A small but variable subgroup of patients with myasthenia gravis (MG) who have antibodies to muscle-specific kinase (MuSKAb-MG) can present with distinct phenotypes and are often treatment-resistant. The prevalence, clinical phenotypes and outcomes of treatment of patients with MuSKAb-MG in Thailand were determined. Eight (16.3%) of the 49 patients with generalized MG who were negative for acetylcholine receptor antibodies (AChRAb) were positive for muscle-specific kinase antibodies. Most patients had predominant oculobulbar features and respiratory failure occurred in three. At follow up, three out of the seven patients who underwent thymectomy were in complete stable remission and four had improved and were on reduced immunosuppression medication, suggesting a possible benefit of thymectomy.

译文

具有肌肉特异性激酶 (MuSKAb-MG) 抗体的重症肌无力 (MG) 患者的一个小但可变的亚组可以表现出不同的表型,并且通常对治疗耐药。确定了泰国MuSKAb-MG患者的患病率,临床表型和治疗结果。乙酰胆碱受体抗体 (AChRAb) 阴性的49例全身性MG患者中有8例 (16.3% 例) 肌肉特异性激酶抗体呈阳性。大多数患者具有主要的眼球特征,其中三例发生呼吸衰竭。在随访中,接受胸腺切除术的7例患者中有3例完全稳定缓解,4例改善并接受了减少的免疫抑制药物治疗,这表明胸腺切除术可能具有益处。

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