RARE Daily

BMS Reports Positive Phase 2 Results in IPF

May 23, 2023

Rare Daily Staff

Bristol Myers Squibb reported positive results from a phase 2 study of its experimental therapy BMS-986278 in patients with idiopathic pulmonary fibrosis.

The study showed twice-daily administration of 60 mg of BMS-986278 over 26 weeks reduced the rate of decline in percent predicted forced vital capacity (ppFVC) by 62 percent compared to placebo. The data were presented today at the American Thoracic Society 2023 International Conference held May 19-24, 2023, in Washington, D.C.

Pulmonary fibrosis is a chronic, life-threatening interstitial lung disease (ILD) that occurs when lung tissue becomes damaged and scarred, impacting how lungs function. Progressive pulmonary fibrosis is the preferred term to describe patients who have an ILD with a progressive fibrotic phenotype. Idiopathic pulmonary fibrosis (IPF) is the most common type of progressive fibrosing ILD. As an idiopathic disease, there is no identifiable cause, and as of 2021, more than 700,000 adults are living with IPF globally.

Many people living with PPF and IPF are physically impaired, experience a progressive decline in lung function, have difficulty performing simple daily activities due to breathlessness and require continuous supplemental oxygen to ease the burden of normal breathing.

IPF is a fatal disease with a median survival time of 3-5 years following diagnosis and 5-year survival rate of approximately 45 percent. Innovation in treatment has been limited with few new therapies approved in nearly 10 years.

BMS-986278 is a potential first-in-class, oral, small molecule lysophosphatidic acid receptor 1 (LPA1) antagonist currently being evaluated as a novel antifibrotic treatment for patients with idiopathic pulmonary fibrosis and progressive pulmonary fibrosis. Increased LPA levels and activation of LPA1 have been implicated in the pathogenesis of pulmonary fibrosis. A preclinical in vitro and in vivo study found that antagonizing LPA1 may be beneficial in treating lung injury and fibrosis.

The phase 2 trial was a global, randomized study in which patients with idiopathic pulmonary fibrosis received 30 mg or 60 mg of BMS-986278 or matched placebo orally twice-daily. Overall, 278 patients were randomized and 276 received treatment. The study consisted of a 26-week placebo-controlled treatment period, an optional 26-week active treatment extension period and a 4-week post-treatment follow-up period. Patients were permitted to take background antifibrotic treatment.

The primary endpoint was rate of change in percent predicted forced vital capacity (ppFVC) from baseline to week 26. ppFVC compares the observed FVC to that which is expected for a healthy person of the same age, gender, race, and height. Patients who met prespecified blood pressure reduction criteria were to receive a dose reduction to 10 mg of BMS-986278 or matching placebo twice-daily. A separate cohort of patients with progressive pulmonary fibrosis is ongoing.

“These Phase 2 data give us the confidence to initiate our global Phase 3 clinical trial program where we will continue exploring BMS-986278 as a potentially new and meaningful therapeutic option for people with pulmonary fibrosis,” said Samit Hirawat, chief medical officer, Bristol Myers Squibb.

Photo: Samit Hirawat, chief medical officer, Bristol Myers Squibb

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