干燥综合征神经系统病变的临床及病理分析

Clinical and pathological analysis of primary Sjogren's syndrom complicated with neurological system injuries

目的探讨原发性干燥综合征(Sjogren综合征;pSS)神经系统病变的临床及组织病理特征。方法对18例并发神经系统损伤的pSS患者的临床及病理资料进行回顾性分析。结果周围神经系统病变pSS(PNS-pSS)表现为对称性感觉运动性周围神经病、多发性单神经病、感觉性周围神经病、颅神经损伤。中枢神经系统病变pSS(CNS-pSS)表现为多发性硬化、缓慢进展的认知障碍和共济障碍、脑炎。头颅MRI显示脑白质多发异常信号、脑白质、脑干及小脑多发异常信号、脑脓肿。唇黏膜活检示部分腺体萎缩,腺泡及间质内可见淋巴、单核吞噬细胞浸润。腓肠神经活检既有典型血管炎改变也有非血管炎性改变。脑活检可见白质脱髓鞘及小静脉周围炎性细胞浸润以及典型血管炎改变。结论Sjogren综合征导致的神经系统损伤呈多样性,且常先于Sjogren综合征的诊断之前出现。了解其神经系统损伤的临床表现、影像学特点及组织病理改变对认识本病具有重要的临床意义。

Objective To explore the clinical and pathological characteristics of neurological system injuries in primary Sjogren＇s syndrome （pSS）. Method The clinical and pathological data of 18 patients with neurological system injuries were retrospectively reviewed. Result Clinically, pSS complicated with peripheral nervous system injuries （PNS-pSS） presented symmetrical sensory-motor peripheral neuropathy, mononeuropathy multiplx, pure sensory peripheral neuropathy and cranial nerves injuries; pSS complicated with central nervous system injuries （CNS-pSS） presented multiple sclerosis, slowly progressed congnition impairment, ataxia, and encephalitis. Brain MRI showed multiple abnormal signs in alba,brain stem and cerebella, as well as brain abscess. Pathologically, acinar gland atrophy and infiltration with lymphocytes and macrophages were demonstrated. Sural nerve biopsies disclosed typical vasculitis and non-vasculitic neuropathy. Brain biopsies disclosed demyelination in alba and lymphocytes infiltration around small veins, as well as typical vasculitis. Conclusion The spectrum of neurological complications of primary Sjogren＇s syndrome is wide and that neurological manifestations often precede Sjogren＇s syndrome diagnosis. In this regard, the clinical and pathological characteristics of neurological system injuries in primary Sjogren＇s syndrome are essential to be learned.