Survival in familial idiopathic pulmonary fibrosis and fibrotic hypersensitivity pneumonitis is worse than its sporadic presentations.

<bold>Background:</bold> Familial pulmonary fibrosis (FPF) is defined as the presence of two or more family members with interstitial lung disease (ILD) of any etiology. Both idiopathic pulmonary fibrosis and fibrotic hypersensitivity pneumonitis can be familial. Few studies compare survival in different phenotypes of FPF. <bold>Aim:</bold> Compare the survival of the phenotypes of familial-idiopathic pulmonary fibrosis (F-IPF) and familial-fibrotic hypersensitivity pneumonitis (F-FHP) to their sporadic forms. <bold>Method:</bold> Retrospective study involving three outpatient ILD clinics. Variables related to survival were estimated by univariate and multivariate Cox regression. Variables that showed statistical differences (p<0.05) between the familial and non-familial groups were controlled before estimating the survival curves. Survival curves were estimated by the Kaplan-Meyer method and compared using Log-Rank. <bold>Results:</bold> 198 participants were included, 88 with IPF (36 F-IPF/52 sporadic IPF) and 110 with FHP (30 F-FHP/80 sporadic FHP). Age [(hazard ratio (HR), 1.03; 95% CI 1.01 to 1.06; p=0.01)], FVC% (HR, 0.96; 95% CI 0.94 to 0.98; p<0.01), SpO2% (HR, 0.86; 95% CI 0.76 to 0.97; p<0.02) and family history of pulmonary fibrosis (HR, 1.79; 95% CI 1.09 to 2.93; p=0.02) were associated to mortality. After adjusting for these four covariates, patients with F-IPF and F-FHP had lower survival than the ones of the counterpart sporadic forms (46 months versus 75 months, respectively, p<0.05). <bold>Conclusion:</bold> Familial presentations of IPF and FHP have worse survival than patients with the same ILD etiology but with non-familial presentation.