Clinical outcomes of patients with idiopathic pulmonary fibrosis in clinical practice: Follow-up results of the INSIGHTS-IPF registry

Data on outcomes of patients with idiopathic pulmonary fibrosis (IPF) under clinical practice conditions are limited. The INSIGHTS-IPF registry documents a large cohort of consecutive patients in Germany with particular focus on clinical management practices and outcomes. On 3 Feb 2015, 547 patients were included (mean age 69.0 ± 9.3 years, 77.9% male). The median six-minute walk distance was 320 meters (mean, 269 ± 198). The mean % of pred forced vital capacity was 72 ± 20, % pred DLCO was 35± 16. No drugs were administered in 19.9% of the patients, oral steroids in 22.9% (as monotherapy in 7.3%); N-acetylcysteine in 33.1% (monotherapy in 12.1% and triple therapy with azathioprine and steroids in 1.3%), pirfenidone in 43.3% (monotherapy in 26.0%), other drugs in 4.4%, and long-term O2 therapy in 33.3%. Mean follow-up was 0.95 ± 0.5 years corresponding to 388 person years. At 1 year, 56 patients (36.1%) had a FVC decline ≥ 5%, and 25 patients (16.1%) ≥ 10% at 1 year. Patients with higher FVC decline during follow-up had higher mortality rates. A total of 68 patients deceased corresponding to an annual death rate of 14.7%. Hospitalization were reported with a rate of 23.6% (due to IPF 13.1%, other 45.2%), and lung transplantation in 4.9%. Conclusion: The relatively high 1-year mortality can be attributed to the considerable proportion of patients with severe disease.