Division of Hematology, Department of Medicine, Taipei Veterans General Hospital, Taipei, Taiwan, ROC.
School of Medicine, National Yang-Ming University, Taipei, Taiwan, ROC.
J Chin Med Assoc. 2020 Aug;83(8):743-750. doi: 10.1097/JCMA.0000000000000344.
Thrombotic microangiopathy (TMA) syndromes are potentially life-threatening complications and are defined as integrated syndromes of microangiopathic hemolytic anemia, thrombocytopenia, and organ injury. Systemic lupus erythematosus (SLE) is an autoimmune disease that can affect various organs, including the hematopoietic system. SLE can complicate with TMA and can be categorized into two distinct groups by chronological association: TMA occurring as the initial presentation and leading to a diagnosis of SLE concurrently (TMA-cSLE) or TMA developing in patients previously diagnosed as having SLE (TMA-pSLE). We examined the differences in clinical characteristics, treatment responses, and clinical outcomes between these groups.
We reviewed data of patients diagnosed as having TMA and SLE at Taipei Veterans General Hospital between 2002 and 2013. We included 29 patients: 8 and 21 in TMA-cSLE and TMA-pSLE groups, respectively. All underwent plasma exchange. Patients' demographic and clinical characteristics, disease activity, and treatment modality were summarized.
Overall survival (OS) from SLE or TMA diagnosis was poor for the TMA-cSLE group. Median OS from SLE diagnosis was 2.9 months in the TMA-cSLE group and 103.5 months in the TMA-pSLE group (p < 0.001). Median OS from TMA diagnosis was 2.9 months in the TMA-cSLE group and 10.7 months in the TMA-pSLE group (p = 0.58). Time to TMA remission after treatment appeared longer in the TMA-cSLE group (38.00 vs 10.76 days). Multivariate Cox analysis revealed TMA-cSLE and anti-RNP positivity were independent risk factors for mortality in SLE patients with TMA.
The occurrence of TMA with SLE is rare, and its vigorous course results in high mortality and morbidity rates. In patients without a history of autoimmune disease, early suspicion of TMA and working-up for SLE under this condition are vital. Early recognition of TMA-cSLE and prompt plasma exchange with upfront immunosuppressive therapies for TMA-cSLE patients or anti-RNP-positive patients may improve their prognosis.
血栓性微血管病（TMA）综合征是一种潜在危及生命的并发症，其特征为微血管性溶血性贫血、血小板减少和器官损伤的综合征。系统性红斑狼疮（SLE）是一种自身免疫性疾病，可影响包括造血系统在内的各种器官。SLE 可并发 TMA，并可根据时间关联分为两类：以 TMA 为初始表现并同时导致 SLE 诊断的 TMA 与 SLE（TMA-cSLE）或在先前诊断为 SLE 的患者中发生的 TMA（TMA-pSLE）。我们研究了这些组之间在临床特征、治疗反应和临床结局方面的差异。
我们回顾了 2002 年至 2013 年间在台北荣民总医院诊断为 TMA 和 SLE 的患者数据。共纳入 29 例患者：TMA-cSLE 组 8 例，TMA-pSLE 组 21 例，均接受血浆置换治疗。总结患者的人口统计学和临床特征、疾病活动度和治疗方式。
TMA-cSLE 组从 SLE 或 TMA 诊断开始的总生存（OS）较差。TMA-cSLE 组从 SLE 诊断开始的中位 OS 为 2.9 个月，TMA-pSLE 组为 103.5 个月（p＜0.001）。TMA-cSLE 组从 TMA 诊断开始的中位 OS 为 2.9 个月，TMA-pSLE 组为 10.7 个月（p=0.58）。TMA-cSLE 组 TMA 缓解后的治疗时间似乎更长（38.00 天 vs 10.76 天）。多变量 Cox 分析显示，TMA-cSLE 和抗 RNP 阳性是 TMA 合并 SLE 患者死亡的独立危险因素。
SLE 合并 TMA 罕见，但病程活跃导致高死亡率和发病率。对于无自身免疫性疾病病史的患者，早期怀疑 TMA，并在此情况下对 SLE 进行检查至关重要。早期识别 TMA-cSLE，并对 TMA-cSLE 患者或抗 RNP 阳性患者进行即刻血浆置换和强化免疫抑制治疗，可能改善其预后。
