Yang Fan, Tian Junwei, Peng Linyi, Zhang Li, Liu Jia, Tian Xinping, Zhang Wen, Li Mengtao, Zhao Yan, Zhang Fengchun, Zeng Xiaofeng, Wang Li, Leng Xiaomei
Department of Rheumatology and Clinical Immunology, Chinese Academy of Medical Sciences and Peking Union Medical College, National Clinical Research Center for Dermatologic and Immunologic Diseases, Ministry of Science and Technology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, China.
Department of Rheumatology and Nephrology, The First Hospital of Changsha, Hunan, China.
Front Med (Lausanne). 2021 Nov 8;8:772607. doi: 10.3389/fmed.2021.772607. eCollection 2021.
This study aims to describe clinical characteristics and outcome of thrombotic microangiopathy (TMA) in Chinese patients with systemic lupus erythematosus (SLE), and investigate the risk factors. We conducted a retrospective single-center cohort and enrolled patients of TMA associated with SLE between January 2015 and December 2018. Demographic characteristics, clinical features, laboratory profiles, therapeutic strategies, and outcomes were collected. The risk factors of TMA in patients with SLE for mortality using multivariate analysis were estimated. A total of 119 patients with a diagnosis of TMA were enrolled within the study period in our center, and SLE was found in 72 (60.5%) patients. The mean age was 29.2 ± 10.1 and 65 (92.3%) were women. Only 15 patients were found with definite causes, the other 57 cases remained with unclear reasons. Sixty-two patients got improved, while 10 patients died after treatment (mortality rate: 13.9%). Compared with the survival group, the deceased group had a higher prevalence of neuropsychiatric manifestations, infection with two or more sites, increased levels of C-reaction protein (CRP) and D-Dimer, and decreased platelet count. Multivariate analysis showed that the decrease of platelet count is the independent risk factor for in-hospital mortality for TMA in patients with SLE. The receiver operating characteristic (ROC) curve analysis displayed that a cutoff value of <18 × 10/L for platelet count could significantly increase the risk of death. Thrombotic microangiopathy often occurs in patients with active SLE with high mortality (13.9%), and thrombocytopenia, especially when the platelet count is lower than 18 × 10/L, is the risk factor for death.
本研究旨在描述中国系统性红斑狼疮（SLE）患者血栓性微血管病（TMA）的临床特征及转归，并探究危险因素。我们进行了一项回顾性单中心队列研究，纳入2015年1月至2018年12月期间与SLE相关的TMA患者。收集患者的人口统计学特征、临床特征、实验室检查结果、治疗策略及转归情况。采用多因素分析评估SLE患者发生TMA死亡的危险因素。在研究期间，本中心共纳入119例诊断为TMA的患者，其中72例（60.5%）患有SLE。患者平均年龄为29.2±10.1岁，女性65例（92.3%）。仅15例患者病因明确，其余57例病因不明。62例患者病情好转，10例患者治疗后死亡（死亡率：13.9%）。与存活组相比，死亡组神经精神症状、两个或以上部位感染的发生率更高，C反应蛋白（CRP）和D-二聚体水平升高，血小板计数降低。多因素分析显示，血小板计数降低是SLE患者TMA院内死亡的独立危险因素。受试者工作特征（ROC）曲线分析显示，血小板计数<18×10⁹/L的临界值可显著增加死亡风险。血栓性微血管病常发生于活动期SLE患者，死亡率较高（达13.9%），血小板减少，尤其是血小板计数低于18×10⁹/L时，是死亡的危险因素。
