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重症肌无力的异质性:眼肌型和全身型的HLA表型及自身抗体反应

Heterogeneity in myasthenia gravis: HLA phenotypes and autoantibody responses in ocular and generalized types.

作者信息

Kida K, Hayashi M, Yamada I, Matsuda H, Yoshinaga J, Takami S, Yashiki S, Sonoda S

出版信息

Ann Neurol. 1987 Mar;21(3):274-8. doi: 10.1002/ana.410210309.

DOI:10.1002/ana.410210309
PMID:3606034
Abstract

HLA phenotypes and autoantibody responses were studied in 71 Japanese patients with myasthenia gravis. HLA-A2, Bw61, and DRw9 were associated with ocular myasthenia gravis (corrected p [CP] less than 0.05 relative risk [RR] = 2.88; CP less than 0.02, RR = 3.60; and CP less than 0.001, RR = 4.63, respectively) and HLA-DRw8 was associated with generalized myasthenia gravis (CP less than 0.001, RR = 5.40). Neither HLA-B8 nor DR3 was found in Japanese patients. The titer of antiacetylcholine receptor antibody (AChR Ab) and the incidence of autoantibodies other than AChR Ab were higher in patients with generalized myasthenia gravis than in those with the ocular type (2.77 +/- 0.62 versus 0.17 +/- 0.03 pmol/ml, p less than 0.001; and 60.6 versus 29.0%, p less than 0.02, respectively). Patients with a high titer of AChR Ab or with autoantibodies had an increased frequency of HLA-DRw8 (CP less than 0.02, RR = 4.61, and CP less than 0.005, RR = 4.53, respectively); whereas patients with a low titer of AChR Ab or without autoantibodies had an increased frequency of HLA-DRw9 (CP less than 0.001, RR = 8.26, and CP less than 0.005, RR = 4.08, respectively). These findings suggest that ocular and generalized myasthenia gravis might have different immunogenetic backgrounds.

摘要

对71例日本重症肌无力患者的人类白细胞抗原(HLA)表型和自身抗体反应进行了研究。HLA - A2、Bw61和DRw9与眼肌型重症肌无力相关(校正P值[CP]小于0.05,相对风险[RR]=2.88;CP小于0.02,RR = 3.60;CP小于0.001,RR = 4.63),而HLA - DRw8与全身型重症肌无力相关(CP小于0.001,RR = 5.40)。在日本患者中未发现HLA - B8和DR3。全身型重症肌无力患者的抗乙酰胆碱受体抗体(AChR Ab)滴度及AChR Ab以外的自身抗体发生率高于眼肌型患者(分别为2.77±0.62对0.17±0.03 pmol/ml,P小于0.001;60.6%对29.0%,P小于0.02)。AChR Ab滴度高或有自身抗体的患者中HLA - DRw8频率增加(CP小于0.02,RR = 4.61;CP小于0.005,RR = 4.53);而AChR Ab滴度低或无自身抗体的患者中HLA - DRw9频率增加(CP小于0.001,RR = 8.26;CP小于0.005,RR = 4.08)。这些发现提示眼肌型和全身型重症肌无力可能具有不同的免疫遗传背景。

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Late onset myasthenia gravis is associated with HLA DRB1*15:01 in the Norwegian population.
挪威人群中迟发性重症肌无力与 HLA DRB1*15:01 相关。
PLoS One. 2012;7(5):e36603. doi: 10.1371/journal.pone.0036603. Epub 2012 May 9.
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Possible distinct pathogenesis in low responder myasthenia gravis: association of soluble interleukin-2 receptor with acetylcholine receptor antibody titre or abnormal thymus.低反应性重症肌无力可能存在的不同发病机制:可溶性白细胞介素-2受体与乙酰胆碱受体抗体滴度或胸腺异常的关联。
J Neurol Neurosurg Psychiatry. 1996 Aug;61(2):207-8. doi: 10.1136/jnnp.61.2.207.
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J Neurol Neurosurg Psychiatry. 1993 Apr;56(4):382-5. doi: 10.1136/jnnp.56.4.382.
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