Vrethem M, Lindvall B, Holmgren H, Henriksson K G, Lindström F, Ernerudh J
Department of Neurology, Linköping University Hospital, Sweden.
Acta Neurol Scand. 1990 Aug;82(2):126-31. doi: 10.1111/j.1600-0404.1990.tb01601.x.
Seventeen consecutive patients with primary Sjögren's syndrome (PSS) received neurophysiological examination, analysis of antibodies against peripheral nerve-myelin (PNM) and muscle biopsy, to show the prevalence and characteristics of peripheral neuropathy (PN) and myopathy; 3 PSS cases showed a clinical mild sensorimotor polyneuropathy, 1 of them had been treated with cytostatic drugs; 1 had mononeuropathia multiplex; and 1 clinical carpal tunnel syndrome. In these 5 patients neurophysiological investigation verified the clinical diagnosis of peripheral nerve involvement; 2 with PN had serum-antibodies against PNM; 1 of IgM-, and 1 of IgA-isotype. Muscle biopsies were taken from 15 PSS patients; 11 showed inflammatory myositis or inflammatory perivascular infiltrates and 3 showed signs of denervation. A combination of inflammation and morphological signs of myopathy, compatible with the biopsy diagnosis of polymyositis, was seen in 4, 1 of whom showed clinical signs of polymyositis. We conclude that the peripheral nervous and muscular systems are often involved in PSS, but commonly with relatively mild symptoms and laboratory findings. The common findings of inflammatory myopathy indicate an immune reaction in muscles in addition to other autoimmune manifestations of the disease.
对17例连续性原发性干燥综合征（PSS）患者进行了神经生理学检查、抗周围神经髓鞘（PNM）抗体分析及肌肉活检，以显示周围神经病变（PN）和肌病的患病率及特征；3例PSS患者表现为临床轻度感觉运动性多发性神经病，其中1例曾接受细胞毒性药物治疗；1例有多发性单神经病；1例有临床腕管综合征。在这5例患者中，神经生理学检查证实了周围神经受累的临床诊断；2例PN患者血清中有抗PNM抗体；1例为IgM型，1例为IgA型。对15例PSS患者进行了肌肉活检；11例显示炎性肌炎或血管周围炎性浸润，3例显示失神经支配迹象。4例出现与多发性肌炎活检诊断相符的炎症和肌病形态学表现，其中1例有临床多发性肌炎体征。我们得出结论，周围神经和肌肉系统在PSS中常受累，但通常症状和实验室检查结果相对较轻。炎性肌病的常见表现表明，除了该疾病的其他自身免疫表现外，肌肉中还存在免疫反应。
