Browse ATS 2021 Abstracts

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ATS 2021 will feature presentations of original research from accepted abstracts. Mini Symposia and Thematic Poster Sessions are abstract based sessions.

Please use the form below to browse scientific abstracts and case reports accepted for ATS 2021. Abstracts presented at the ATS 2021 will be published in the Online Abstract Issue of the American Journal of Respiratory and Critical Care Medicine, Volume 203, May 3, 2021.

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Pirfenidone for Progressive Fibrotic Sarcoidosis (PIRFS): Results of a Double Blind Placebo Controlled Pilot Study

Session Title
TP25 - TP025 SARCOIDOSIS: DIAGNOSIS, PROGNOSIS, AND TREATMENT
Abstract
A1829 - Pirfenidone for Progressive Fibrotic Sarcoidosis (PIRFS): Results of a Double Blind Placebo Controlled Pilot Study
Author Block: R. P. Baughman1, R. Gupta2, M. A. Judson3, E. E. Lower1, J. I. Stewart4, R. Reeves5, A. U. Wells6; 1Univ of Cincinnati Med Ctr, Cincinnati, OH, United States, 2Thoracic Medicine and Surgery, Temple University, Philadelphia, PA, United States, 3Div of Pulm/CCM, Albany Medical College, Albany, NY, United States, 4Temple Lung Center, Philadelphia, PA, United States, 5ILD/Sarcoidosis, University of Cincinnati, Cincinnati, OH, United States, 6Respiratory, Royal Brompton Hospital, London, United Kingdom.
Introduction: Over 25% of patients with fibrotic pulmonary sarcoidosis (FS) die from respiratory failure but many patients have stable disease for years. Pulmonary function test (PFT) and high resolution computer tomography (HRCT) findings have been shown to predict mortality in FS. Pirfenidone reduces the rate of progression in idiopathic pulmonary fibrosis. We evaluated the usefulness of PFT and HRCT in predicting progression over 18 months period in a double blind, placebo controlled trial of patients with FS. Methods: Patients with FS were evaluated at three sarcoidosis sites. Sarcoidosis patients with a composite physiologic index (CPI) of >40 or more than 20% fibrosis on HRCT were eligible. Patients receiving treatment for pulmonary hypertension were excluded. Initial evaluation included forced vital capacity (FVC), diffusion lung carbon monoxide (DLCO), HRCT, and six minute walk distance (6MWD). Patients were randomized to pirfenidone (PIR) or placebo (PLA) [2:1 ratio]. The dosage of pirfenidone (or placebo) was increased from one 267 mg capsule (or placebo) three times a day to three tablets three times a day over a six week period as tolerated. Patients were seen every three months with spirometry and 6MWD determination at each visit. The primary endpoint was time to clinical worsening (TCW) [death, lung transplant, or >absolute 10% drop in percent predicted FVC]. Secondary endpoints included changes in FVC % predicted and 6MWD at end of study visit. Results: Sixteen patients were enrolled, with one patient (assigned PLA) withdrawing before receiving the first dose. Of 15 treated patients, 4 (27%) reached TCW (1 death, 1 lung transplant, 2 with >10% absolute change in FVC % predicted). All four had a DLCO of <40% predicted. Four of 8 patients with DLCO <40% met the TCW endpoint (Figure). TCW was shorter with DLCO <40% predicted versus >40% predicted (Logrank Chi square=5.3018, p=0.0213). TCW, change in DLCO, and change in FVC did not differ significantly between the 4 PLA treated patients and the 11 PIR treated patients. No severe adverse events occurred. Conclusion: In this pilot study, we found that a quarter of pulmonary fibrotic sarcoidosis patients with a CPI >40 or more than 20% fibrosis on HRCT met predefined TCW events within the 18 months of the study. Only patients with DLCO <40% predicted or CPI>40 met TWC. This small study was underpowered to determine pirfenidone efficacy. Future studies of anti-fibrotic drugs should consider restricting to those with a DLCO <40% predicted.