Romi F, Skeie G O, Aarli J A, Gilhus N E
Department of Neurology, Haukeland University Hospital, 5021 Bergen, Norway.
Arch Neurol. 2000 Nov;57(11):1596-600. doi: 10.1001/archneur.57.11.1596.
Myasthenia gravis (MG) is caused by autoantibodies to the acetylcholine receptor (AChR). Non-AChR muscle autoantibodies are present in many MG serum samples, mainly from patients with thymoma or late-onset MG. The exact relationship between MG severity and several non-AChR muscle antibodies is unknown.
To study the correlation between the severity of MG and the concentration of antibodies against striated muscle tissue sections, titin, citric acid antigen, ryanodine receptor, and AChR.
The severity of MG was graded in 146 consecutive patients with MG, and their serum samples were tested for the presence of autoantibodies. Ten patients who were titin antibody positive were observed in longitudinal follow-up.
No significant difference was found in MG severity between late-onset and thymoma MG. Titin, citric acid antigen, and ryanodine receptor antibodies occurred significantly more often among patients with severe MG than among patients with less severe disease. Changes in MG severity correlated with changes in titin antibody titer in the individual patient. Titin antibodies showed a better longitudinal correlation with disease severity than the AChR antibodies.
Non-AChR muscle autoantibodies occurred more frequently in severe MG regardless of MG subgroup. Thymoma per se does not generate a more severe MG. It may well be the presence of a humoral immune response to non-AChR muscle antigens such as titin, citric acid antigen, and ryanodine receptor that leads to a severe disease, not the presence of thymoma or a late age of onset. These antibodies can serve as important prognostic markers in MG regardless of the presence of thymoma.
重症肌无力(MG)由抗乙酰胆碱受体(AChR)自身抗体引起。许多MG血清样本中存在非AChR肌肉自身抗体,主要来自胸腺瘤患者或晚发型MG患者。MG严重程度与几种非AChR肌肉抗体的确切关系尚不清楚。
研究MG严重程度与抗横纹肌组织切片、肌联蛋白、柠檬酸抗原、兰尼碱受体和AChR抗体浓度之间的相关性。
对146例连续性MG患者的MG严重程度进行分级,并检测其血清样本中自身抗体的存在情况。对10例肌联蛋白抗体阳性患者进行纵向随访观察。
晚发型MG和胸腺瘤型MG在MG严重程度上无显著差异。严重MG患者中肌联蛋白、柠檬酸抗原和兰尼碱受体抗体的出现频率显著高于病情较轻的患者。个体患者中MG严重程度的变化与肌联蛋白抗体滴度的变化相关。与AChR抗体相比,肌联蛋白抗体与疾病严重程度的纵向相关性更好。
无论MG亚组如何,非AChR肌肉自身抗体在严重MG中出现的频率更高。胸腺瘤本身并不会导致更严重的MG。很可能是对肌联蛋白、柠檬酸抗原和兰尼碱受体等非AChR肌肉抗原的体液免疫反应导致了严重疾病,而非胸腺瘤的存在或发病年龄较晚。无论胸腺瘤是否存在,这些抗体都可作为MG重要的预后标志物。