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1例合并多器官自身免疫(包括多发性肌炎和心肌炎)的胸腺瘤。

A case of thymoma-associated multiorgan autoimmunity including polymyositis and myocarditis.

作者信息

Furuta Chihiro, Yano Motoki, Numanami Hiroki, Yamaji Masayuki, Taguchi Rumiko, Haniuda Masayuki

机构信息

Division of Chest Surgery, Department of Surgery, Aichi Medical University, 1-1 Yazakokarimata, Nagakute, 480-1195, Japan.

出版信息

Surg Case Rep. 2021 Oct 20;7(1):226. doi: 10.1186/s40792-021-01309-1.

Abstract

BACKGROUND

Polymyositis and myocarditis associated with thymoma are exceptionally rare conditions and usually accompanied by myasthenia gravis (MG) and have been recognized as critical conditions. Thymoma-associated multiorgan autoimmunity was reported recently with skin, liver, and intestinal manifestations similar to those seen in graft-versus-host disease.

CASE PRESENTATION

A 77-year-old female presented to our department with exacerbation of ptosis and local recurrence of thymoma. Chest computed tomography revealed local recurrence of thymoma. Following 6 month observation, erythema on the extremities and body trunk suddenly appeared. Afterwards, the patient developed progressive muscle weakness and fatigue. We diagnosed as myocarditis and polymyositis. She was transferred to the intensive-care unit and received artificial ventilation. Steroid pulse therapy was induced immediately. The blood test findings were markedly improved, but the symptoms of MG and weakness of the muscles persisted. Various treatment including eculizumab was induced, and the symptoms of MG and weakness of the muscles were improved. On the 136th day of hospitalization, she was discharged.

CONCLUSION

We were able to cure this patient, as we were able to start treatment immediately after the appearance of severe symptoms. An early diagnosis and treatment are important for curing such patients.

摘要

背景

与胸腺瘤相关的多发性肌炎和心肌炎极为罕见,通常伴有重症肌无力(MG),并被视为危急病症。最近报道了胸腺瘤相关的多器官自身免疫,其皮肤、肝脏和肠道表现与移植物抗宿主病相似。

病例介绍

一名77岁女性因上睑下垂加重和胸腺瘤局部复发就诊于我院。胸部计算机断层扫描显示胸腺瘤局部复发。经过6个月观察,患者四肢和躯干突然出现红斑。此后,患者出现进行性肌肉无力和疲劳。我们诊断为心肌炎和多发性肌炎。她被转入重症监护病房并接受人工通气。立即进行了类固醇冲击治疗。血液检查结果明显改善,但重症肌无力症状和肌肉无力仍然存在。采用了包括依库珠单抗在内的各种治疗方法,重症肌无力症状和肌肉无力得到改善。住院第136天,患者出院。

结论

我们能够治愈该患者,因为在出现严重症状后能够立即开始治疗。早期诊断和治疗对于治愈此类患者很重要。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f96b/8528923/dbcaad3d762d/40792_2021_1309_Fig1_HTML.jpg

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