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重症肌无力患者并发意外纤维性纵隔炎:病例报告。

Unexpected fibrous mediastinitis in a patient with myasthenia gravis - a case report.

机构信息

Department of Thoracic Surgery, West China Hospital, Sichuan University, No. 37, Guoxue Alley, Chengdu, Sichuan, 610041, China.

Department of Thoracic Surgery, Guang'an People's Hospital, Guangan, Sichuan, 638000, China.

出版信息

J Cardiothorac Surg. 2023 Nov 14;18(1):322. doi: 10.1186/s13019-023-02417-9.

DOI:10.1186/s13019-023-02417-9
PMID:37964310
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10647180/
Abstract

BACKGROUND

Fibrous mediastinitis (FM) is a rare mediastinal lesion characterized by proliferation of fibrous tissue within the mediastinum. Previous reports have shown that this lesion can be caused by histoplasmosis and tuberculosis. In extremely rare cases, FM can also be caused by autoimmune diseases such as antineutrophil cytoplasmic antibody-associated vasculitis and large-vessel arteritis.

CASE PRESENTATION

In our case, we report unexpected fibrous mediastinitis found after robotic thymectomy in a patient with myasthenia gravis (MG). The preoperative imaging indicated no obvious lesion in the mediastinum and the patient denied histories of both histoplasmosis and tuberculosis. After the operation, both proliferation of fibrous tissue and ectopic germinal centres (GCs) could be found in the thymus.

CONCLUSION

This rare case might enrich our knowledge of the relationship between FM and autoimmune diseases.

摘要

背景

纤维性纵隔炎(FM)是一种罕见的纵隔病变,其特征是纵隔内纤维组织增生。既往报道显示,这种病变可由组织胞浆菌病和结核病引起。在极少数情况下,FM 也可由自身免疫性疾病引起,如抗中性粒细胞胞质抗体相关性血管炎和大动脉炎。

病例介绍

在我们的病例中,我们报告了一例重症肌无力(MG)患者在机器人胸腺切除术后发现的意外纤维性纵隔炎。术前影像学检查未提示纵隔有明显病变,患者也否认有组织胞浆菌病和结核病病史。术后,胸腺内可见纤维组织增生和异位生发中心(GCs)。

结论

这一罕见病例可能丰富我们对 FM 与自身免疫性疾病关系的认识。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/654e/10647180/8d15b302d150/13019_2023_2417_Fig2_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/654e/10647180/e7f05e7ecdce/13019_2023_2417_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/654e/10647180/8d15b302d150/13019_2023_2417_Fig2_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/654e/10647180/e7f05e7ecdce/13019_2023_2417_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/654e/10647180/8d15b302d150/13019_2023_2417_Fig2_HTML.jpg

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Ann Thorac Surg. 2022 Sep;114(3):e223-e225. doi: 10.1016/j.athoracsur.2021.11.046. Epub 2021 Dec 22.
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Fibrosing/Sclerosing Lesions of the Mediastinum: A Review.纵隔纤维/硬化性病变:综述。
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Idiopathic Mediastinal Fibrosis: a Systemic Immune-Mediated Disorder. A Case Series and a Review of the Literature.特发性纵隔纤维化:一种系统性免疫介导疾病。病例系列及文献综述。
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