Family history of pulmonary fibrosis predicts worse survival in patients with interstitial lung disease
Chest Jan 27, 2021
Cutting C, Bowman WS, Dao N, et al. - Given that differential survival in interstitial lung disease (ILD) patients is predicted by a number of genetic markers connected to familial pulmonary fibrosis, researchers herein investigated whether survival differs between patients with and without self-reported familial pulmonary fibrosis. From ILD patients who agreed to clinical registry enrollment at University of Texas-Southwestern and University of California at Davis, family history was obtained systematically. They used multi-level, mixed effects Cox proportional hazards regression to compare transplant-free survival. Findings revealed that in idiopathic pulmonary fibrosis (IPF) and non-IPF ILD patients, decreased transplant-free survival was predicted by patient-reported familial pulmonary fibrosis. Since survival in patients with familial non-IPF ILD approximates that of sporadic IPF, thus, considering early intervention for such patients was advised. Experts recommend determining family history as well as considering it in risk stratification, until clinical genetic testing is broadly accessible and affords actionable results.
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