Department of Rheumatology, South Campus, Ren Ji Hospital, School of Medicine, Shanghai Jiao Tong University, Jiang Yue Road 2000, Shanghai, 201112, China.
Clin Rheumatol. 2018 Jan;37(1):213-218. doi: 10.1007/s10067-017-3793-4. Epub 2017 Aug 30.
Thrombotic microangiopathy (TMA) includes a series of life-threatening disorders. Systemic lupus erythematosus (SLE) is one of the most common acquired causes. To identify predictors of prognosis in patients with TMA secondary to SLE, we conducted a single-center historical study. From January 2013 to June 2016, of 2182 SLE hospitalized patients in the Ren Ji Hospital, a total of 21 consecutive patients with TMA secondary to SLE were identified. The 90-day short-term mortality was 33.3%. The kidney involvement (66.7%) was associated with poor prognosis, while the administration of rituximab (n = 13) was an independent protective factor according to logistic regression analysis. Compared to conventional treatment, i.e., plasma exchange, high-dose glucocorticoids, and intravenous immunoglobulin, the overall survival is significantly higher among patients receiving rituximab add-on (92.2 vs 33.3%, p = 0.0173); however, five out of seven patients with renal involvement in the rituximab group were eventually hemodialysis dependent. Our data indicated that add-on rituximab in the background of conventional therapy may improve the overall but not the renal survival in SLE-TMA patients.
血栓性微血管病（TMA）包括一系列危及生命的疾病。系统性红斑狼疮（SLE）是最常见的获得性病因之一。为了确定继发于 SLE 的 TMA 患者预后的预测因素，我们进行了一项单中心历史研究。2013 年 1 月至 2016 年 6 月，在仁济医院住院的 2182 例 SLE 患者中，共确定了 21 例继发于 SLE 的 TMA 患者。90 天短期死亡率为 33.3%。肾脏受累（66.7%）与预后不良相关，而根据逻辑回归分析，利妥昔单抗（n=13）的给药是独立的保护因素。与常规治疗（即血浆置换、大剂量糖皮质激素和静脉注射免疫球蛋白）相比，接受利妥昔单抗联合治疗的患者总生存率显著更高（92.2%比 33.3%，p=0.0173）；然而，利妥昔单抗组中 7 例有肾脏受累的患者中有 5 例最终依赖血液透析。我们的数据表明，在常规治疗的基础上加用利妥昔单抗可能会提高 SLE-TMA 患者的总体生存率，但不能提高肾脏生存率。
