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Educational Articles
Lung function course of patients with progressive pulmonary fibrosis after initiation of anti-fibrotic treatment: real-world data from the Dutch national registry
May 2025
Authors: Mark G. J. P. Platenburg, Gizal Nakshbandi, Catharina C. Moor, Aernoud A. van Batenburg, Rémy L. M. Mostard, Mareye Voortman, Linda A.A. Moonen, Nicolle Hekelaar, Maria J. Overbeek, Brigitte A.H.A. Bogaarts, Henk Kramer, Emiel. R. Marges, Bart B. Boerrigter, Paul Bresser, Eveline L. Schakenraad, Jan van der Maten, Niels C.A. van der Sloot, Stefan Walen, Pedro Miranda Afonso, Marlies S. Wijsenbeek, Jan C. Grutters
Comment by Mark Lavercombe: The approval of anti-fibrotic agents for the treatment of idiopathic pulmonary fibrosis (IPF) has dramatically changed the landscape for clinicians treating interstitial lung diseases (ILD), and other forms of progressive fibrotic ILD – termed progressive pulmonary fibrosis (PPF) – are now also recommended for consideration of anti-fibrotic therapy. In this paper, data from 538 patients with PPF who were treated with either nintedanib (88.7%) or pirfenidone (11.3%) were evaluated for change in lung function before and after commencement of therapy. The mean annualised decline in FVC in PPF before treatment with anti-fibrotics was 412ml, which reduced to 18ml after treatment. Similar results were also seen in patients with IPF. Despite the reduction in overall rate of decline, a proportion of patients progressed despite therapy – factors contributing to this need to be elucidated in future research.
Medical Education Editor for Respirology
Dr Mark Lavercombe was appointed in this specialist editor role in 2021 to further expand and strengthen Respirology’s contribution to medical education.
Located in Melbourne, Australia, he works as a clinician in respiratory health and sleep disorders at Western Health and is affiliated with The University of Melbourne as a distinguished expert in medical education.
He became a member of our Editorial Board in 2016 with the responsibility of selecting new Respirology publications with educational value, which has grown into a curated article selection featured on this page, as a benefit to APSR members.
Dr Lavercombe also authors a column in Respirology on his most recent choices and their specific value to continued medical education for our readers around the world.
