Jordan, S., Distler, J., Maurer, B., Allanore, Y., Van Laar, J., Distler, O., Fraticelli, P., Pomponio, G., Gabrielli, B., Riboldi, P., Ferraccioli, G., Valentini, G., Bombardieri, S., Malorni, W., Gerli, R., Lunardi, C., Faggioli, P., Corvetta, A., Gabrielli, A., Ghassemi, P., Baron, M., Blati, M., Kapoor, M., Della Rossa, A., Casigliani, S., Doveri, M., D'Ascanio, A., Tavoni, A., Bazzichi, L., Jordan, S., Distler, J., Maurer, B., Allanore, Y., Van Laar, J., Distler, O., Fraticelli, P., Pomponio, G., Gabrielli, B., Riboldi, P., Ferraccioli, G., Valentini, G., Bombardieri, S., Malorni, W., Gerli, R., Lunardi, C., Faggioli, P., Corvetta, A., Gabrielli, A., Ghassemi, P., Baron, M., Blati, M., Kapoor, M., Della Rossa, A., Casigliani, S., Doveri, M., D'Ascanio, A., Tavoni, A., and Bazzichi, L.
Objectives. Objective of this multicentre, observational study was to assess effects and safety of rituximab (RTX) using the European Scleroderma Trial and Research Group (EUSTAR) cohort. Methods. EUSTAR centres were asked to provide specific data about SSc patients treated with RTX. Primary endpoints were predefined for different disease manifestations and compared between baseline and follow-up. Normally distributed data, analysed by paired t-test, are shown as mean (s.d.), and non-parametric data, analysed by Wilcoxon matched paired signed-rank test, are shown as median and interquartile range. Results. Data on 72 SSc patients treated with RTX were captured from 27 EUSTAR centres (51 females/21 males, 52 diffuse/19 limited, age 51 (44-60) years, disease duration 6 (3-10) years, 47 anti-Scl-70 positive). The most frequent RTX application scheme was 1000 mg × 2 within 2 weeks (57/72 patients). Co-treatment with other immunosuppressive drugs was reported in 28 patients. The modified Rodnan skin score (mRSS) significantly decreased vs baseline at 7 (5-9) months follow-up (n = 47, 18.2 + 10.9 vs 14.5 + 9.9, P = 0.0002). This was true for both patients with later disease stages and also for patients with earlier, extended skin fibrosis (dSSc with mRSS >16 at baseline, n = 26; 26.5 + 6.8 vs 20.4 + 8.9, P < 0.0001, reduction by 29.9%). S-HAQ was unchanged, but the European SSc activity score improved after rituximab treatment [n = 10; 3.7 (2.6-6.4) vs 1.7 (0.9-2.5), P = 0.01]. RTX had no effects on lung fibrosis (FVC, DLCO, TLC, HRCT score) in n = 11 patients with evidence for SSc-ILD. In SSc-polyarthritis patients, the DAS-28 declined at 6 months follow-up without reaching statistical significance [n = 8; 4.8 (2.5-7.5) vs 3.7 (2.6-6.6); p = 0.3]. Of 8, 5patients were RF and/or anti-CCP antibody positive. Similar results were obtained for secondary outcome measures (tender and swollen joint count, VAS, CRP, ESR). Additional positive effects of RTX were seen on SSc-relate