SAT0346 THE EFFICACY AND SAFETY OF RITUXIMAB IN 27 CASES OF TREATMENT RESISTANT SYSTEMIC SCLEROSIS WITH SEVERE DISEASE ASSESSED BY ACTIVITY SCORES
Authors: Y. Yalçinkaya, B. Artim-Esen, S. Amikishiyev, N. Aliyeva, A. Gul, L. Ocal, M. Inanc
Journal: Annals of the Rheumatic Diseases (2020)

Background: Treatment options for systemic sclerosis (SSc) remain limited especially in severe skin, lung and musculoskeletal involvements. B-cell targeted therapy with anti-CD20 Rituximab (RTX), is widely available, reports from case series are encouraging as a a rescue therapy and might have an improving effect on organ involvement in SSc. Objectives: We aimed to retrospectively analyze the efficacy and safety of rituximab (RTX) courses in patients with severe systemic sclerosis who were refractory to standard immunosuppressive treatment. Methods: Twenty-seven SSc patients fulfilling ACR/EULAR classification criteria (2013) who received RTX treatment due to acive disease despite treatment with immunosuppresives were analyzed. Disease activity was evaluated by using EScSG/EUSTAR activity scores prior to and after RTX treatment. Disease severity was also assessed at baseline by Medsger’s index. Results: The demographics and characteristics of SSc patients were as follows: the median age of 50 (30-70), duration of Raynaud’s 10 (3-26) and non-Raynaud symptom 8.5 (3-18) years and summarised in table 1. RTX was given as a single cycle (2 infusions of 1000 mg) in 12 cases, 2 cyles in 5 cases, ≥3 cyles in 10 cases. DMARDs were prescribed in 19 (73%) patients (14 MMF, 5 MTX) concomitantly with RTX. The main RTX indications were skin and lung involvement (n=9), skin and arthritis (n=6), skin(n=5), lung (n=3), myositis (n=2), cardiac involvement (n=1) and digital vasculopathy (n=1). Medsger severity score was 7.39±3.091(3-13) at baseline. Table 1. Prevelance of Characteristics of SSc Patients n(%) female/male 25 /2 diffuse/limited cutaneous SSc 22 (81.5) / 5 (19,2) Clinical Characteristics synovitis / flexion contractures 12(44,4) / 10 (37,1) tendon friction rubs / myositis 7 (26,9) / 4 (15,4) renal crisis 1 (3,8) GI involvement 19 (69,2) lung involvement 16 (61,5) Serology ANA 23 (85,2) Anti-Scl70 / Anti-sentromer 16(61,5) / 1(3,8) Anti-Ro 6 (22,2) Previous Immunosuppressives CYC / MMF AZA / MTX Low dose steroids 19 (73,1) / 19 (73,1) 12 (46,2) / 16 (61,5) 27 (100) Disease activity and severity scores prior to and after RTX were summarised table 2. Disease activity scores were improved after RTX in patients who had a median follow-up period of 1 year (0,5-5 years). After RTX treatment, according to EscSG /EUSTAR scores 13 (%46.2) and 10 (%34.6) patients out of 26 were assessed as inactive. Table 2. Disease activity scores prior to and after RTX treatment Prior to RTX (n=26 ) After RTX (n=18 ) median Δ Change (n=18 ) EscSG activity score 4,89±1,82 (2,0-9,0) 2,37±1,10 (0,50-4,50) -2,00 (P&lt;0,001) EUSTAR activity score 4,57±2,68(1,0-10,0) 2,30±2,15 (0,0-7,25) -2,00 (P=0,002) There were severe infecions in 4 patients (Pneumonia in 2, infected digital ulcers in 2) and an episode of sinusitis in one during treatment period. One patient was deceased because of pneumonia and sepsis after the first cycle of RTX. Conclusion: In our SSc cohort, RTX treatment was used in severe patients, who had predominantly diffuse cutaneous disease with lung and joint involvements, severe vasculopathy and anti-Scl-70 positivity. Concomitant DMARDs were used in three-forth of the patients in addition to RTX cycles. Disease activity scores that assessed retrospectively were shown to be improved after RTX and 37-48% of the cases were assessed as inactive by using activity scores. Serious infections like pneumonia and infected digital ulcers were observed in 14,8% of cases during the follow-up. The addition of RTX treatment can be effective in selected patients with active disease despite immunosuppressive therapy. Disclosure of Interests: None declared