Contemporary Fibrotic Interstitial Lung Disease Outcomes: A Multicentre National Cohort Study.

Authors

Tyler Pitre, Christopher J Ryerson, Shaun Ong, Kerri A Johannson, Deborah Assayag, Veronica Marcoux, Martin Kolb, Helene Manganas, Daniel-Costin Marinescu, Jolene H Fisher

Published in

Respirology (Carlton, Vic.). Sep 07, 2026. Epub Sep 07, 2026.

Abstract

This study determined contemporary transplant-free survival in fibrotic interstitial lung disease (ILD), identified baseline factors associated with death or transplant, and assessed performance of the ILD-GAP (Gender, Age, Physiology) index in a contemporary cohort.
Patients with idiopathic pulmonary fibrosis (IPF), fibrotic hypersensitivity pneumonitis (fHP), connective tissue disease-associated ILD (CTD-ILD), or unclassifiable ILD (uILD) prospectively enrolled in the Canadian Registry for Pulmonary Fibrosis were included. Transplant-free survival at 1, 3, and 5 years was estimated overall and by subtype using enrollment as time zero. Cox proportional hazards models evaluated associations between patient characteristics and time to death or transplant. ILD-GAP scores were calculated at 1, 2, and 3 years, and discriminability was assessed with Harrell's C statistic.
Among 4236 patients with fibrotic ILD enrolled in the Canadian Registry for Pulmonary Fibrosis (CARE-PF), 31% had IPF, 40% CTD-ILD, 9% fHP, and 20% uILD. Overall transplant-free survival was 91% at 1 year, 72% at 3 years, and 57% at 5 years. Survival was lowest in IPF (87%, 61%, 39%) and highest in CTD-ILD (95%, 81%, 70%). Compared with IPF, CTD-ILD was associated with better 5-year survival after adjustment (hazard ratio 0.64 [0.55-0.76]). ILD-GAP showed moderate discrimination at 1 (C = 0.75), 2 (C = 0.74), and 3 years (C = 0.73).
In this registry cohort anchored at enrollment, CTD-ILD had superior transplant-free survival versus IPF, and ILD-GAP provided moderate prognostic accuracy.

PMID:
42706029
Bibliographic data and abstract were imported from PubMed on 08 Sep 2026.

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