摘要： 目的 分析伴其他自身免疫性疾病的重症肌无力患者之临床特点、药物疗效、预后和转归。方法 共83 例重症肌无力患者，分为伴其他自身免疫性疾病重症肌无力（AIDMG）组（24 例）和不伴其他自身免疫性疾病重症肌无力（NAIDMG）组（59 例），比较两组患者性别、发病年龄、首发症状、胸腺异常等临床特点、不同治疗措施之疗效和预后。结果 两组患者性别（χ² = 8.467，P = 0.004）、眼睑下垂侧别（χ² = 9.830，P = 0.007）、发病2 年内病程情况（χ² = 15.255，P = 0.001）差异具有统计学意义，而发病年龄（χ² = 1.728，P = 0.228）、首发症状（χ² = 0.252，P = 0.791）、胸腺异常（χ² = 3.200，P = 0.202）组间差异无统计学意义。两组患者溴吡斯的明（χ² = 0.411，P = 0.395）、糖皮质激素（χ² = 0.156，P = 0.513）、静脉注射免疫球蛋白（χ² = 0.359，P = 0.462）、免疫抑制剂（χ² = 0.081，P = 0.526）、胸腺切除术（χ² = 0.337，P = 0.391）等治疗措施之疗效，以及眼肌型重症肌无力进展为全身型重症肌无力比例（χ² = 1.826，P = 0.148）、进展时间（Fisher确切概率法：P = 0.639）、首发症状（Fisher确切概率法：P = 0.196）和复发时间（Fisher确切概率法：P = 1.000）差异均无统计学意义。结论 AIDMG 患者多见于女性，首发症状为眼睑下垂，以双侧同时受累为主；发病2年内较NAIDMG 患者更易复发。

关键词: 重症肌无力, 自身免疫疾病

Abstract: Objective  To analyze the clinical characteristics, drug efficacy and prognosis of patients with myasthenia gravis (MG) associated with other autoimmune diseases. Methods  Eighty-three MG patients were divided into 2 groups. One group included MG patients with autoimmune diseases (AIDMG, N = 24), and the other included MG patients without autoimmune diseases (NAIDMG, N = 59). Firstly, clinical features such as sex, age of onset, initial symptoms and thymus abnormalities were compared between patients with AIDMG and NAIDMG. Secondly, effect of different therapies, including pyridostigmine, corticosteroids, immunoglobulin, immunosuppressants and thymectomy was compared between 2 groups. Finally, prognosis including relapse rate and recurrence time during the first 2 years after MG onset was compared. Whether and when ocular myasthenia gravis (OMG) progressing to general myasthenia gravis (GMG) and the first onset of GMG symptoms during the first 2 years were also compared between 2 groups.  Results  The difference of gender predominance (χ² = 8.467, P = 0.004), ptosis affecting left or right or both sides (χ² = 9.830, P = 0.007) and disease course within 2 years after onset (χ² = 15.255, P = 0.001) between AIDMG group and NAIDMG group were statistically significant. Other clinical features such as age of onset (χ² = 1.728, P = 0.228), initial symptoms (χ² = 0.252, P = 0.791), thymus abnormalities (χ² = 3.200, P = 0.202) were not significantly different between 2 groups. Differences of therapeutical effect such as pyridostigmine (χ² = 0.411, P = 0.395), corticosteroids (χ² = 0.156, P = 0.513), immunoglobulin (χ² = 0.359, P = 0.462), immunosuppressants (χ² = 0.081, P = 0.526) and thymectomy (χ² = 0.337, P = 0.391) between 2 groups were not statistically significant. The ratio of OMG progressing to GMG (χ² = 1.826, P = 0.148), time of progressing (Fisher's exact test: P = 0.639), first onset symptom (Fisher's exact test: P = 0.196) and recurrence time (Fisher's exact test: P = 1.000) were not significantly different between 2 groups. Conclusions  AIDMG was more common in female patients. Bilateral ptosis involvement at the same time was more common in AIDMG. Relapse rate during the first 2 years after MG onset was higher in AIDMG than that in NAIDMG.

Key words: Myasthenia gravis, Autoimmune diseases