P.083 Efficacy and safety of nintedanib in systemic sclerosis-associated interstitial lung disease: one-year results from the remscle cohort

Introduction

Interstitial lung disease (ILD) is the leading cause of morbidity and mortality in Systemic Sclerosis (SSc). Nintedanib has been shown to reduce the rate of functional decline in clinical trials, but data from real-world practice remain limited. The aim of this study was to evaluate the effectiveness and safety of nintedanib in patients with SSc-ILD from the REMSCLE registry after one year of treatment

Material and Methods

Patients with SSc-ILD treated with nintedanib and with at least one year of follow-up were included. Baseline characteristics, concomitant treatments, and adverse events were collected. Effectiveness was assessed through changes in forced vital capacity (FVC), the proportion of patients meeting progression criteria (Goh 2017, INBUILD, ATS 2022 or absolute FVC decline >5%), and radiological ILD extent, comparing the year prior to treatment initiation with one year on treatment (paired t-test/Wilcoxon for continuous variables, McNemar for categorical variables).

Results

A total of 43 patients were recruited, 77% female. Cutaneous subtypes were lcSSc 65%, dcSSc 21%, and ssSSc 14%. 40% were ever-smokers. Anti-Scl70 antibodies were present in 42%, anti-centromere in 12% and RNA polymerase III in 10%. Raynaud’s phenomenon was present in 91% and digital ulcers in 44%. Gastrointestinal involvement was frequent (84%), musculoskeletal manifestations occurred in 37% and cardiac involvement was present in 81%. Among the 20 patients who underwent right heart catheterization, pulmonary hypertension was confirmed in 11 (55%). At baseline, all patients had ILD confirmed by HRCT, with a median FVC of 61.5%[45.9%–73.4%] and DLCO of 33.9%[23.0%–48.3%]. Dose reduction was required in 49%, and temporary discontinuation in 20.7%, mainly due to diarrhea or elevated transaminases. Permanent discontinuation occurred in 41.7%, primarily due to diarrhea (33.3%) or need for lung transplantation (33.3%). The most frequent adverse events were diarrhea (50%) and transaminase elevation (21.9%). Complete evaluation was available for 25 patients. During the year prior to treatment, FVC declined by 5.6%, 72% met at least one progression criterion, and radiological ILD extent was 33%. After 12 months of treatment, FVC improved significantly (+12.2%, p = 0.001), the proportion of patients meeting at least one progression criterion decreased to 20% (p < 0.01 for all criteria), and radiological extent remained stable, with a slight, non-significant increase (38%, p = 0.07).

Conclusions

In this multicenter cohort, nintedanib was associated with a significant reduction in ILD progression, improvement in lung function and stabilization of radiological extent. The safety profile was consistent with that described in clinical trials.