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IgG4相关性主动脉炎

IgG4-Related Aortitis.

作者信息

Fong Chun Wa, Lio Lin Ian, Pon Monica, Mok Toi Meng, Hou Ng

机构信息

Department of Plastic Surgery, Centro Hospitalar Conde São Januário, Macau SAR.

Department of Psychiatry, Centro Hospitalar Conde São Januário, Macau SAR.

出版信息

Eur J Case Rep Intern Med. 2018 Sep 27;5(9):000881. doi: 10.12890/2018_000881. eCollection 2018.

DOI:10.12890/2018_000881
PMID:30756063
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC6346820/
Abstract

UNLABELLED

Immunoglobulin G4-related disease (IgG4-RD) is a systemic immune-mediated fibroinflammatory condition characterized by tumefactive lesions that can affect multiple organs. Serum IgG4 levels may be elevated. Early recognition is sometimes difficult but is important to avoid irreversible organ damage. We describe the case of a 28-year-old male patient who presented with a 2-year history of recurrent low-grade fever, night sweats and non-specific manifestations. We eventually diagnosed IgG4-related aortitis by PET-CT scan. The patient was successfully treated with prednisolone and mycophenolate mofetil with complete clinical and radiological resolution.

LEARNING POINTS

IgG4-related disease is a systemic immune-mediated disease that can affect many organs and systems.Aortitis can be one of the differential diagnoses in patients with fever of unknown origin, back pain and other unspecific symptoms.Delayed diagnosis and treatment may cause major complications or irreversible damage, so timely diagnosis and appropriate treatment is very important.

摘要

未标注

免疫球蛋白G4相关疾病(IgG4-RD)是一种全身性免疫介导的纤维炎症性疾病,其特征为可影响多个器官的肿块样病变。血清IgG4水平可能升高。早期识别有时很困难,但对于避免不可逆的器官损害很重要。我们描述了一名28岁男性患者的病例,该患者有2年反复低热、盗汗及非特异性表现的病史。我们最终通过PET-CT扫描诊断为IgG4相关主动脉炎。患者接受泼尼松龙和霉酚酸酯治疗成功,临床和影像学表现完全缓解。

学习要点

IgG4相关疾病是一种可影响许多器官和系统的全身性免疫介导疾病。主动脉炎可能是不明原因发热、背痛及其他非特异性症状患者的鉴别诊断之一。诊断和治疗延迟可能导致严重并发症或不可逆损害,因此及时诊断和适当治疗非常重要。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/aa85/6346820/880c883967e2/881_Fig2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/aa85/6346820/bbe35df1f6f7/881_Fig1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/aa85/6346820/880c883967e2/881_Fig2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/aa85/6346820/bbe35df1f6f7/881_Fig1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/aa85/6346820/880c883967e2/881_Fig2.jpg

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本文引用的文献

1
Recent advances in understanding and managing IgG4-related disease.IgG4相关疾病的认识与管理的最新进展
F1000Res. 2017 Feb 23;6. doi: 10.12688/f1000research.9399.1. eCollection 2017.
2
IgG4-related disease: A concise review of the current literature.IgG4相关性疾病:当前文献的简要综述
Reumatol Clin. 2017 May-Jun;13(3):160-166. doi: 10.1016/j.reuma.2016.05.009. Epub 2016 Jun 18.
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IgG4-related cardiovascular disorders.IgG4相关性心血管疾病。
Int Heart J. 2014;55(4):287-95. doi: 10.1536/ihj.13-321. Epub 2014 Jun 5.
4
Characterizing IgG4-related disease with ¹⁸F-FDG PET/CT: a prospective cohort study.¹⁸F-FDG PET/CT 对 IgG4 相关疾病的特征描述:一项前瞻性队列研究。
Eur J Nucl Med Mol Imaging. 2014 Aug;41(8):1624-34. doi: 10.1007/s00259-014-2729-3. Epub 2014 Apr 25.
5
The diagnostic utility of serum IgG4 concentrations in IgG4-related disease.血清 IgG4 浓度在 IgG4 相关疾病中的诊断效用。
Ann Rheum Dis. 2015 Jan;74(1):14-8. doi: 10.1136/annrheumdis-2013-204907. Epub 2014 Mar 20.