Progressive Pulmonary Fibrosis (PPF) is a relentless lung condition characterized by continuous scarring and stiffening of lung tissue, leading to severe breathing difficulties and reduced quality of life. PPF is a subset of pulmonary fibrosis that occurs outside Idiopathic Pulmonary Fibrosis (IPF), encompassing various causes of progressive lung scarring with similar clinical manifestations but different underlying etiologies.

DelveInsight’s Progressive pulmonary fibrosis (PPF) – Epidemiology Forecast – 2032 in the 7MM in 2022, estimated 131,518 diagnosed cases. Better diagnostics, awareness, aging populations, environmental factors, and evolving risk factors drive the increasing prevalence of Progressive Pulmonary Fibrosis (PPF). This underscores the urgent need for research and novel treatments in respiratory medicine to address the complex nature of PPF.

On September 10, 2023, at the European Respiratory Society International Congress, Milan, Italy, a poster titled “Role of nintedanib in the treatment of progressive pulmonary fibrosis (PPF) in daily clinical practice” was presented. Antifibrotic drugs like nintedanib decelerate the advancement of Progressive Pulmonary Fibrosis (PPF) by curbing the over-accumulation of collagen and lung inflammation. This preserves pulmonary function and enhances the overall well-being of patients, offering a viable therapeutic approach for individuals with PPF. The study found that Nintedanib effectively slowed the decline in lung function, providing stability over time. Additionally, it led to significant enhancements in the 6-Minute Walk Test (6MWT), resulting in extended walking distances with minimal oxygen desaturation. However, a substantial proportion of patients necessitated a reduction in drug dosage to alleviate side effects.

On the same day, Dr. Alex West and his colleague presented a poster outlining the earliest look at the data relating to FVC in patients with PPF at ERS. The poster presentation at ERS, titled “Nebulized pirfenidone in non-idiopathic pulmonary fibrosis (IPF) progressive pulmonary fibrosis (PPF): first look at FVC data.” Avalyn Pharma’s primary candidate, AP01, is an inhalable formulation of pirfenidone specially designed for effective delivery through inhalation. The poster presented demonstrated that Nebulized Pirfenidone is not only safe but also capable of achieving a peak epithelial lining fluid concentration (Cmax) approximately 35 times higher than that achieved through standard oral pirfenidone, with less than 1/15th of the systemic absorption. This finding implies that the nebulized administration route promises enhanced effectiveness and improved tolerability. However, further clinical trials and research are warranted in this area.

In the ERS International Congress, another company, Bristol Myers Squibb, presented RCT Abstract-BMS-986278 for progressive pulmonary fibrosis (PPF): results from a Phase II randomized controlled trial on the same day. Among PPF patients, BMS-986278 (60 mg) demonstrated a reduction in the rate of ppFVC decline over 26 weeks compared to a placebo (PBO) (by 69% versus placebo), and it exhibited a favorable safety profile with good tolerability. Previously, findings from the IPF subset of the Phase II trial showcased a remarkable 62% relative decrease in the rate of ppFVC decline when comparing 60 mg of BMS-986278 to a placebo, whether used alone or in conjunction with background therapy. When considered alongside data from the IPF cohort, these findings support the ongoing development of BMS-986278 in various manifestations of PPF. The LPA1 antagonist has yielded encouraging outcomes in a less common manifestation of the condition. This clinical success increases the competitive challenge for two well-established medications in interstitial lung disease: Roche’s Esbriet and Boehringer Ingelheim’s Ofev.

As a result of these promising results, BMS-986278 will undergo further evaluation as part of the worldwide Phase III ALOFT (An LPA1 antagonist for Pulmonary Fibrosis Trial) initiative.

DelveInsight’s Progressive Pulmonary Fibrosis (PPF) – Market estimates the market of PPF in the 7MM to be USD 1,042 million. In conclusion, the data showcased at ERS conferences, particularly concerning BMS-986278, holds immense promise for respiratory medicine. Fueled by the insights and innovations presented at ERS conferences, this community stands at the forefront of progress in treating and managing Progressive Pulmonary Fibrosis (PPF).