Idiopathic pulmonary fibrosis (IPF) is the most common subtype of idiopathic interstitial pneumonias (IIPs), which belong to a group of rare diseases termed interstitial lung diseases (ILDs). ILDs are characterised by damage to the lung parenchyma (the functional part of the lung that includes the alveolar tissue, bronchioles, bronchi, blood vessels, and interstitium) that occurs as a consequence of aberrant inflammation and fibrosis, thickening, and scarring of the connective tissue. The IPF market is underserved with just two licensed pharmaceutical treatments that are available globally, Roche’s Esbriet/Pirespa (pirfenidone) and Boehringer Ingelheim’s Ofev (nintedanib), which were both approved by the FDA in 2014. Esbriet, a dual anti-fibrotic and anti-inflammatory agent, was launched in Europe in 2012 and in Japan in 2008. Ofev—a small molecule tyrosine kinase inhibitor—gained approvals in only Europe and Japan, in 2015. These two approved therapies merely slow the progression of fibrosis but do not reverse it.

Despite the small number of approved therapies for IPF, there is some activity in the late-stage pipeline landscape with pipeline assets, 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). These studies were initiated based on positive data collected from the INCREASE study (NCT02630316), which showed improvements in lung function and reduced exacerbations.

Both the FDA and European Medicines Agency (EMA) granted treprostinil an orphan designation in 2020 to treat IPF, which boosted the profile of the agent and thus incentivises further development. BI-1015550 is an oral phosphodiesterase-4B (PDE4B) inhibitor currently in Phase III development (NCT05321069); it acts by preferential inhibition of the PDE4B subtype and is associated with anti-inflammatory and anti-fibrotic properties. The FDA granted BI-1015550 a breakthrough therapy designation and an orphan drug designation in February and August 2022, respectively. The agent has first-in-class potential as there are no PDE4B inhibitors approved for IPF, thus creating a big market opportunity for BI to monopolise.

Pamrevlumab is an intravenous agent that binds to the connective tissue growth factor (CTGT) protein; it is being investigated in two ZEPHYRUSA Phase III programs (NCT03955146 and NCT04419558). This development was prompted by the Phase II PRAISE trial (NCT01890265), which was completed in 2017; the results of the trial were published in the Lancet (Richeldi, L. et al. (2020), The Lancet. Respiratory Medicine, 8(1), pp. 25–33) and indicated that the proportion of patients with disease progression was lower in the pamrevlumab group versus placebo and that the agent was well-tolerated. The FDA awarded the agent an orphan drug designation and fast track designation in 2012 and 2018, respectively. 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 faster drug approval.

Although there are only two approved therapies currently for IPF, it is difficult to compare them directly to the aforementioned late-stage pipeline agents as they all have different routes of administration. 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. Moreover, 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 capitalise on as patients are still waiting for better treatment options.

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