Family History of Pulmonary Fibrosis Predicts Worse Survival in Patients with Interstitial Lung Disease.

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A number of genetic markers linked to familial pulmonary fibrosis predict differential survival in interstitial lung disease (ILD) patients. While genetic testing is not routinely performed for ILD, family history is commonly obtained and may inform outcome risk.Does survival vary between patients with and without self-reported familial pulmonary fibrosis?Family history was acquired systematically for consecutive ILD patients who consented to clinical registry enrollment at UTSW and UC-Davis. Patients were stratified by idiopathic pulmonary fibrosis (IPF) and non-IPF ILD diagnosis and sub-stratified by presence or absence of familial pulmonary fibrosis, defined as one or more additional affected family members. Transplant-free survival was compared using multi-level, mixed effects Cox proportional hazards regression.Of the 1,262 patients included, 534 (42%) had IPF and 728 (58%) had non-IPF ILD. Of those with non-IPF ILD, 18.5% had connective tissue disease, 15.6% chronic hypersensitivity pneumonitis, and 23.5% unclassifiable ILD. Familial pulmonary fibrosis was reported in 134 (25.1%) IPF patients and 90 (12.4%) non-IPF ILD patients. Those with familial IPF had an 80% increased risk of death or transplant compared to those with sporadic IPF (HR 1.8, 95% CI 1.37-2.37, p<0.001), while those with familial non-IPF ILD had a two-fold increased risk compared to their sporadic counterparts (HR 2.08, 95% CI 1.46-2.96; p <0.001). Outcome risk among those with familial non-IPF ILD was no different than those with sporadic IPF (HR 1.27; 95% CI 0.89-1.84; p=0.19).Patient-reported familial pulmonary fibrosis is predictive of reduced transplant-free survival in IPF and non-IPF ILD patients. Because survival among patients with familial non-IPF ILD approximates that of sporadic IPF, early intervention should be considered for such patients. Until clinical genetic testing is widely available and provides actionable results, family history should be ascertained and considered in risk stratification.


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