重症肌无力伴胸腺瘤或胸腺增生的临床特征及预后

Characteristics and Outcome in Myathenia Gravis Accompanied with Thymoma or Thymic Hyperplasia

目的分析伴胸腺瘤或胸腺增生的重症肌无力患者临床特点及预后。方法回顾性分析2007年1月至2016年12月伴胸腺瘤重症肌无力患者118例和伴胸腺增生重症肌无力患者42例,统计并比较两组发病年龄、性别、首发症状、临床分型、受累肌群、肌无力危象、低频重复神经电刺激及完全稳定缓解资料。结果与伴胸腺增生重症肌无力患者相比,伴胸腺瘤重症肌无力患者平均发病年龄大,Ⅲ型比例高,术后完全稳定缓解率低(P<0.05),更易发生呼吸肌受累(χ~2=6.364,P=0.011)及肌无力危象(χ~2=5.455,P=0.022)。不同病理分型胸腺瘤伴发的重症肌无力患者之间呼吸肌受累(χ~2=8.532,P=0.036)和肌无力危象(χ~2=8.956,P=0.030)发生率存在显著性差异,以B3型最高,A+AB型最低。伴胸腺瘤重症肌无力患者重复神经电刺激阳性率高于伴胸腺增生重症肌无力患者,但无显著性差异(χ~2<1.357,P>0.05);不同病理分型胸腺瘤重症肌无力患者有上升趋势,但无显著性差异(χ~2<6.623,P>0.05)。结论伴胸腺瘤重症肌无力多见于中老年人,临床症状重,易有呼吸肌受累和肌无力危象,伴胸腺增生重症肌无力更能从手术中获益。重症肌无力低频重复神经电刺激阳性率与胸腺病变类型无明显关联。重症肌无力呼吸肌受累和肌无力危象与胸腺瘤病理分型有关。

Objective To explore the clinical characteristics and outcome in myathenia gravisac companied with thymoma or thymic hyperplasia.Methods From January,2007to December,2016,118myathenia gravis patients with thymoma,and42myathenia gravis patients with thymic hyperplasia were enrolled.Their age of onset,gender,initial symptom,osserman classification,muscles involved,myasthenia crisis,low-frequency repetitive nerve stimulation and complete stable remission rate data were retrospectively analyzed.Results Compared with myathenia gravis patients with thymic hyperplasia,myathenia gravis patients with thymoma showed older age in onset,higher proportion in type III and lower rate in complete stable remission rate(P<0.05),and more respiratory muscle involved(χ2=6.364,P=0.011)and myasthenia crisis(χ2=5.455,P=0.022).There were significant differences in respiratory muscle involved(χ2=8.532,P=0.036)and the incidence rate of myasthenia crisis(χ2=8.956,P=0.030)among thymoma with different pathological types,in which type B3was the highest,and type A+AB was the lowest.The low-frequency repetitive nerve stimulation positive rate was higher in myathenia gravis patients with thymoma than with thymic hyperplasia,however,there was no significant difference(χ2<1.357,P>0.05).There was a rising trend with pathological classification altered in the low-frequency repetitive nerve stimulation positive rate with no significant difference(χ2<6.623,P>0.05).Conclusion Myathenia gravis patients with thymoma were common in middle-aged to old people,who were serious in clinical symptom,particularly respiratory muscle involved and myasthenia crisis.Myathenia gravis patients with thymic hyperplasia were more benefit from thymectomy.The low-frequency repetitive nerve stimulation positive rate of myathenia gravis patients does not correlate with thymic lesion type.Respiratory muscle involved and myasthenia crisis are relevant with pathological types of thymoma.