Press Release


Higher survival with antifibrotic therapy in IPF

Observational study discovers significant long term results

Illustration of lung fibrosis © Angelika Kramer

Does antifibrotic therapy for idiopathic pulmonary fibrosis (IPF) have an impact on mortality? As there is a paucity of observational data, the researchers around Prof. Jürgen Behr (Head of Clinical Pneumology, LMU Munich, ILBD/CPC) and Dr. Marion Frankenberger (ILBD/CPC, study coordinator) looked at the database of the INSIGHTS-IPF registry. Their aim: to assess the course of disease of IPF patients with and without antifibrotic therapy under real-life conditions. Thus, they analysed data from a non-interventional, prospective cohort study of consecutively enrolled IPF patients from 20 interstitial lung disease expert centres in Germany.

The results showed that 1-year and 2-year survival rates were significantly higher for patients with versus without antifibrotic therapy. The risk of death was 37% lower in patients with antifibrotic therapy. The results were robust (and remained statistically significant) on multivariable analysis and were published in European Respiratory Journal.

Another observation: The overall decline of FVC and DLCO was slow and did not differ significantly between patients with or without antifibrotic therapy. The conclusion of the scientists: in clinical practice, without antifibrotic therapy premature mortality of IPF patients eventually occurs despite clinically stable measurements for FVC and DLCO.

The INSIGHTS-IPF registry

The Investigating Significant Health Trends in Idiopathic Pulmonary Fibrosis registry is one of the largest IPF registries worldwide. It offers the opportunity to analyse the course of disease and long-term effectiveness of antifibrotic therapy in IPF. It is a nationwide, investigator-initiated observational study. The registry has been continuously enrolling consecutive incident and prevalent patients in routine clinical care across 20 pulmonary specialist centres in Germany since November 2012.

Link to the publication:
Survival and course of lung function in the presence or absence of antifibrotic treatment in patients with idiopathic pulmonary fibrosis: long-term results of the INSIGHTS-IPF registry.