Abstract

Introduction: In a randomized control trial (SENSCIS), nintedanib has been shown to significantly slow the decline in FVC (Forced Vital Capacity) in systemic sclerosis-associated interstitial lung disease (SSc-ILD). Objectives: Evaluation of the tolerance profile and efficacy of nintedanib in SSc-ILD patients in real life

Patients & Methods : Real-life data were obtained in an ambispective way with collection of clinical and functional data before and after nintedanib start in patients with SSc-ILD using the GFRS, SNFMI and Orphalung networks

Results : 116 patients were included in 14 French centers. Mean age of patients was 57±11 years. 59 (51%) had diffuse SSc, 63 (57%) had antitopo-1 antibodies. SSc-ILD was extensive (>20% of the lung) in 58% of cases, and CT pattern was a nonspecific interstitial pneumonia (NSIP) in 83% of cases. Mean FVC at nintedanib introduction was 2.01±0.44L (66±13%). Mean DLCO was 37 ±10%. At 6 month and 12 months, mean loss of FVC was respectively 12 ± 110 mL (n=75) and 7±102 mL (n=56) versus 182±208 mL in the year before treatment initiation. Adverse events were found in 48% and 32% patients at 6 and 12 months respectively with a majority of digestive side effects

Discussion & conclusion: SSc-ILD population in this real-life study of nintedanib has similar epidemiological characteristics to those of SENSCIS but baseline FVC and DLCO were lower. The results of the present study suggest that nintedanib limits functional decline in SSc-ILD at 6 and 12 months in real life with a tolerance profile similar to idiopathic pulmonary fibrosis. Additional data will be furnished at 18 and 24 months of follow up