Romi Fredrik
Department of Neurology, Haukeland University Hospital, 5021 Bergen, Norway.
Autoimmune Dis. 2011;2011:474512. doi: 10.4061/2011/474512. Epub 2011 Aug 10.
One half of cortical thymoma patients develop myasthenia gravis (MG), while 15% of MG patients have thymomas. MG is a neuromuscular junction disease caused in 85% of the cases by acetylcholine receptor (AChR) antibodies. Titin and ryanodine receptor (RyR) antibodies are found in 95% of thymoma MG and 50% of late-onset MG (MG onset ≥50 years), are associated with severe disease, and may predict thymoma MG outcome. Nonlimb symptom profile at MG onset with bulbar, ocular, neck, and respiratory symptoms should raise the suspicion about the presence of thymoma in MG. The presence of titin and RyR antibodies in an MG patient younger than 60 years strongly suggests a thymoma, while their absence at any age strongly excludes thymoma. Thymoma should be removed surgically. Prethymectomy plasmapheresis/iv-IgG should be considered before thymectomy. The pharmacological treatment does not differ from nonthymoma MG, except for tacrolimus which is an option in difficult thymoma and nonthymoma MG cases with RyR antibodies.
皮质型胸腺瘤患者中有一半会发生重症肌无力(MG),而MG患者中有15%患有胸腺瘤。MG是一种神经肌肉接头疾病,85%的病例由乙酰胆碱受体(AChR)抗体引起。在95%的胸腺瘤MG患者和50%的晚发型MG(MG发病年龄≥50岁)患者中发现肌联蛋白和兰尼碱受体(RyR)抗体,这些抗体与严重疾病相关,可能预测胸腺瘤MG的预后。MG发病时出现延髓、眼部、颈部和呼吸道症状等非肢体症状特征,应提高对MG患者存在胸腺瘤的怀疑。60岁以下的MG患者中存在肌联蛋白和RyR抗体强烈提示胸腺瘤,而在任何年龄不存在这些抗体则强烈排除胸腺瘤。胸腺瘤应手术切除。胸腺切除术前应考虑进行血浆置换/静脉注射免疫球蛋白。除了他克莫司可用于治疗困难的胸腺瘤和伴有RyR抗体的非胸腺瘤MG病例外,药物治疗与非胸腺瘤MG并无不同。