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

Abstract

RationaleThere is a paucity of observational data on antifibrotic therapy for idiopathic pulmonary fibrosis (IPF).ObjectiveWe aimed to assess the course of disease of IPF patients with and without antifibrotic therapy under real-life conditions.MethodsWe analysed data from a non-interventional, prospective cohort study of consecutively enrolled IPF patients from 20 ILD expert centres in Germany. Data quality was ensured by automated plausibility checks, on-site monitoring, and source data verification. Propensity scores were applied to account for known differences in baseline characteristics between patients with and without antifibrotic therapy.ResultsAmong the 588 patients suitable for analysis, the mean age was 69.8±9.1 years, and 81.0% were males. The mean duration of disease since diagnosis was 1.8±3.4 years. The mean % predicted value at baseline for forced vital capacity (FVC) and diffusion capacity (DLCO) were 68.6±18.8 and 37.8±18.5, respectively. During a mean follow-up of 1.2±0.7 years, 194 (33.0%) patients died. The one-year and two-year survival rates were 87% versus 46% and 62% versus 21%, respectively, for patients with versus without antifibrotic therapy. The risk of death was 37% lower in patients with antifibrotic therapy (HR=0.63, 95%CI: 0.45; 0.87; p=0.005). The results were robust (and remained statistically significant) on multivariable analysis. Overall decline of FVC and DLco was slow and did not differ significantly between patients with or without antifibrotic therapy.ConclusionsSurvival was significantly higher in IPF patients with antifibrotic therapy, but the course of lung function parameters was similar in patients with and without antifibrotic therapy. This suggests that in clinical practice premature mortality of IPF patients eventually occurs despite stable measurements for FVC and DLco.

Baseline characteristics and survival of patients of idiopathic pulmonary fibrosis: a longitudinal analysis of the Swedish IPF Registry

“…Thus, approximately 65% of the patients received anti-fibrotic treatment in our study, which is considerably more than in Germany (44%) [9], Finland (26%) [10], and Australia (23%) [6]. The present study shows that patients on anti-fibrotic therapy appear to survive longer than untreated patients, a result similar to what other registries have reported [6,8,20]. In order to avoid a potential bias in mortality analysis, Fig.…”

“…Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive fibrosing interstitial lung disease (ILD) of unknown cause [1][2][3].The disease is characterized by an aberrant accumulation of fibrotic tissue in the lung parenchyma, resulting in extensive alterations of lung structure and function and leads finally to respiratory failure and death [2,4,5]. Long-term observational studies in clinically diverse IPF populations from all over the world are increasing [1,[6][7][8][9][10][11][12][13][14][15] and provide us with important information on disease behaviour, management, and effectiveness of approved treatments.…”

Ongoing challenges in pulmonary fibrosis and insights from the nintedanib clinical programme

“…There is a lack of real-world data on post-exacerbation survival in patients treated with nintedanib or pirfenidone. However, data from the European IPF Registry (eurIPFreg), the INSIGHTS-IPF registry (Germany) and the Australian Idiopathic Pulmonary Fibrosis Registry suggest that patients with IPF who receive antifibrotic treatment have better overall survival than patients with IPF who do not receive nintedanib or pirfenidone [23,116,117]. Post-hoc analyses of the INPULSIS trials observed an association between nintedanib treatment and a numerical reduction in mortality following an acute exacerbation [45,47].…”

ERS International Congress, Madrid, 2019: highlights from the Interstitial Lung Diseases Assembly

“…The real-life EMPIRE registry showed that patients with usual interstitial pneumonia (UIP) and antineutrophil cytoplasmic antibody (ANCA)-positive ILD without signs of vasculitis had some distinct features but showed a similar survival curve to the ANCA-negative patients with IPF [9]. The INSIGHTS-IPF registry demonstrated that IPF patients, irrespective of antifibrotic therapy, remained relatively stable in terms of FVC and diffusing capacity of the lung for carbon monoxide (D LCO ) over a 2-year period, but the risk of death was 37% lower in patients on antifibrotic treatment [10]. The DIAMORFOSIS study, an international survey about the diagnostic and therapeutic management of patients with IPF and lung cancer, showed that there is wide variation in current practice, highlighting the need for a consensus statement on this topic.…”