Introduction: BMS-986278, an oral lysophosphatidic acid receptor 1 (LPA) antagonist, has been shown to reduce the rate of decline in percent of predicted FVC (ppFVC) in patients with idiopathic pulmonary fibrosis (IPF) in a phase 2 study (NCT04308681).

Aims & Objectives: To assess BMS-986278 efficacy and safety in the parallel cohort of patients with PPF in NCT04308681.

Methods: Patients with fibrotic interstitial lung disease (ILD) with prior progression within 2 yrs were randomized 1:1:1 to receive placebo (PBO) or BMS-986278 (30mg or 60mg) twice daily. Stable background use of nintedanib, pirfenidone, and/or select immunosuppressives was allowed. Rate of change in ppFVC over 26 wks was evaluated.

Results: Overall, 125 patients were randomized and 123 were treated (mean ppFVC, 66.7%; background antifibrotics, 38%; usual interstitial pneumonia (UIP) pattern, 52%). Unclassifiable ILD was the most common PPF diagnosis (27%). Rates of change in ppFVC were −4.3% (PBO), −2.7% (30mg), and −1.1% (60mg). The treatment difference of BMS-986278 vs PBO was 1.6% (95% CI: −1.0, 4.1) for 30mg and 3.2% (95% CI: 0.7, 5.6) for 60mg, which were relative reductions of 37% and 74%, respectively. The treatment difference was consistent in the presence or absence of background antifibrotics and UIP pattern. For PBO, 30mg, and 60mg, AEs occurred in 78%, 83%, and 67%, respectively; discontinuations due to AEs occurred in 15%, 3%, and 0%, respectively.

Conclusions: In patients with PPF, BMS-986278 reduced the rate of ppFVC decline over 26 wks vs PBO and was safe and well tolerated. These findings, together with the IPF cohort data, support continued development of BMS-986278 in diverse forms of PPF.