Late-stage pipeline for idiopathic pulmonary fibrosis is small but growing

diopathic 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.