35例伴其他自身免疫性疾病的重症肌无力临床特点

摘要

Objective To analyze the clinical features of 35 patients with myasthenia gravis (MG) associated with other autoimmune diseases (ADMG). Methods One hundred seventy MG patients were divided into 2 groups. One group included MG patients with autoimmune diseases ( n=35), and the other included MG patients without auto⁃immune diseases (NADMG, n=135). Clinical features such as gender, age of onset, types of MG, myasthenic crisis, thy⁃mus abnormalities, different therapies, ocular MG progressing to general MG, and relapse of MG within one year were compared between ADMG and NADMG. Results 85.7%ADMG patients were female which was higher compared with NADMG (P< 0.001); median age of onset was 40 years old (23~45 years old) in ADMG group which was younger than NADMG group. Ocular MG and thymic hyperplasia were more frequent in ADMG group than in NADMG group (45.7%vs. 24.4%, P=0.013;14.3%vs. 1.5%,P=0.001,respectively). Other clinical features such as myasthenic crisis, thymoma, different therapies, ocular MG progressing to general MG, and relapse of MG within one year were not signifi⁃cantly different between the two groups. In multivariate analysis, female (OR=4.76, 95% CI:1.64-13.77; P= 0.004), age of onset (OR=0.96, 95%CI:0.92-0.99;P=0.019), ocular MG (OR=3.10, 95%CI:1.30-7.41;P=0.011) and thy⁃ mic hyperplasia(OR=16.26, 95%CI:2.22-119.11;P=0.006) were significantly different between the two groups. Con⁃clusions ADMG is more common in female patients with ocular MG and thymic hyperplasia.%目的：分析35例伴其他自身免疫性疾病的重症肌无力患者（myasthenia gravis associates with other autoimmune disease, ADMG）的临床特点。方法共170例重症肌无力患者，分为ADMG组（35例）和不伴其他自身免疫性疾病重症肌无力组（maythenia gravis without other autoimmune disease, NADMG，135例），比较两组患者性别、发病年龄、重症肌无力分型、肌无力危象、胸腺异常、药物治疗、重症肌无力进展及复发等临床特点。结果 ADMG女性30例，占85.7%，较NADMG组（54.8%）多见（P＜0.001）；中位年龄为40岁（23～45岁），较NADMG组年轻（P=0.039）。眼肌型重症肌无力在ADMG组多见（45.7%vs.24.4%；P=0.013）。且AD⁃MG组胸腺增生较NADMG组常见（14.3% vs.1.5%；P=0.001）。而肌无力危象、胸腺瘤、药物治疗、眼肌型重症肌无力进展为全身型重症肌无力、1年内重症肌无力复发组间差异无统计学意义。进一步多因素分析表明，两组患者性别（OR=4.76，95%CI：1.64~13.77；P=0.004）、年龄（OR=0.96，95%CI：0.92~0.99；P=0.019）、眼肌型重症肌无力（OR=3.10，95%CI：1.30~7.41；P=0.011）、胸腺增生（OR=16.26，95%CI：2.22~119.11；P=0.006）差异具有统计学意义。结论 ADMG组患者多见于青年女性，表现为眼肌型重症肌无力，并伴发胸腺增生。