of interest: M.P. Mohning has nothing to disclose. Conflict of interest: T.J. Huie has nothing to disclose. Conflict of interest: M. Koslow has nothing to disclose. Conflict of interest: D.A. Lynch has nothing to disclose. Conflict of interest: S.D. Groshong has nothing to disclose. Conflict of interest: K.F. Fier has nothing to disclose.

Copyright ©The authors 2021.

Figures

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FIGURE 1
Trial design.

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