Molecular endpoints for establishing target engagement by novel idiopathic pulmonary fibrosis therapies

Idiopathic pulmonary fibrosis (IPF) is a disease of progressive lung remodelling characterised by metaplastic epithelial cells, re-epithelialised air spaces (microscopic honeycombing), lymphoid aggregates, leukocyte accumulation (including macrophages, dendritic cells and mast cells), angiogenesis, lymphangiogenesis, fibroblast foci and excess matrix deposition [1, 2]. On average, untreated patients diagnosed with IPF succumb to the disease within 3 years of diagnosis [2]. This dismal prognosis has improved following approval of pirfenidone and nintedanib for disease management [3, 4]. These drugs cut disease progression in half, and likely improve survival. By showing that IPF is a treatable condition, approval of pirfenidone and nintedanib has accelerated interest in developing other medications that effectively treat IPF, with several nearing phase 3 of development [5].

Source: European Respiratory Journal - March 17, 2019 Category: Respiratory Medicine Authors: Wolters, P. J., Cottin, V. Tags: Interstitial and orphan lung disease Editorials Source Type: research