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[结节病和桥本甲状腺炎后发生重症肌无力1例]

[A case of myasthenia gravis following sarcoidosis and Hashimoto's thyroiditis].

作者信息

Nishida H, Tanaka Y, Nakao N, Ibi T, Sahashi K

机构信息

Department of Neurology, Gifu Prefectural Hospital.

出版信息

Rinsho Shinkeigaku. 2000 Aug;40(8):797-800.

Abstract

Here, we report on an elderly woman with sarcoidosis and Hashimoto's disease who later developed myasthenia gravis. She was 68-year-old with a long history of Hashimoto's disease who had a clinical diagnosis of sarcoidosis with uveritis at the age of 66 years. On laboratory examination, angiotensin-converting enzyme, lysozyme and gamma-globulin were elevated and there was bilateral hilar lymphoadenopathy. She was admitted to our hospital because of left blepharoptosis and mild fatigability in the proximal muscles at the age of 68 years. Myasthenia gravis, type IIa, was confirmed by elevated titer of anti-acetylcholine receptor antibody in serum (0.8 nmol/l, normal < 0.6), positive edrophonium test and decremental EMG response. Oral prednisolone therapy was effective. Her muscle biopsy revealed HLA ABC-positive fibers in all fascicles, and HLA-DR positive fibers in the perifascicular areas. Myasthenia gravis complicated by sarcoidosis and Hashimoto's thyroiditis is extremely rare, suggesting that the common underlying immunological abnormalities for the three disorders such as a certain defective cellular immunity are responsible for the pathomechanism to induce the patient condition.

摘要

在此,我们报告一例患有结节病和桥本氏病的老年女性,该患者后来发展为重症肌无力。她68岁,有长期的桥本氏病病史,66岁时临床诊断为结节病伴葡萄膜炎。实验室检查显示,血管紧张素转换酶、溶菌酶和γ-球蛋白升高,且存在双侧肺门淋巴结肿大。她因68岁时出现左眼睑下垂和近端肌肉轻度疲劳而入住我院。血清抗乙酰胆碱受体抗体滴度升高(0.8 nmol/l,正常<0.6)、依酚氯铵试验阳性及肌电图反应递减,确诊为IIa型重症肌无力。口服泼尼松龙治疗有效。她的肌肉活检显示所有肌束中HLA ABC阳性纤维,以及束周区域HLA-DR阳性纤维。重症肌无力合并结节病和桥本氏甲状腺炎极为罕见,提示这三种疾病共同的潜在免疫异常,如某种细胞免疫缺陷,是导致患者病情的发病机制。

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