Transplant-free survival and prognostic factors were assessed in 4,236 adults with fibrotic ILD (IPF, CTD-ILD, fHP, uILD) prospectively enrolled in the Canadian Registry for Pulmonary Fibrosis (CARE-PF), with outcomes tracked at 1, 3, and 5 years from enrollment. Performance of the ILD-GAP index was also evaluated.
Overall transplant-free survival was 91% at 1 year, 72% at 3 years, and 57% at 5 years. IPF had the worst outcomes (87%, 61%, 39%), while CTD-ILD had the best (95%, 81%, 70%; adjusted HR vs. IPF: 0.64 [95% CI 0.55–0.76]). The ILD-GAP index showed moderate discrimination (Harrell's C = 0.75 at 1 year, 0.73 at 3 years).
Registry-based observational design limits causal inference. Enrollment was used as time zero, which may introduce selection bias toward patients already engaged in specialist care. The ILD-GAP index showed only moderate discrimination, suggesting prognostic gaps remain.
CTD-ILD carries meaningfully better long-term prognosis than IPF — roughly twice the 5-year transplant-free survival (70% vs. 39%) — reinforcing the importance of accurate ILD subtyping. The ILD-GAP index performs modestly in contemporary cohorts and should not be used alone for major clinical decisions.
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