Division of Paediatrics, Keelung Chang Gung Memorial Hospital, 222, Maijin Road, Keelung, Taiwan.
Division of Paediatric Neurology, Chang Gung Memorial Hospital and Chang Gung Children's Hospital, Chang Gung University College of Medicine, 33305, No. 5, Fuxing St., Guishan Dist, Taoyuan, Taiwan.
BMC Neurol. 2019 Jul 18;19(1):170. doi: 10.1186/s12883-019-1397-0.
Myasthenia gravis is the most common disease affecting the neuromuscular junction. The most common etiology among patients with juvenile myasthenia gravis is the production of antibodies against the acetylcholine receptor. However, the clinical outcome in relation to serum levels of anti-acetylcholine receptor antibodies in juvenile myasthenia gravis has rarely been discussed. We aimed to analyze the correlation between the presence of anti-acetylcholine receptor antibodies and outcome in juvenile myasthenia gravis.
Patients diagnosed with juvenile myasthenia gravis younger than of 20 years of age were retrospectively recruited from January 1995 to February 2017 in a tertiary referral medical center. According to the Myasthenia Gravis Foundation of America outcome scale, the primary outcome was complete symptom remission and cessation of medications for at least 1 year measured 2 years after diagnosis. Secondary outcome was complete symptom remission at the last outpatient clinic.
A total of 54 patients were followed up for over 2 years. Nine patients (9/54, 16.7%) achieved complete remission without medication use at 2 years after diagnosis. Thirteen (24.1%) patients achieved complete remission during longer follow-up periods. Those with negative anti-acetylcholine receptor antibodies were more likely to achieve complete remission at 2 years (6/15 [40%] vs. 3/39 [7.7%], 95% Confidence interval [CI] 1.670 to 38.323) and at the last outpatient clinic follow-up (8/15 [53.3%] vs. 5/39 [12.8%], 95% CI 2.367 to 20.704). Thirteen patients with comorbid autoimmune thyroid diseases were older than those without disease (11.8 ± 5.8 years old vs. 8.0 ± 6.3 years old, 95% CI 0.018 to 7.33). Moreover, patients negative for anti-acetylcholine receptor antibodies were less likely comorbid with autoimmune thyroid disease (1/35 [2.9%] vs. 12/71 [16.9%], 95% CI 0.018 to 1.161).
Juvenile myasthenia gravis patients without anti-acetylcholine antibodies exhibited significantly increased complete remission rates and a reduced likelihood of comorbid autoimmune thyroid diseases compared with those with anti-acetylcholine receptor antibodies among Chinese.
重症肌无力是最常见的影响神经肌肉接头的疾病。在青少年重症肌无力患者中，最常见的病因是产生抗乙酰胆碱受体抗体。然而，在青少年重症肌无力中，血清抗乙酰胆碱受体抗体水平与临床结果之间的关系很少被讨论。我们旨在分析抗乙酰胆碱受体抗体的存在与青少年重症肌无力的结果之间的相关性。
我们回顾性地招募了 1995 年 1 月至 2017 年 2 月在一家三级转诊医疗中心就诊的年龄小于 20 岁的青少年重症肌无力患者。根据美国重症肌无力基金会的结果量表，主要结果是在诊断后 2 年内完全缓解且停止用药至少 1 年。次要结果是最后一次门诊就诊时完全缓解。
共有 54 例患者随访超过 2 年。9 例（9/54，16.7%）患者在诊断后 2 年内无需药物治疗即可完全缓解。13 例（24.1%）患者在较长的随访期间达到完全缓解。那些抗乙酰胆碱受体抗体阴性的患者在 2 年内更有可能达到完全缓解（6/15 [40%] vs. 3/39 [7.7%]，95%置信区间[CI] 1.670 至 38.323）和最后一次门诊随访时（8/15 [53.3%] vs. 5/39 [12.8%]，95%CI 2.367 至 20.704）。合并自身免疫性甲状腺疾病的 13 例患者比未合并疾病的患者年龄更大（11.8±5.8 岁 vs. 8.0±6.3 岁，95%CI 0.018 至 7.33）。此外，抗乙酰胆碱受体抗体阴性的患者更不可能合并自身免疫性甲状腺疾病（1/35 [2.9%] vs. 12/71 [16.9%]，95%CI 0.018 至 1.161）。
与抗乙酰胆碱受体抗体阳性的中国青少年重症肌无力患者相比，抗乙酰胆碱抗体阴性的青少年重症肌无力患者完全缓解率显著增加，且合并自身免疫性甲状腺疾病的可能性降低。
