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系统性红斑狼疮(SLE)的中枢神经系统表现的原因尚未确定。虽然有些作者提出脉管炎和抗神经元抗体的因素,但是,本文报道2例伴有脑部受累的致死性 SLE 患者与严重的凝血异常有关。例1为36岁女性。有左臂麻木、共济失调和发音困难史。12年前诊断为盘状红斑狼疮,33岁时发生关节痛,抗核因子阳性(1:200)并找到 LE 细胞。体检发现左侧轻偏瘫,右侧面肌无力。血小板计数少于10×10~9/L,纤维蛋白降解产物32μg/ml。周围血象提示微血管病性溶血,血清补体水平降低。脑脊液(CSF)β凝血酶球蛋白明显增高(128mg/ml)。血清抗核因子阳性(1:500),抗双链 DNA 抗体阳性(1:320)。治疗开始用富含血小板的血浆、地塞米松和氢化可的松静脉滴注,但患者迅速死亡。尸检发现脑部有由血小板和纤维蛋白血栓引起的弥漫性的微小梗塞,无脉管炎证据,免疫过氧化物酶检
The causes of central nervous system manifestations of systemic lupus erythematosus (SLE) have not been established. Although some authors have suggested vasculitis and anti-neuronal antibodies, two of these lethal SLE patients with brain involvement are reported here to be associated with severe coagulopathy. Example 1 is a 36-year-old woman. Left arm numbness, ataxia and dysarthria history. 12 years ago diagnosed as discoid lupus erythematosus, joint pain occurred 33 years old, anti-nuclear factor positive (1: 200) and find LE cells. Physical examination showed left hemiparesis, right facial muscle weakness. Platelet count less than 10 × 10 ~ 9 / L, fibrin degradation products 32μg / ml. Peripheral blood showed microvascular hemolysis, serum complement levels decreased. Cerebrospinal fluid (CSF) beta thrombin globulin was significantly higher (128mg / ml). Serum anti-nuclear factor positive (1: 500), anti-double-stranded DNA antibody positive (1: 320). Treatment began with intravenous drip of platelet-rich plasma, dexamethasone and hydrocortisone, but the patient died rapidly. An autopsy revealed diffuse small infarcts of the brain caused by platelets and fibrin thrombi, evidence of vasculitis, immunoperoxidase