Long term outcomes of immunmodulatory drugs in SSc-ILD – data rom the German SSc network

Background: Data from prospective clinical trials support the use of immunomodulatory therapies (IT) for treatment of SSc-ILD. However, outcomes for SSc-ILD in respect to IT use in large real-life cohorts has only sparsely been reported.

Methods: The German Network for Systemic Scleroderma (DNSS), which includes SSc pts. prospectively, was analyzed for SSc-ILD. Patients were categorized in IT vs. no-IT users and outcome was assessed.

Results: SSc-ILD was reported in 1886 out of 4306 pts. 1109 used IT while 777 did not. Baseline characteristics at ILD diagnosis were similar with regards to gender, FVC (no IT 82% vs. IT 78%, p = 0.117) and use of PH drugs. Significant differences in no-IT vs. IT were found for age, time since SSc diagnosis (10 vs. 7 years p < 0.001), SSc subtype (p < 0.001, diffuse 45% vs. 53%), DLCO (62% vs. 58%, p < 0.001), mRSS (10.8 vs. 12.3, p = 0.005), specific organ involvements (e.g. esophagus p = 0.002, kidneys p = 0.002), auto-antibody profile, and steroid use (33% vs. 56%, p < 0.001). Disease progression (defined as either death or decline of FVC ≥ 10% or DLCO ≥ 15%) did not differ between groups (27.8% vs. 28.6%, p = 0.712). All-cause mortality was similar with 14.6% for no-IT and 13.9% for IT. Also decline of FVC ≥ 10% with 34% in the no-IT and 28% in the IT group (p = 0.24) and of DLCO ≥ 15% (35.5% vs. 30%, p = 0.088) were comparable. A multinomial logistic regression model revealed an increased risk for the development of SSc-ILD by factor 1.16 if IT was applied (p = 0.065).

Conclusions: In this large real-life cohort of SSc-patients, the use of immunomodulatory therapies had no significant impact on outcomes in SSc-ILD. Yet, differences in baseline characteristics have to be taken into account.