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目的探讨利妥昔单抗(RTX)治疗血管性血友病因子裂解蛋白酶(ADAMTS-13)活性正常的系统性红斑狼疮(SLE)相关血栓性血小板减少性紫癜(TTP)的疗效。方法分析中国人民解放军海军总医院内分泌风湿免疫科先后应用RTX治疗3例ADAMTS-13活性正常的SLE相关TTP患者的临床特征,随访患者治疗后的转归,并总结相关文献。结果 3例SLE相关TTP患者均有不同程度的发热、血小板减少和微血管病性溶血性贫血,伴神经系统和肾损害,血乳酸脱氢酶(LDH)明显升高,外周血涂片可见破碎红细胞,而ADAMTS-13活性正常。2例患者行肾活检显示1例为狼疮肾炎Ⅳ+Ⅴ型伴血栓性微血管病,1例为狼疮肾炎Ⅱ型。3例患者在确诊SLE相关TTP前均接受甲泼尼龙冲击联合静脉注射免疫球蛋白和吗替麦考酚酯(MMF)治疗,但病情加重,确诊后其中2例患者联合血浆置换治疗病情仍无改善,给予RTX治疗;1例患者在确诊后直接联合RTX治疗。经上述治疗后3例患者病情均达到完全缓解,平均随访14个月呈持续缓解,其中1例在随访6个月时因重症肺炎死亡。回顾文献结合本文系列报道,共6例应用RTX治疗ADAMTS-13活性正常的SLE相关TTP,显示所有患者均在糖皮质激素联合血浆置换和(或)联合免疫抑制剂治疗失败后加用RTX,或在初始治疗的同时即加用RTX,病情得到缓解。结论 SLE患者出现发热、贫血、血小板减少及LDH升高可能提示SLE相关TTP,而外周血涂片和肾活检检查有助于确诊。ADAMTS-13活性正常的SLE相关TTP患者神经系统和肾损害发生率高,病情重,对血浆置换联合糖皮质激素和(或)免疫抑制剂的治疗反应差,而RTX对这类患者有效,可改善患者预后。
Objective To investigate the efficacy of rituximab (RTX) in the treatment of thrombocytopenic purpura (TTP) associated with systemic lupus erythematosus (SLE) with normal activity of von Willebrand factor cleaving protease (ADAMTS-13). Methods The clinical features of three cases of SLE-related TTP patients with normal ADAMTS-13 activity were analyzed by RTX in the Department of Endocrinology and Rheumatology of the Chinese PLA General Hospital. Follow-up patients were followed-up and the relevant literature was summarized. Results All 3 cases of SLE-related TTP patients had different degrees of fever, thrombocytopenia and microangiopathy hemolytic anemia, with nervous system and renal damage, blood lactate dehydrogenase (LDH) was significantly increased, peripheral blood smears showed broken red blood cells , While ADAMTS-13 activity is normal. Two patients underwent renal biopsy showed that 1 case of lupus nephritis Ⅳ + Ⅴ with thrombotic microangiopathy, and 1 case of lupus nephritis type Ⅱ. Three patients were treated with methylprednisolone in combination with intravenous immunoglobulin and mycophenolate mofetil (MMF) before the diagnosis of SLE-related TTP. However, the patients were exacerbated. Two of the patients were diagnosed with combined plasma replacement therapy Improved, given RTX treatment; 1 patient in the diagnosis of direct joint RTX treatment. All of the three patients achieved complete remission after the above treatment, with an average of 14 months follow-up was sustained remission, of which 1 case died of severe pneumonia at 6 months follow-up. In retrospective literature combining this series of reports, a total of 6 patients treated with RTX for SLE-related TTP with normal ADAMTS-13 activity showed that all patients were treated with RTX following a failure of glucocorticoid replacement therapy and / or combination immunosuppressive therapy At the same time initial treatment with RTX, the condition was alleviated. Conclusion Fever, anemia, thrombocytopenia and elevated LDH in patients with SLE may be associated with SLE-related TTP. Peripheral blood smears and renal biopsy may be helpful for diagnosis. STI-related TTP patients with active ADAMTS-13 have a high incidence of neurological and renal impairment, severe illness, poor response to plasma exchange with glucocorticoid and / or immunosuppressant, and RTX is effective in these patients Improve patient’s prognosis.