TY - JOUR T1 - Long term outcomes of immunomodulatory drugs in SSc-ILD - data from the German SSc-network JF - European Respiratory Journal JO - Eur Respir J DO - 10.1183/13993003.congress-2019.PA5185 VL - 54 IS - suppl 63 SP - PA5185 AU - Michael Kreuter AU - Francesco Bonella AU - Norbert Blank AU - Elise Siegert AU - Jörg Henes AU - Margitta Worm AU - Cord Sunderkoetter AU - Marc Schmalzing AU - Alexander Kreuter AU - Claudia Guenther AU - Laura Susok AU - Gabriele Zeidler AU - Ina Koetter AU - Ulf Mueller-Ladner AU - Thomas Krieg AU - Aaron Juche AU - Tim Schmeiser AU - Gabriela Riemekasten AU - Elisabeth Aberer AU - Noemi Gaebelein-Wissing AU - Jörg H. W. Distler AU - Miklos Sárdy AU - Christiane Pfeiffer AU - Kathrin Kuhr AU - Hanns-Martin Lorenz AU - Pia Moinzadeh AU - Nicolas Hunzelmann Y1 - 2019/09/28 UR - http://erj.ersjournals.com/content/54/suppl_63/PA5185.abstract N2 - 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.FootnotesCite this article as: European Respiratory Journal 2019; 54: Suppl. 63, PA5185.This is an ERS International Congress abstract. No full-text version is available. Further material to accompany this abstract may be available at www.ers-education.org (ERS member access only). ER -