Thymus histology and concomitant autoimmune diseases in Japanese patients with muscle-specific receptor tyrosine kinase-antibody-positive myasthenia gravis
Thymus histology and concomitant autoimmune diseases in Japanese patients with muscle-specific receptor tyrosine kinase-antibody-positive myasthenia gravis
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DOI：
10.1111/ene.12169
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发表时间：
2013-09-01
影响因子：
5.1
通讯作者：
Kawakami, A.
中科院分区：
文献类型：
--
作者：
Nakata, R.;Motomura, M.;Kawakami, A.
背景与目的：抗肌肉特异性受体酪氨酸激酶(MuSK)-抗体（Ab）阳性重症肌无力（MG）患者与抗乙酰胆碱受体(AChR)-Ab阳性重症肌无力（MG）患者胸腺组织学特征、共存自身免疫性疾病及相关自身抗体的差异尚不明确。方法：对全国83例麝香抗体阳性患者的胸腺组织学类型、自身免疫性疾病及相关抗体进行调查，并与我院随访的achr - ab阳性患者（n = 83）进行比较。胸腺瘤相关自身抗体检测为titin抗体。结果：麝香抗体阳性患者未出现胸腺瘤，但在achr - ab阳性患者中有21例（25.3%）出现胸腺瘤。麝香抗体阳性患者中没有Titin抗体，而achr - ab阳性患者中有25例（30.1%）呈阳性。8例musk - ab阳性患者（9.6%）伴有自身免疫性疾病，其中以桥本甲状腺炎和类风湿性关节炎为主，而22例achr - ab阳性患者（26.5%）伴有一种或多种自身免疫性疾病，其中以Graves病为主。结论：麝香- ab阳性MG与achr - ab阳性MG在胸腺瘤、胸腺增生、自身免疫性疾病及自身抗体阳性发生率上存在差异，提示与achr - ab阳性MG相比，胸腺似乎不参与麝香- ab阳性MG的发病机制。
Background and purpose: The differences in the characteristics of thymus histology, coexisting autoimmune diseases and related autoantibodies between anti-muscle-specific receptor tyrosine kinase (MuSK)-antibody (Ab)-positive myasthenia gravis (MG) patients, and anti-acetylcholine receptor (AChR)-Ab-positive MG patients are not clearly defined. Methods: The types of thymus histology, coexisting autoimmune diseases and associated Abs in 83 MuSK-Ab-positive patients nationwide were investigated and were compared with those in AChR-Ab-positive patients followed at our institute (n = 83). As for the autoantibodies associated with thymoma, titin Abs were measured. Results: Thymoma was not present in any of the MuSK-Ab-positive patients but presented in 21 patients (25.3%) amongst the AChR-Ab-positive patients. Titin Abs were absent in MuSK-Ab-positive patients but positive in 25 (30.1%) of the AChR-Ab-positive patients. Concomitant autoimmune diseases were present in eight MuSK-Ab-positive patients (9.6%) amongst whom Hashimoto's thyroiditis and rheumatoid arthritis predominated, whereas 22 AChR-Ab-positive patients (26.5%) had one or more concomitant autoimmune diseases of which Graves' disease predominated. Conclusions: Differences in frequency of thymoma and thymic hyperplasia, coexisting autoimmune diseases and autoantibody positivity between MuSK-Ab-positive and AChR-Ab-positive MG were indicated, suggesting that, in contrast with AChR-Ab-positive MG, thymus does not seem to be involved in the pathogenic mechanisms of MuSK-Ab-positive MG.