ABSTRACT
Background and Objective
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.
Methods
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.
Results
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).
Conclusion
In this registry cohort anchored at enrollment, CTD‐ILD had superior transplant‐free survival versus IPF, and ILD‐GAP provided moderate prognostic accuracy.
