European Respiratory Society (ERS) International Congress 2023 is taking place in Milan, Italy, from September 9 to September 13, focusing on respiratory diseases, including Idiopathic Pulmonary Fibrosis (IPF).  

Idiopathic Pulmonary Fibrosis (IPF) is a progressive lung disease of unknown cause, characterized by lung tissue scarring (fibrosis). It leads to impaired lung function, breathlessness, and reduced quality of life. IPF has no known cure, and treatment focuses on managing symptoms and slowing disease progression.

A concerning trend over the past decade has been the notable increase in reported cases of IPF, prompting urgent discussions and research into this enigmatic condition. According to data from DelveInsight, an estimated 193,870 individuals have received a diagnosis of IPF across the 7MM (the United States, EU4 countries [Germany, France, Italy, and Spain] and the UK, and Japan]. Approximately 94,580 cases are estimated to have been diagnosed for IPF, specifically in the US, forming around half of the 7MM diagnosed population. The approach to treating Idiopathic Pulmonary Fibrosis (IPF) includes oxygen therapy, pulmonary rehab, and supportive care. Pharmacological options like ESBRIET (Pirfenidone) and OFEV (nintedanib) aim to slow the disease but have a limited impact on mortality.

As the need for effective IPF treatments continues to grow, several pharmaceutical companies have seized the opportunity to present their latest research at the ERS Congress. These companies are actively working on both early and late-stage clinical products related to IPF. Here are some noteworthy highlights:

Pliant Therapeutics is advancing Bexotegrast (PLN-74809), a Phase II drug designed as a dual selective inhibitor of αvß6 and αvß1 integrins. At the ERS International Conference 2023, the company presented an oral and late-breaking poster titled, “Safety, tolerability and antifibrotic activity of bexotegrast: Phase 2a INTEGRIS-IPF Study (NCT04396756)” involves the safety, tolerability, and efficacy results of bexotegrast. The trial with IPF patients employed a randomized and placebo-controlled design, testing Bexotegrast at varying doses, including 40 mg, 80 mg, 160 mg, and 320 mg. Remarkably, the 320 mg dose exhibited statistically significant improvements in Forced Vital Capacity (FVC) at different time points and a strong treatment effect on FVC percentage predicted (FVCpp). With positive safety and tolerability outcomes, this drug has progressed to the BEACON Phase IIb trial.

Vicore Pharma presented two posters on its lead program, C21, under development for treating idiopathic pulmonary fibrosis (IPF), at the European Respiratory Society (ERS) International Congress 2023 in Milan, Italy. C21 is an angiotensin II type 2 receptor agonist (ATRAG), and it can potentially improve lung function, especially in less severe IPF cases. Posters titled “Characterization of high responders in the phase 2a IPF AIR trial of C21 using baseline quantitative CT image analysis” and “An open-label, pharmacodynamic trial investigating vascular effects of the angiotensin II type 2 receptor agonist (ATRAG) C21” showcases the transformative potential of ATRAGs for IPF patients, offering a mechanistic perspective and exciting results from the Phase IIa AIR study. The study demonstrated lower airway volumes in high responders at the 24-week endpoint, and there were also trends indicating lower airway, vessel, and fibrosis volumes at the 36-week endpoint. Although these differences were not statistically significant, the findings suggest that C21 may benefit those with less severe IPF. Vicore Pharma is preparing for the Phase IIb ASPIRE study, and C21 has received orphan drug designation for IPF in Europe and the United States. Its unique mechanism involves targeting fibrosis by activating the protective arm of the renin–angiotensin system to promote alveolar repair.

Avalyn Pharma is also making strides in IPF treatment, focusing on inhaled pirfenidone, or AP01. They presented data from their Phase Ib ATLAS study in a late-breaking poster at the European Respiratory Society’s International Congress on September 10 and 11. The study’s findings shed light on the positive impact of AP01 on the forced vital capacity (FVC) of adults with IPF, comparing it to historical controls. What sets AP01 apart is its delivery method, as it is administered via a specialized nebulizer, maximizing its lung-specific effects while minimizing systemic exposure. In the Phase Ib ATLAS study involving 91 IPF patients, the 100 mg BID dose of AP01 demonstrated substantial lung function stabilization over 48 weeks. Importantly, this treatment exhibited good tolerability and resulted in fewer systemic side effects than oral pirfenidone, with mild cough being the most common side effect. An expanded access study is underway, and the open-label ATLAS study has shown promise as an innovative approach to IPF treatment, with some patients continuing their treatment for over 3 years.

Alentis Therapeutics, a clinical-stage biotech company, delivered an oral presentation titled “Claudin-1 is a potential airway-centric therapeutic target for pulmonary fibrosis” at the ERS International Congress in Milan, Italy, on September 11. This presentation explores the role of Claudin-1 (CLDN1), a transmembrane protein involved in tight junctions, in idiopathic pulmonary fibrosis (IPF). CLDN1 is a transmembrane protein involved in tight junctions and has been identified as a potential therapeutic target for IPF. Alentis Therapeutics is developing ALE.F02, a monoclonal antibody highly specific for exposed CLDN1, with applications in treating liver and kidney fibrosis. Research findings indicate that CLDN1 levels increase as IPF progresses, particularly in bronchiolar and alveolar epithelia and around fibrotic areas in patient tissues. Treating IPF precision-cut lung slices (PCLS) with ALE.F02 has shown promising results, reducing fibrosis and disease biomarkers such as MMP7, TIMP1, Col1a1, and MCP-1.

According to the DelveInsight report titled “Idiopathic Pulmonary Fibrosis – Market Insight, Epidemiology, and Market Forecast – 2032,” the estimated market size for IPF in the United States was USD 2.5 billion in 2022. This figure is anticipated to undergo significant changes following the approval of the emerging drugs. These emerging drugs are expected to challenge the market share of existing approved medications while also competing with generic alternatives. By 2032, the IPF market is projected to expand to approximately USD 5.4 billion in the 7MM, considering the introduction of several other drugs and the aforementioned key players drugs.