What does this research mean for the field?

Among patients with fibrotic interstitial lung disease, connective tissue disease-associated ILD has superior transplant-free survival compared to idiopathic pulmonary fibrosis, and the ILD-GAP index provides moderate prognostic accuracy. Novelty: ClaimNovelty.INCREMENTAL. Consensus alignment: ConsensusAlignment.NEUTRAL.

What question did this study set out to answer?

To determine contemporary transplant-free survival across fibrotic interstitial lung disease subtypes, identify baseline predictors of mortality or lung transplantation, and evaluate the prognostic accuracy of the ILD-GAP index.

September 8, 2026Respirology

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

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Authors

TPTyler Pitre CRChristopher J. Ryerson SOShaun Ong

Discussion

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Overview

Multicenter cohort study reveals varied transplant-free survival across fibrotic interstitial lung disease subtypes, indicating moderate ILD-GAP prognostic accuracy.

Key Points

To determine contemporary transplant-free survival across fibrotic interstitial lung disease subtypes, identify baseline predictors of mortality or lung transplantation, and evaluate the prognostic accuracy of the ILD-GAP index.
Prospective multicenter cohort study analyzing 4,236 patients with fibrotic interstitial lung disease (IPF, fHP, CTD-ILD, or uILD) enrolled in the Canadian Registry for Pulmonary Fibrosis (CARE-PF).
Transplant-free survival was calculated at 1, 3, and 5 years using registry enrollment as baseline.
Multivariable Cox proportional hazards models identified predictors of death or transplantation, and Harrell's C statistic assessed the discriminative performance of the ILD-GAP index at 1, 2, and 3 years.
Among 4,236 patients (31% IPF, 40% CTD-ILD, 9% fHP, 20% uILD), overall transplant-free survival was 91% at 1 year, 72% at 3 years, and 57% at 5 years.
Five-year transplant-free survival was lowest for IPF (39%; 87% at 1 year, 61% at 3 years) and highest for CTD-ILD (70%; 95% at 1 year, 81% at 3 years), with CTD-ILD showing significantly lower risk versus IPF (adjusted HR 0.64, 95% CI 0.55–0.76).
The ILD-GAP index demonstrated moderate discrimination for mortality or transplantation at 1 year (C = 0.75), 2 years (C = 0.74), and 3 years (C = 0.73).

Cite This Study

Pitre et al. (2026) studied this question.

synapsesocial.com/papers/6a9fd79b58e84d0ff5b465bc https://doi.org/10.1002/resp.70305

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September 8, 2026Respirology

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

View Full Paper

Ask AI

Bookmark

Authors

TPTyler Pitre CRChristopher J. Ryerson SOShaun Ong

Discussion

Member takes

Overview

Multicenter cohort study reveals varied transplant-free survival across fibrotic interstitial lung disease subtypes, indicating moderate ILD-GAP prognostic accuracy.

Key Points

To determine contemporary transplant-free survival across fibrotic interstitial lung disease subtypes, identify baseline predictors of mortality or lung transplantation, and evaluate the prognostic accuracy of the ILD-GAP index.
Prospective multicenter cohort study analyzing 4,236 patients with fibrotic interstitial lung disease (IPF, fHP, CTD-ILD, or uILD) enrolled in the Canadian Registry for Pulmonary Fibrosis (CARE-PF).
Transplant-free survival was calculated at 1, 3, and 5 years using registry enrollment as baseline.
Multivariable Cox proportional hazards models identified predictors of death or transplantation, and Harrell's C statistic assessed the discriminative performance of the ILD-GAP index at 1, 2, and 3 years.
Among 4,236 patients (31% IPF, 40% CTD-ILD, 9% fHP, 20% uILD), overall transplant-free survival was 91% at 1 year, 72% at 3 years, and 57% at 5 years.
Five-year transplant-free survival was lowest for IPF (39%; 87% at 1 year, 61% at 3 years) and highest for CTD-ILD (70%; 95% at 1 year, 81% at 3 years), with CTD-ILD showing significantly lower risk versus IPF (adjusted HR 0.64, 95% CI 0.55–0.76).
The ILD-GAP index demonstrated moderate discrimination for mortality or transplantation at 1 year (C = 0.75), 2 years (C = 0.74), and 3 years (C = 0.73).

Cite This Study

Pitre et al. (2026) studied this question.

synapsesocial.com/papers/6a9fd79b58e84d0ff5b465bc https://doi.org/10.1002/resp.70305

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