论文

重症肌无力伴胸腺瘤或胸腺增生的临床特征及预后

  • 杨玲 ,
  • 王国防 ,
  • 丁意平 ,
  • 刁珊珊 ,
  • 徐耑
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  • 苏州大学附属第一医院,江苏苏州市 215006。
杨玲(1991-),女,汉族,江苏宿迁市人,硕士研究生,主要研究方向:神经肌肉接头疾病。

收稿日期: 2017-07-05

  修回日期: 2017-09-11

  网络出版日期: 2017-12-28

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

  • YANG Ling ,
  • WANG Guo-fang ,
  • DING Yi-ping ,
  • DIAO Shan-shan ,
  • XU Zhuan
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  • The First Affiliated Hospital of Soochow University, Suzhou, Jiangsu 215006, China

Received date: 2017-07-05

  Revised date: 2017-09-11

  Online published: 2017-12-28

摘要

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(χ2=6.364, P=0.011)及肌无力危象(χ2=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(χ2=8.532, P=0.036)和肌无力危象(χ2=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(χ2<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(χ2<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。

本文引用格式

杨玲 , 王国防 , 丁意平 , 刁珊珊 , 徐耑 . 重症肌无力伴胸腺瘤或胸腺增生的临床特征及预后[J]. 中国康复理论与实践, 2017 , 23(12) : 1425 -1430 . DOI: 10.3969/j.issn.1006-9771.2017.12.012

Abstract

Objective To explore the clinical characteristics and outcome in myathenia gravisac companied with thymoma or thymic hyperplasia. Methods From January, 2007 to December, 2016, 118 myathenia gravis patients with thymoma, and 42 myathenia 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 B3 was 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.

参考文献

[1] Marx A, Pfister F, Schalke B, et al. The different roles of the thymus in the pathogenesis of the various myasthenia gravis subtypes [J]. Autoimmun Rev, 2013, 12(9): 875-884.
[2] Cavalcante P, Le PR, Berrih-Aknin S, et al. The thymus in myasthenia gravis: Site of "innate autoimmunity" [J]. Muscle Nerve, 2011, 44(4): 467-484.
[3] Buckley C, Douek D, Newsom-Davis J, et al. Mature, long-lived CD 4 + and CD 8 + T cells are generated by the thymoma in myasthenia gravis [J]. Ann Neurol, 2001, 50(1): 64-72.
[4] Luther C, Poeschel S, Varga M, et al. Decreased frequency of intrathymic regulatory T cells in patients with myasthenia-associated thymoma [J]. J Neuroimmunol, 2005, 164(1-2): 124-128.
[5] Marx A, Willcox N, Leite MI, et al. Thymoma and paraneoplastic myasthenia gravis [J]. Autoimmunity, 2010, 43(5-6): 413-427.
[6] 陈玉萍,王卫,王中魁,等. 重症肌无力伴发胸腺瘤患者的临床特点分析[J]. 中华内科杂志, 2012, 51(8): 623-625.
[7] 邢岩,崔丽英,管宇宙,等. 72例重症肌无力伴胸腺瘤的临床及神经电生理研究[J]. 中华医学杂志, 2007, 87(45): 3188-3191.
[8] Meriggioli MN, Sanders DB. Autoimmune myasthenia gravis: emerging clinical and biological heterogeneity [J]. Lancet Neurol, 2009, 8(5): 475-490.
[9] Akaishi T, Yamaguchi T, Suzuki Y, et al. Insights into the classification of myasthenia gravis [J]. PLoS One, 2014, 9(9): e106757.
[10] Nakajima J, Okumura M, Yano M, et al. Myasthenia gravis with thymic epithelial tumour: a retrospective analysis of a Japanese database [J]. Eur J Cardiothorac Surg, 2016, 49(5): 1510-1515.
[11] Karasaki T, Murakawa T, Nagayama K, et al. New onset of myasthenia gravis 10 years after proton beam therapy for thymoma [J]. Gen Thorac Cardiovasc Surg, 2016, 64(5): 290-293.
[12] Huang X, Liu WB, Men LN, et al. Clinical features of myasthenia gravis in southern China: a retrospective review of 2,154 cases over 22 years [J]. Neurol Sci, 2013, 34(6): 911-917.
[13] Romi F. Thymoma in myasthenia gravis: from diagnosis to treatment [J]. Autoimmune Dis, 2011, 2011: 474512.
[14] Romi F, Aarli JA, Gilhus NE. Myasthenia gravis patients with ryanodine receptor antibodies have distinctive clinical features [J]. Eur J Neurol, 2007, 14(6): 617-620.
[15] Romi F, Skeie GO, Aarli JA, et al. The severity of myasthenia gravis correlates with the serum concentration of titin and ryanodine receptor antibodies [J]. Arch Neurol, 2000, 57(11): 1596-1600.
[16] Chu XY, Xue ZQ, Wang RW, et al. Predictors of postoperative myasthenic crisis in patients with myasthenia gravis after thymectomy [J]. Chin Med J (Engl), 2011, 124(8): 1246-1250.
[17] 刘宇,束余声. 胸腺切除术后发生肌无力危象危险因素的Meta分析[J]. 中华胸心血管外科杂志, 2015, 31(11): 660-664.
[18] Liang T, Boulos MI, Murray BJ, et al. Detection of myasthenia gravis using electrooculography signals [J]. Conf Proc IEEE Eng Med Biol Soc, 2016, 2016: 896-899.
[19] Nakata M, Kuwabara S, Kawaguchi N, et al. Is excitation-contraction coupling impaired in myasthenia gravis [J]. Clin Neurophysiol, 2007, 118(5): 1144-1148.
[20] Jing F, Cui F, Chen Z, et al. Clinical and electrophysiological markers in myasthenia gravis patients [J]. Eur Neurol, 2015, 74(1-2): 22-27.
[21] De Roxas RC, Bagnas MA, Baldonado JJ, et al. Clinical profile and outcome of postthymectomy versus non-thymectomy myasthenia gravis patients in the philippine general hospital: a 6-year retrospective study [J]. Front Neurol, 2016, 7: 96.
[22] Bak V, Spalek P, Rajcok M, et al. Importance of thymectomy and prognostic factors in the complex treatment of myasthenia gravis [J]. Bratisl Lek Listy, 2016, 117(4): 195-200.
[23] Spillane J, Hayward M, Hirsch NP, et al. Thymectomy: role in the treatment of myasthenia gravis [J]. J Neurol, 2013, 260(7): 1798-1801.
[24] Nazarbaghi S, Amiri-Nikpour MR, Mahmodlou R, et al. Clinical outcomes of myasthenia gravis with thymoma and thymic hyperplasia undergoing extended transsternal thymectomy: a single-center experience [J]. N Am J Med Sci, 2015, 7(11): 503-508.
[25] Mao Z, Hu X, Lu Z, et al. Prognostic factors of remission in myasthenia gravis after thymectomy [J]. Eur J Cardiothorac Surg, 2015, 48(1): 18-24.
[26] Bak V, Spalek P, Rajcok M, et al. Importance of thymectomy and prognostic factors in the complex treatment of myasthenia gravis [J]. Bratisl Lek Listy, 2016, 117(4): 195-200.
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