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首页> 外文期刊>Journal of neurology >Screening for lipoprotein receptor-related protein 4-, agrin-, and titin-antibodies and exploring the autoimmune spectrum in myasthenia gravis
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Screening for lipoprotein receptor-related protein 4-, agrin-, and titin-antibodies and exploring the autoimmune spectrum in myasthenia gravis

机译：筛选脂蛋白受体相关蛋白4-，农毒素和棘肽 - 抗体，并探索Myasthenia的自身免疫光谱

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In autoimmune myasthenia gravis (MG), the identification of antibodies and characterization of serological subgroups is of great importance for diagnosis and management of the disease. Our aims were to study the frequency of antibodies against lipoprotein-related protein 4 (LRP4), agrin, and titin using the most recent techniques, and to characterize corresponding clinical features and autoimmune diseases (AID) in 100 MG-patients. The antibody frequencies in the 55 AChR-antibody positive patients were 7% LRP4, 5% agrin, 53% titin, and in the 45 AChR-antibody negative patients 2% MuSK, 2% LRP4, 2% agrin, and 27% titin. LRP4-MG presented late-onset age, mild symptoms, good therapeutic response, and no thymic changes. Agrin-MG showed early onset age, mild-to-severe symptoms, and moderate treatment response. The phenotype of titin-MG depended on AChR-antibodies: AChR-antibody negative patients presented with mostly mild limb muscle weakness, whereas AChR-antibody positive patients showed more frequently severe symptoms, including myasthenic crisis, bulbar predominance, and thymoma. Additional AID were detected in 32% of MG-patients, most frequently Hashimoto's thyroiditis (21%). Based on our data, we recommend the detection of LRP4-antibodies for at least AChR-antibody negative MG-patients and titin-antibodies for all MG-patients. We propose taking an accurate medical history for typical symptoms of Hashimoto's thyroiditis in MG-patients.
机译：在自身免疫性重症肌无力（MG）中，抗体的鉴定和血清学亚群的特征对于疾病的诊断和治疗具有重要意义。我们的目的是使用最新技术研究脂蛋白相关蛋白4（LRP4）、agrin和titin抗体的频率，并描述100 MG患者的相应临床特征和自身免疫疾病（AID）。55例AChR抗体阳性患者的抗体频率为7%LRP4、5%agrin、53%titin，45例AChR抗体阴性患者的抗体频率为2%MuSK、2%LRP4、2%agrin和27%titin。LRP4-MG起病年龄晚，症状轻，疗效好，无胸腺改变。Agrin MG起病年龄早，症状轻到重，治疗反应中等。titin-MG的表型取决于AChR抗体：AChR抗体阴性患者大多表现为轻度肢体肌无力，而AChR抗体阳性患者则更常表现为严重症状，包括肌无力危象、延髓占优势和胸腺瘤。32%的MG患者检测到额外的辅助，最常见的是桥本甲状腺炎（21%）。根据我们的数据，我们建议至少对AChR抗体阴性的MG患者检测LRP4抗体，并对所有MG患者检测titin抗体。我们建议对MG患者桥本甲状腺炎的典型症状进行准确的病史记录。

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