When it comes to lung diseases, asthma, chronic obstructive pulmonary disease (COPD), and cystic fibrosis might come to mind. One that may be less familiar is idiopathic pulmonary fibrosis (IPF), which is a rare respiratory disease characterized by damage to the lungs. The IPF market is underserved with just two licensed pharmaceutical treatments, Roche’s Esbriet/Pirespa (pirfenidone) and Boehringer Ingelheim’s Ofev (nintedanib), which are available globally. However, there is some activity in the late-stage pipeline that could translate into better treatment options for patients, says GlobalData, a leading data and analytics company.

According to GlobalData’s Pharmaceutical Intelligence Center, there are three Phase III assets that are currently in development for IPF, including United Therapeutic’s Tyvaso (treprostinil), Boehringer Ingelheim’s BI-1015550, and FibroGen’s pamrevlumab.

Tyvaso is an inhaled prostacyclin mimetic and is being investigated in two Phase III trials, TETON (NCT04708782) and an open-label extension (OLE) study (NCT04905693). BI-1015550 is an oral phosphodiesterase-4B (PDE4B) inhibitor currently in Phase III development (NCT05321069). Pamrevlumab, an intravenous agent that binds to the connective tissue growth factor (CTGT) protein, is being investigated in two ZEPHYRUS Phase III programs (NCT03955146 and NCT04419558).

Ramla Salad, Pharma Analyst at GlobalData, comments: “The development of these late-stage agents is exciting as they have mechanisms of action (MOAs) that are different from what is seen in currently available marketed therapies. Additionally, two of these late-stage agents also have routes of administration that are different from the oral options that are currently available in the market.”

As IPF is a rare disease, regulators have incentivized the development of therapies by issuing orphan drug, breakthrough, and fast track designations.

Salad continues: “Pamrevlumab is the only agent in the late-stage pipeline with a fast-track designation, meaning FibroGen can frequently meet with the FDA and discuss the drug development plan, which can expedite the review process and potentially lead to a faster drug approval.”

It is clear that there is a high unmet need in the IPF space, which translates into a huge market opportunity that United Therapeutics, Boehringer Ingelheim, and FibroGen are all hoping to capitalize on as patients are still waiting for better treatment options.

Salad concludes: “Boehringer Ingelheim’s expertise in the space with the already-approved Ofev could give that company a competitive edge as they have long-term data available, which could inform them of trends within the IPF treatment space.”