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自身免疫性重症肌无力合并侵袭性胸腺瘤患者的病例系列

A Case Series of Patients With Autoimmune Myasthenia Gravis in Association With Invasive Thymoma.

作者信息

McCombe Pamela, Raj Meenakshi, Henderson Robert, Blum Stefan

机构信息

*The University of Queensland Centre for Clinical Research and Department of Neurology, Royal Brisbane and Women's Hospital, Herston, Queensland, Australia; and †Department of Neurology, Royal Brisbane and Women's Hospital, Herston, Queensland, Australia.

出版信息

J Clin Neuromuscul Dis. 2016 Mar;17(3):129-34. doi: 10.1097/CND.0000000000000101.

Abstract

OBJECTIVES

In some patients, autoimmune myasthenia gravis (MG) is associated with thymic hyperplasia or thymoma, and in some patients the thymoma is invasive. Little is known about the clinical course of subjects who present with MG and are found to have invasive thymoma.

METHODS

We reviewed the patients at our clinic with MG and invasive thymoma, and have described their clinical features.

RESULTS

Six MG patients were diagnosed with invasive thymoma. The subjects had extensive multimodal therapy. Two subjects died from uncontrolled neuromuscular respiratory failure despite aggressive immunosuppression, 2 subjects had other autoimmune disorders (Morvan syndrome and polymyositis), and 2 subjects are controlled on immunosuppression.

CONCLUSIONS

Patients with invasive thymoma and MG can have a poor outcome, with difficulty in controlling myasthenic symptoms and can have other clinically important autoimmune diseases.

摘要

目的

在一些患者中,自身免疫性重症肌无力(MG)与胸腺增生或胸腺瘤相关,且部分患者的胸腺瘤具有侵袭性。对于罹患MG且被发现患有侵袭性胸腺瘤的患者的临床病程,人们了解甚少。

方法

我们回顾了我院门诊中患有MG和侵袭性胸腺瘤的患者,并描述了他们的临床特征。

结果

6例MG患者被诊断为侵袭性胸腺瘤。这些患者接受了广泛的多模式治疗。尽管进行了积极的免疫抑制治疗,但仍有2例患者死于无法控制的神经肌肉呼吸衰竭,2例患者患有其他自身免疫性疾病(莫旺综合征和多发性肌炎),2例患者的病情通过免疫抑制得到控制。

结论

患有侵袭性胸腺瘤和MG的患者预后可能较差,难以控制肌无力症状,并且可能患有其他具有临床重要性的自身免疫性疾病。

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