Exacerbations, hospitalisations and mortality in patients with idiopathic pulmonary fibrosis in clinical practice: INSIGHTS-IPF registry

The INSIGHTS-IPF registry documents a large cohort of consecutive patients with idiopathic pulmonary fibrosis (IPF) in Germany with particular focus on current clinical management practices and outcomes. In 820 patients, mean age was 69.7 ± 9.0 years, 79.9% were males. Mean duration of disease since diagnosis was 1.9 ± 3.2 years. The mean % of pred forced vital capacity at baseline was 72.5 ± 19.5, % predicted DLCO was 35.5 ± 16.5. During a mean follow-up of 1.59 ± 1.05 years accounting for 1100 person-years, 194 patients died (28.0%, annualized rate 13.2%). Death was related to IPF in 86 patients (annualized 4.1%), and a respiratory failure including acute exacerbations was recorded as cause of death for 49 (annualized 1.8%). 259 patients (37.4%, annualized 20.9%) were hospitalized at least once, and 75 (10.8%, annualized 2.9%) were hospitalized due to an acute exacerbation. Eighty patients died after hospitalisation for any cause (annualized 3.3%) after a mean of 6.4 ± 6.2 months after hospital discharge. Thirty-four of 75 died after hospitalisation due to an acute exacerbation. Thirty-three patients (4.8%, annualized 0.8%) underwent lung transplantation. Higher GAP stages at study entry were significantly associated with mortality overall (Stage I 7.5%, II 27.1%, III 47.1%; p The relatively high event rate during follow-up may be attributed to the considerable proportion of patients with severe disease.