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病例报告:显微镜下多血管炎患者主动脉根部出现肿块。

Case report: Granulomatosis with polyangiitis patient presented with a mass in the aortic root.

作者信息

Teng Yinyan, Chen Mei, Chen Zhongwei, Zhang Xiaowei, Li Zhongyun, Liao Shusheng

机构信息

Department of Ultrasound, The First Affiliated Hospital of Wenzhou Medical University, Wenzhou, Zhejiang, China.

Department of Ultrasonography, The First Affiliated Hospital of Ningbo University, Ningbo, Zhejiang, China.

出版信息

Front Immunol. 2024 Nov 28;15:1373769. doi: 10.3389/fimmu.2024.1373769. eCollection 2024.

Abstract

Granulomatosis with polyangiitis (GPA) is an autoimmune inflammatory disease that affects small- and medium-sized blood vessels in the body, representing a rare entity. Cardiac involvement was identified as an independent risk factor for death in GPA patients, yet it has not been systematically elucidated in previous literature. Cardiac lesions in patients with GPA can manifest in various ways, such as pericarditis, myocarditis, coronary vasculitis, valvular abnormalities, conduction system abnormalities, and heart failure. Herein, we report a 55-year-old woman with GPA; she had a 2-year history of recurrent episodes of headache, accompanying sickness, and fatigue, which have been aggravated for the past half-month. The main manifestation is presenting as a mass in the aortic root, which was successfully diagnosed by multimodality imaging (including two-dimensional echocardiography, contrast-enhanced ultrasound, and computed tomography). After treatment with methylprednisolone and cyclophosphamide, the patient's symptoms significantly improved and she remained asymptomatic over 6 months of follow-up. This article will enrich our knowledge about cardiac involvement in GPA patients and highlights the value of imaging, especially ultrasound, in the diagnosis and post-treatment follow-up of this condition.

摘要

肉芽肿性多血管炎(GPA)是一种自身免疫性炎症性疾病,累及体内中小血管,较为罕见。心脏受累被确定为GPA患者死亡的独立危险因素,但此前文献尚未对其进行系统阐述。GPA患者的心脏病变可表现为多种形式,如心包炎、心肌炎、冠状动脉血管炎、瓣膜异常、传导系统异常和心力衰竭。在此,我们报告一名55岁的GPA女性患者;她有2年反复发作头痛、伴恶心和疲劳的病史,在过去半月病情加重。主要表现为主动脉根部有一肿块,通过多模态成像(包括二维超声心动图、超声造影和计算机断层扫描)成功确诊。经甲泼尼龙和环磷酰胺治疗后,患者症状明显改善,随访6个月无症状。本文将丰富我们对GPA患者心脏受累情况的认识,并强调成像,尤其是超声,在该病诊断和治疗后随访中的价值。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/33be/11635199/33a5896dd021/fimmu-15-1373769-g001.jpg

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