Lung Function Course of Patients With Pulmonary Fibrosis After Initiation of Anti‐Fibrotic Treatment: Real‐World Data From the Dutch National Registry
Year: 2025
Type: article
Study design: Observational Study
Abstract: BACKGROUND AND OBJECTIVE: Real-world data on lung function course of patients with progressive pulmonary fibrosis (PPF) treated with anti-fibrotic medication are limited. We evaluated forced vital capacity (FVC) decline in patients with PPF and idiopathic pulmonary fibrosis (IPF) who started anti-fibrotic treatment. METHODS: This was a nationwide multi-centre registry study in 16 hospitals throughout the Netherlands. Patients treated with anti-fibrotic medication, with at least two in-hospital pulmonary function tests before and after the initiation of anti-fibrotic treatment, were included. Linear mixed-effects modelling was used to analyse lung function trajectories 1 year before and after the start of anti-fibrotic treatment. RESULTS: Data from 538 patients (n = 142 with PPF, n = 396 with IPF) were analysed. In PPF, the mean annualised FVC decline was 412 mL (95% confidence interval [CI]: 308-517 mL) before the initiation of anti-fibrotic treatment, and 18 mL (95% CI: 9-124 mL) in the first year after. The corresponding declines for IPF were 158 mL (95% CI: 78-239 mL) and 38 mL (95% CI: 24-101 mL). In both groups, treatment significantly slowed down FVC decline, although the change was larger in the PPF group (p = 0.0006). In the first year after treatment initiation, 23.9% of patients with PPF and 28.0% with IPF had disease progression. CONCLUSION: The FVC decline significantly slowed after the initiation of treatment for both IPF and PPF. Nevertheless, a significant proportion of patients exhibited disease progression, despite the start of anti-fibrotic treatment. Early identification of these patients is crucial for treatment adaptations and inclusion in clinical trials.
Source: Respirology
DOI: 10.1111/resp.70030
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
Institutions: St. Antonius Ziekenhuis, Erasmus MC, Maastricht University Medical Centre, Zuyderland Medisch Centrum, University Medical Center Utrecht, Rijnstate Hospital, Medisch Spectrum Twente, Medisch Centrum Haaglanden, Stichting Haaglanden Medisch Centrum, VieCuri Medisch Centrum, Martini Ziekenhuis, Leiden University, Leiden University Medical Center, Amsterdam University Medical Centers, Amsterdam UMC Location Vrije Universiteit Amsterdam, OLVG, Catharina Ziekenhuis, Medisch Centrum Leeuwarden, Jeroen Bosch Ziekenhuis, Isala
Language: English
Subfield: Pulmonary and Respiratory Medicine
Field: Medicine
Domain: Health Sciences
Open Access status: hybrid
Estimated APC paid: $4,330
Funder: Boehringer Ingelheim
OpenAlex ID: W4408759581