Background/Purpose: It has been proved in several studies with a small number of patients that Rituximab (RTX) can prevent worsening of interstitial lung disease (ILD) and improve skin fibrosis in patients with Systemic Sclerosis (SSc). Recently, a multicentre case-control study of the EUSTAR cohort has confirmed these favorable results (Jordan S, et al. Ann Rheum Dis 2014 Jan 17 [Epub ahead of print]). Moreover, RTX may be effective on calcinosis. However, little is known about its long-term effect. Our objective was to assess the efficacy of RTX on skin involvement, ILD and calcinosis in series of patients with refractory SSc.

Methods: Patients with refractory SSc treated with RTX (off label use) were recruited from 3 hospitals. At baseline, the following data were collected: gender, age, type and duration of the SSc, clinical features, modified Rodnan skin score (mRSS), HRCT, pulmonary function tests (PFTs), walking test, sPAP (measured by echo), previous and present treatments, and indication and dosage of RTX. Throughout the follow up, clinical changes, as well as changes in HRCT and PFTs, were registered. We also recorded the changes in the dosage of corticosteroids, number of RTX cycles, duration of treatment, and withdrawals. The package SPSS 17.0 was used for descriptive statistics, and quantitative variables were compared using the t-test for paired samples.

Results: Thirty SSc patients treated with RTX were included in the analysis. The majority were women (86.7%), with a mean age of 54 years, and a mean of 9.4 years of evolution of the disease. Subtypes: DSSc 50%, LSSc 37%, and Overlap syndromes 13.3%. The baseline mean mRSS was 15. Clinical features: ILD 80% (NSIP 67%), calcinosis 37%, pulmonary hypertension 10%, joint disease 49%. The baseline mean FVC, DLCO and TLC values were 70%, 47% and 73%, respectively.

The indication for RTX was: ILD (73.4%), arthritis (36.6%), calcinosis (33.3%) and severe skin involvement (19.7%). The most used previous treatments were cyclophosphamide(50%) and mycophenolate (46.6%). RTX was always used in a RA dosage, mainly in monotherapy (46.7%), or in association with  mycophenolate (40%). When data were collected, patients had received a mean of 1.7 cycles (1-5) of RTX, with a dosing interval which ranged from 6 to 15 months, and a mean of 12.8 months (1-43) of treatment. The mean mRSS was significantly reduced at follow-up (17.2±10.9 vs. 14±9.8; p=0.012), without significant changes of the HRCT (76.9%) and/or PFTs in patients with ILD. The 40% of patients with calcinosis reported improvement. There was also a good response, in terms of TJC and SJC, in patients with arthritis (p=0.024 and 0.019, respectively). The dose of corticosteroids was significantly reduced (10.1±8.8 vs. 5.3±2.9 mg, p=0.003). Two patients with severe ILD died despite RTX, and there were 3 withdrawals for various reasons.

Conclusion: RTX is an effective long-term therapy in refractory SSc which can improve skin fibrosis, arthritis and calcinosis, and also prevent deterioration of ILD (stabilization of lung function).

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/long-term-efficacy-of-rituximab-in-systemic-sclerosis/