Suppr超能文献

重症肌无力中的胸腺瘤:从诊断到治疗

Thymoma in myasthenia gravis: from diagnosis to treatment.

作者信息

Romi Fredrik

机构信息

Department of Neurology, Haukeland University Hospital, 5021 Bergen, Norway.

出版信息

Autoimmune Dis. 2011;2011:474512. doi: 10.4061/2011/474512. Epub 2011 Aug 10.

Abstract

One half of cortical thymoma patients develop myasthenia gravis (MG), while 15% of MG patients have thymomas. MG is a neuromuscular junction disease caused in 85% of the cases by acetylcholine receptor (AChR) antibodies. Titin and ryanodine receptor (RyR) antibodies are found in 95% of thymoma MG and 50% of late-onset MG (MG onset ≥50 years), are associated with severe disease, and may predict thymoma MG outcome. Nonlimb symptom profile at MG onset with bulbar, ocular, neck, and respiratory symptoms should raise the suspicion about the presence of thymoma in MG. The presence of titin and RyR antibodies in an MG patient younger than 60 years strongly suggests a thymoma, while their absence at any age strongly excludes thymoma. Thymoma should be removed surgically. Prethymectomy plasmapheresis/iv-IgG should be considered before thymectomy. The pharmacological treatment does not differ from nonthymoma MG, except for tacrolimus which is an option in difficult thymoma and nonthymoma MG cases with RyR antibodies.

摘要

皮质型胸腺瘤患者中有一半会发生重症肌无力(MG),而MG患者中有15%患有胸腺瘤。MG是一种神经肌肉接头疾病,85%的病例由乙酰胆碱受体(AChR)抗体引起。在95%的胸腺瘤MG患者和50%的晚发型MG(MG发病年龄≥50岁)患者中发现肌联蛋白和兰尼碱受体(RyR)抗体,这些抗体与严重疾病相关,可能预测胸腺瘤MG的预后。MG发病时出现延髓、眼部、颈部和呼吸道症状等非肢体症状特征,应提高对MG患者存在胸腺瘤的怀疑。60岁以下的MG患者中存在肌联蛋白和RyR抗体强烈提示胸腺瘤,而在任何年龄不存在这些抗体则强烈排除胸腺瘤。胸腺瘤应手术切除。胸腺切除术前应考虑进行血浆置换/静脉注射免疫球蛋白。除了他克莫司可用于治疗困难的胸腺瘤和伴有RyR抗体的非胸腺瘤MG病例外,药物治疗与非胸腺瘤MG并无不同。

相似文献

1
Thymoma in myasthenia gravis: from diagnosis to treatment.重症肌无力中的胸腺瘤:从诊断到治疗
Autoimmune Dis. 2011;2011:474512. doi: 10.4061/2011/474512. Epub 2011 Aug 10.
2
Paraneoplastic myasthenia gravis: immunological and clinical aspects.副肿瘤性重症肌无力:免疫学及临床方面
Eur J Neurol. 2008 Oct;15(10):1029-33. doi: 10.1111/j.1468-1331.2008.02242.x. Epub 2008 Aug 20.
5
Myasthenia gravis patients with ryanodine receptor antibodies have distinctive clinical features.
Eur J Neurol. 2007 Jun;14(6):617-20. doi: 10.1111/j.1468-1331.2007.01785.x.

引用本文的文献

2
Imaging Findings in Neurogenic Ptosis.神经源性上睑下垂的影像学表现
J Korean Soc Radiol. 2025 Jul;86(4):483-500. doi: 10.3348/jksr.2024.0107. Epub 2025 Jul 25.
6
Ectopic Thymoma in the Thorax With Associated Myasthenia Gravis.胸部异位胸腺瘤伴重症肌无力
Cureus. 2024 Oct 22;16(10):e72146. doi: 10.7759/cureus.72146. eCollection 2024 Oct.
8
Thymoma With Myasthenia Gravis: A Study of Two Cases.胸腺瘤合并重症肌无力:两例病例研究
Cureus. 2024 Aug 25;16(8):e67739. doi: 10.7759/cureus.67739. eCollection 2024 Aug.

本文引用的文献

5
Thymoma and paraneoplastic myasthenia gravis.胸腺瘤与副肿瘤性重症肌无力。
Autoimmunity. 2010 Aug;43(5-6):413-27. doi: 10.3109/08916930903555935.
6
Immunosuppressive therapies in myasthenia gravis.重症肌无力的免疫抑制疗法。
Autoimmunity. 2010 Aug;43(5-6):428-35. doi: 10.3109/08916930903518107.
9
Myasthenia gravis patients with ryanodine receptor antibodies have distinctive clinical features.
Eur J Neurol. 2007 Jun;14(6):617-20. doi: 10.1111/j.1468-1331.2007.01785.x.
10
Morphological effects of myasthenia gravis patient sera on human muscle cells.
Muscle Nerve. 2006 Jan;33(1):93-103. doi: 10.1002/mus.20443.

文献检索

告别复杂PubMed语法,用中文像聊天一样搜索,搜遍4000万医学文献。AI智能推荐,让科研检索更轻松。

立即免费搜索

文件翻译

保留排版,准确专业,支持PDF/Word/PPT等文件格式,支持 12+语言互译。

免费翻译文档

深度研究

AI帮你快速写综述,25分钟生成高质量综述,智能提取关键信息,辅助科研写作。

立即免费体验