利妥昔单抗治疗狼疮和干燥综合征并难治性血小板减少患者有效

作者 Jiang B，et al.

翻译 刘蕊

摘要：目的：最近的研究表明利妥昔单抗（RTX）存在治疗自身免疫性疾病继发自身免疫性血小板减少（AITP）的潜力。在本研究中，我们回顾性评估RTX治疗继发于系统性红斑狼疮（SLE）和干燥综合征（SS）的难治性AITP患者的疗效和安全性。

方法：收集了2012年3月至2014年6月期间，在风湿病学临床Renji医院进行过一次或多次利妥昔单抗治疗的21名难治性AITP的SLE和/或SS患者进行。分析每次随访时记录的临床和实验室指标。结果：所有患者的中位年龄为37.05±3.15岁（范围，13-67岁; 20例女性和1例男性）。利妥昔单抗治疗前的中位AITP持续时间为5.46年。 21名患者曾经的免疫抑制治疗包括：如皮质类固醇（n = 19），环孢菌素（n = 9），霉酚酸酯（n = 2），甲氨蝶呤（n = 3），环磷酰胺（n=2）长春新碱（n=3）和羟氯喹（n = 15），7名患者同时接受静脉注射免疫球蛋白治疗。两名患者进行脾切除术无改善。在随访期间，17名患者（80.95％）重复应用利妥昔单抗治疗。利妥昔单抗治疗的总体反应率（包括完全反应，52.38％;部分反应，28.57％）为80.95％。 1个月后观察到血小板计数显着增加（中位数，32.24×10 / mL比66.53×10 / mL）（P <0 .05）。复发主要发生在前9个月，并且在第一次利妥昔单抗输注后保持反应的平均持续时间为10.27个月（范围，2-17个月）。在rtx治疗后，抗血小板抗体，特别是igg型显着降低（p <0.05）。15例（71.4％）患者没有观察到不良反应;然而，2例死于重症肺炎，另一例发生淋巴瘤。结论：利妥昔单抗是对常规免疫抑制治疗无效的自身免疫性血小板减少的系统性红斑狼疮和干燥综合征患者的另一种有效的治疗选择。对于大多数患者，利妥昔单抗是安全的并且有良好的耐受性。

附原文Abstract OBJECTIVE: Recent studies suggested a potential of rituximab (RTX) in treating autoimmune thrombocytopenia (AITP) secondary to autoimmune diseases. In this study, we retrospectively evaluated the efficacy and safety of RTX therapy in patients with refractory AITP secondary to systemic lupus erythematosus (SLE) and Sj?gren syndrome (SS). METHODS: Twenty-one SLE and/or SS patients with treatment-resistant AITP were treated once or repeatedly with RTX at the Rheumatology Clinic Renji Hospital, during the period March 2012 to June 2014. Clinical and laboratory variables recorded at every follow-up visit were analyzed. RESULTS: The median age of all patients was 37.05 ± 3.15 years (range, 13-67 years; 20 female and 1 male). The median AITP duration before RTX treatment was 5.46 years. Previous treatments of 21 patients included immunosuppressive agents such as corticosteroids (n = 19), cyclosporine (n = 9), mycophenolate mofetil (n = 2), methotrexate (n = 3), cyclophosphamide (n = 2), vincristine (n = 3), and hydroxychloroquine (n = 15), and 7 patients received concomitantly intravenous immunoglobulin therapy. Two patients had undergone splenectomy without improvement. Seventeen patients (80.95%) were treated repeatedly with RTX during the follow-up period. The overall response rate to RTX treatment (including complete response, 52.38%; partial response, 28.57%) was 80.95%. A significant increase (P < 0.05) of platelet counts was seen after 1 month (median, 32.24 × 10/ml vs 66.53 × 10/ml). relapses occurred mostly during the first 9 months, and maintaining duration of response was 10.27 months (range, 2-17 months) on average after the first rtx infusion. antiplatelet antibodies, especially igg isotype, decreased significantly (p < 0.05) after rtx treatment. no adverse effects were observed among 15 patients (71.4%); however, 2 cases died of severe pneumonia, and another developed lymphoma. conclusions: rituximab is an additional potent therapeutic treatment option for sle and ss patients with aitp refractory to conventional immunosuppressive treatments. for most patients, rtx was safe and well tolerated.

引自Jiang B1, Li T, Guo L,et al. Efficacy and Safety of Rituximab in Systemic Lupus Erythematosus and Sj?gren Syndrome Patients With Refractory Thrombocytopenia: A Retrospective Study of 21 Cases. J Clin Rheumatol. 2015 Aug;21(5):244-50. doi: 10.1097/RHU.0000000000000273.