Abstract：
BACKGROUND: Thrombotic thrombocytopenia purpura (TTP) associated with systemic lupus erythematous (SLE), features the appearance of inhibitory autoantibodies against ADAMTS13. Rituximab and belimumab (BEL), as both targeting B cells, seem to be an optimal therapy to induce clinical remission, prevent relapse of disease and contribute to glucocorticoid induction. However, the clinical outcome of SLE-TTP treated with sequential therapy between rituximab and BEL remain elusive.
METHODS: We reported the clinical outcomes of 4 patients diagnosed with SLE-TTP who were administrated a combination of corticosteroids, plasma exchange, and rituximab at stage of induction. BEL was utilized to rapidly reduce the reliance on these agents and prevent relapse of TTP at maintenance stage. Ultimately, 4 patients fully recovered with a SLE Disease Activity Index score of 0 and reached the goal at dose of prednisolone <7.5 mg/d without relapse.
CONCLUSIONS: Sequential treatment of rituximab and BEL could be an encouraging approach in treatment of SLE-TTP and rapid glucocorticoid reduction.
METHODS: We reported the clinical outcomes of 4 patients diagnosed with SLE-TTP who were administrated a combination of corticosteroids, plasma exchange, and rituximab at stage of induction. BEL was utilized to rapidly reduce the reliance on these agents and prevent relapse of TTP at maintenance stage. Ultimately, 4 patients fully recovered with a SLE Disease Activity Index score of 0 and reached the goal at dose of prednisolone <7.5 mg/d without relapse.
CONCLUSIONS: Sequential treatment of rituximab and BEL could be an encouraging approach in treatment of SLE-TTP and rapid glucocorticoid reduction.
摘要：
背景：与系统性红斑狼疮（SLE）相关的血栓性血小板减少性紫癜（TTP），具有针对ADAMTS13的抑制性自身抗体的出现。利妥昔单抗和贝利木单抗（BEL），都是针对B细胞的，似乎是诱导临床缓解的最佳疗法，预防疾病复发并有助于糖皮质激素诱导。然而,在利妥昔单抗和BEL之间序贯治疗的SLE-TTP的临床结局仍然难以捉摸.
方法：我们报告了4例诊断为SLE-TTP的患者的临床结果，这些患者接受了皮质类固醇的联合治疗，血浆置换,和利妥昔单抗在诱导阶段。BEL用于快速减少对这些药物的依赖并防止TTP在维持阶段复发。最终,4例患者完全康复，SLE疾病活动指数评分为0，在泼尼松龙剂量<7.5mg/d时达到目标，无复发。
结论：利妥昔单抗和BEL的序贯治疗可能是治疗SLE-TTP和快速减少糖皮质激素的一种令人鼓舞的方法。
方法：我们报告了4例诊断为SLE-TTP的患者的临床结果，这些患者接受了皮质类固醇的联合治疗，血浆置换,和利妥昔单抗在诱导阶段。BEL用于快速减少对这些药物的依赖并防止TTP在维持阶段复发。最终,4例患者完全康复，SLE疾病活动指数评分为0，在泼尼松龙剂量<7.5mg/d时达到目标，无复发。
结论：利妥昔单抗和BEL的序贯治疗可能是治疗SLE-TTP和快速减少糖皮质激素的一种令人鼓舞的方法。
