MuSK-positive myasthenia gravis is rare in the Polish population
MuSK-positive myasthenia gravis is rare in the Polish population
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DOI：
10.1111/j.1468-1331.2008.02176.x
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发表时间：
2008-07-01
影响因子：
5.1
通讯作者：
Kwiecinski, H.
中科院分区：
文献类型：
--
作者：
Kostera-Pruszczyk, A.;Kaminska, A.;Kwiecinski, H.
背景和目的：在不同人群中，0-48% 的全身血清阴性 MG 病例被诊断为 MuSK 阳性重症肌无力 (MG)。抗 MuSK 抗体的存在通常与严重的病程和对胸腺切除术缺乏反应有关。我们首次分析了波兰人群中 MuSK 阳性 MG 的血清学和临床特征。方法：对 151 名患者进行了抗 AChR 和抗 MuSK 抗体检测：62 名血清阴性 MG，其中 14 名眼部血清阴性 MG，48 名年龄匹配的血清阳性 MG 患者和 41 名对照者。结果：所有血清阳性 MG 患者和疾病对照均为 MuSK 阴性。仅在四名血清阴性 MG 患者（占全身血清阴性病例的 8.7%）中检测到抗 MuSK 抗体：三名女性和一名男性。所有四人主要有延髓受累，并接受了胸腺切除术，但没有明显的获益。经过免疫抑制治疗后，所有患者的临床症状均得到改善，缓解期可持续长达 7 年。结论：MuSK 阳性 MG 在波兰人群中很少见，仅占血清阴性全身性 MG 病例的 8.7%。这与越来越多的北纬地区 MuSK 抗体较低的新证据是一致的。
Background and purpose: MuSK-positive myasthenia gravis (MG) is diagnosed in 0-48% of cases with generalized seronegative MG in different populations. The presence of anti-MuSK antibodies generally relates to a severe course and lack of response to thymectomy. We analyzed for the first time the serology and clinical characteristics of MuSK-positive MG in the Polish population. Methods: One hundred and fifty-one patients were tested for the presence of anti-AChR and anti-MuSK antibodies: 62 with seronegative MG, including 14 with ocular seronegative MG, 48 age-matched patients with seropositive MG and 41 controls. Results: All patients with seropositive MG and the disease controls were MuSK-negative. Anti-MuSK antibodies were detected only in four patients with seronegative MG (8.7% of generalized seronegative cases): three women and one man. All four had predominantly bulbar involvement, and underwent thymectomy, with no apparent benefit. All of them improved clinically after immunosuppressive treatment with remissions lasting up to 7 years. Conclusion: MuSK-positive MG is rare in Polish population accounting for only 8.7% of seronegative cases with generalized MG. This is consistent with emerging evidence for lower MuSK antibodies at more northerly latitudes.