系统性红斑狼疮神经系统病变的临床及病理特征

目的探讨系统性红斑狼疮（SLE）神经系统病变的临床及病理特征。方法回顾性分析6例SLE神经系统病变患者的临床及病理资料。结果本组患者累及中枢神经系统4例,其中癫痫1例、脑梗死2例、白质脑病1例;多发性单神经病3例,腓肠神经活检示1例有典型血管炎改变,2例无血管炎改变,腓肠神经内可见有髓纤维丢失、轴索和髓鞘断裂呈块状深染及髓磷脂小球形成。肌活检1例示肌纤维轻度变性坏死。皮肤活检3例示胶原变性,小静脉周围可见炎性细胞浸润。结论SLE可导致神经系统广泛病变,故其临床表现和病理改变复杂多样。

Clinical and pathological characteristics of neurological lesions in patients with systemic lupus erythematosus

Objective To explore the clinical and pathological characteristics of neurological lesions in systemic lupus erythematosus (SLE).Methods The clinical and pathological data of 6 SLE patients with neurological lesions were analyzed retrospectively.Results Clinically, the central nervous system was involved in four patients with presentation of epilepsy in one case, cerebral infarction in two cases and leukoencephalopathy in one case. Mononeuropathy was involved in three cases. Pathologically, sural nerve biopsies disclosed typical vasculitis in one of three cases. There were loss of myelinated fibers, segmented and dark-stained axons and myelin, and myelin bead formation in the sural nerves. Muscular biopsy of one case showed mild degeneration and necrosis. Three skin biopsies demonstrated degeneration of fibrinogen, perivenous infiltration of inflammatory cells.Conclusions SLE may lead different damage of nervous system. Therefore, the changes of its clinical and pathological manifestations are complicated.