韩国队列中眼肌型与全身型重症肌无力的比较研究
Chaerin Kwon1, Jeeyoung Oh1,2,3, Hyun Jin Shin1,3,4
1Graduate School of Medicine, Konkuk University, Seoul, Republic of Korea.
目的:
通过整合分析临床特征、诊断结果和治疗模式,比较韩国队列中的眼肌型重症肌无力(OMG)与全身型重症肌无力(GMG)。
方法:
我们对138例重症肌无力患者(98例为眼肌型重症肌无力,40例为全身型重症肌无力)进行了回顾性研究。评估了患者的 demographic characteristics(人口统计学特征)、ophthalmic symptoms(眼部症状)、serologic results(血清学结果)、repetitive nerve stimulation test (RNST) findings(重复神经电刺激试验(RNST)结果)、thymic pathology(胸腺病理)、autoimmune comorbidities(自身免疫共病)以及 treatment patterns(治疗模式)。
结果:
GMG患者的症状 onset 年龄较轻,女性比例高于OMG患者。合并水平和垂直复视在OMG中更为常见。GMG患者的AChR抗体阳性率显著高于OMG患者(92.5% vs. 36.3%),异常重复神经刺激试验(RNST)结果的比例亦更高(90.0% vs. 23.5%)。在整体队列中,AChR抗体滴度与最大CMAP波幅递减之间存在轻度但具有统计学意义的正相关关系(r = 0.381,p = 0.011);然而,在分别对OMG和GMG患者进行分析时,该关联无统计学意义。胸腺异常(尤其是胸腺瘤)在GMG中更为常见。非甾体类免疫抑制剂(特别是他克莫司和硫唑嘌呤)的使用在GMG患者中显著更频繁,而糖皮质激素的使用在两种亚型间无显著差异。在OMG患者中,同时表现为上睑下垂和复视的患者较单有上睑下垂者更常接受全身性免疫治疗。
结论:
在该韩国队列中,眼肌型重症肌无力(OMG)与全身型重症肌无力(GMG)在人口统计学、血清学、电生理学及治疗特征方面表现出明显差异。认识这些亚型特异性的差异可能有助于实现准确诊断、个体化治疗选择以及改善临床管理。
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