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目的 探讨干燥综合征患者合并肌肉及周围神经损害患者的临床及其肌肉和周围神经病理改变。方法 对 7例患者的临床表现、实验室检查进行系统描述 ,对肌肉及神经组织进行组织学及组织化学染色 ,并行电子显微镜检查。结果 2例神经组织活检可见有髓神经纤维严重脱失 ,有髓神经纤维数目严重减少 ,轻度轴索变性。 5例肌肉活检可见肌纤维萎缩、坏死 ,血管周围有单核样细胞浸润 ,肌内衣有微血管炎的病理改变。结论 干燥综合征患者的肌肉或周围神经并发症在疾病的早期即可以出现 ,肌肉或神经活检可证实有无损害及其严重程度 ,早期诊断及早期治疗对改善干燥综合征合并神经系统损害患者的预后是很有必要的。
Objective To investigate the clinical and muscular and peripheral neuropathological changes in patients with Sjogren’s syndrome complicated by muscle and peripheral nerve lesions. Methods The clinical manifestations and laboratory tests of 7 patients were systematically described. Histological and histochemical staining of muscle and nerve tissue was performed and electron microscopy was performed. Results Two cases of nerve tissue biopsy showed severe demyelination of myelinated nerve fibers, severe reduction of myelinated nerve fibers and mild axonal degeneration. 5 cases of muscle biopsy showed muscle fiber atrophy, necrosis, monocytoid cell infiltration around the blood vessels, intramuscular microvascular inflammation pathological changes. Conclusions Muscle or peripheral neuropathy in patients with Sjögren’s syndrome can occur early in the disease. Muscular or neural biopsy can be used to confirm the presence or absence of a lesion and its severity. Early diagnosis and early treatment are important for improving the outcome of patients with Sjogren’s syndrome and neurological compromise Prognosis is necessary.