Work

Therapeutic advances in pulmonary fibrosis

Year: 2026

Type: review

Abstract: Pharmacological treatment of idiopathic pulmonary fibrosis (IPF) and progressive pulmonary fibrosis (PPF) is evolving from two established antifibrotic agents towards a broader pipeline of therapies. Current standard treatment with nintedanib and pirfenidone slows the decline in forced vital capacity, but does not halt disease progression, and long-term use may be limited by tolerability issues. This review summarises major profibrotic mechanisms relevant to drug development and discusses therapies in advanced or emerging clinical programmes, including the recently FDA-approved nerandomilast, positive results from the phase 3 trial of inhaled treprostinil, as well as promising mid-stage, pathway-directed agents targeting a broad range of profibrotic signalling pathways. We also discuss how negative or terminated programmes highlight recurring limitations related to efficacy, safety, trial duration, endpoint selection, and patient heterogeneity. Finally, we outline current shifts in trial design and outcome assessment, including increasing emphasis on patient-centred and composite endpoints alongside physiological measures, and discuss the rationale for add-on and combination approaches in future antifibrotic therapy for IPF and PPF.

Source: Therapeutic Advances in Respiratory Disease

DOI: 10.1177/17534666261489541

Authors: Ekrem Temizel, Alexandre Franco Amaral, Punchalee Kaenmuang, Niamh Logan, Nazia Chaudhuri