Therapeutic advances in pulmonary fibrosis
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.
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BibTeX
@article{temizel2026therapeutic,
title = {{Therapeutic advances in pulmonary fibrosis}},
author = {Ekrem Temizel and Alexandre Franco Amaral and Punchalee Kaenmuang and Niamh Logan and Nazia Chaudhuri},
journal = {Therapeutic Advances in Respiratory Disease},
year = {2026},
month = oct,
doi = {10.1177/17534666261489541},
url = {https://doi.org/10.1177/17534666261489541},
note = {CC BY},
}
RIS
TY - JOUR
TI - Therapeutic advances in pulmonary fibrosis
AU - Ekrem Temizel
AU - Alexandre Franco Amaral
AU - Punchalee Kaenmuang
AU - Niamh Logan
AU - Nazia Chaudhuri
PY - 2026
DA - 2026/10/24
JO - Therapeutic Advances in Respiratory Disease
DO - 10.1177/17534666261489541
UR - https://doi.org/10.1177/17534666261489541
AB - 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.
SN - 1753-4666
SN - 1753-4658
ER -