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目的探讨原发性干燥综合征(Sjgren综合征)神经系统损伤的临床病理特征及其机制。方法8例患者均符合欧洲原发性Sjgren综合征的诊断标准,其中唇黏膜活检5例,腓肠神经活检5例,肱二头肌活检1例,全部标本均进行了组织和免疫病理学检查。结果对称性感觉运动性周围神经病2例,多发性单神经病2例,感觉性周围神经病1例,脑神经损伤2例,累及中枢神经2例。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,主要为CD8阳性的毒性和抑制性T细胞。腓肠神经活检示1例有典型血管炎改变,4例无血管炎性改变。可见有髓纤维丢失、轴索和髓鞘断裂,小静脉周围可见CD68阳性单核吞噬细胞及CD45RO阳性T细胞。肌活检示肌纤维有轻度变性坏死,间质内可见少量淋巴及吞噬细胞。结论Sjgren综合征导致的神经损伤呈多样性,且常先于Sjgren综合征的诊断之前出现,因此追述有无眼干、口干症状对诊断有重要价值。血管炎及非血管炎性免疫介导的炎性细胞浸润可能是Sjgren综合征神经损伤的重要机制。
Objective To investigate the clinicopathological features and mechanism of nervous system injury in primary Sjgren’s syndrome (Sjgren’s syndrome). Methods All 8 patients met the diagnostic criteria of European primary Sjgren syndrome. Among them, 5 were lip labial biopsy, 5 were sural nerve biopsy and 1 was biceps biopsy. All specimens were examined histologically and immunohistologically . Results Symmetry sensory motor peripheral neuropathy in 2 cases, multiple mononeuropathy in 2 cases, sensory peripheral neuropathy in 1 case, 2 cases of cranial nerve injury, involving the central nervous system in 2 cases. Lip mucosal biopsy showed that part of the gland atrophy, acinar and interstitial lymph nodes can be seen, mononuclear phagocyte infiltration, mainly CD8-positive toxic and inhibitory T cells. One case of sural nerve biopsy showed typical changes of vasculitis and 4 cases of vasculitis without inflammatory changes. Visible loss of medullary fibers, axonal and myelin rupture, can be seen around the small veins of CD68-positive mononuclear phagocytes and CD45RO-positive T cells. Muscle biopsy showed mild degeneration and necrosis of muscle fibers, interstitial lymphocytes and phagocytes can be seen in a small amount. Conclusion Sjgren’s syndrome caused a variety of nerve damage, and often preceded the diagnosis of Sjgren’s syndrome. Therefore, it is of great value in the diagnosis of dry eyes and dry mouth symptoms. Vasculitis and non-vascular inflammatory immune-mediated inflammatory cell infiltration may be an important mechanism of nerve injury in Sjgren’s syndrome.