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嗜酸性肉芽肿性多血管炎(Churg-Strauss综合征)伪装成急性ST段抬高型心肌梗死,免疫抑制治疗后完全缓解:一例报告

Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome) masquerading as acute ST-elevation myocardial infarction with complete resolution after immunosuppressive therapy: a case report.

作者信息

Chai Joshua T, McGrath Sam, Lopez Begoña, Dworakowski Rafal

机构信息

Department of Cardiology, King's College Hospital, Denmark Hill, London, UK.

Department of Rheumatology, King's College Hospital, Denmark Hill, London, UK.

出版信息

Eur Heart J Case Rep. 2018 Sep 21;2(3):yty075. doi: 10.1093/ehjcr/yty075. eCollection 2018 Sep.

Abstract

BACKGROUND

Eosinophilic granulomatosis with polyangiitis (EGPA, formerly known as Churg-Strauss syndrome) is a rare autoimmune condition characterized by inflammation of small- and medium-sized blood vessels, which usually presents with systemic vasculitis preceded by airway allergic hypersensitivity.

CASE SUMMARY

Here, we report a highly unusual case of acute ST-elevation myocardial infarction in a young and fit man with no cardiovascular risk factors. His emergency coronary angiography revealed disproportionately severe widespread coronary artery disease. We describe the diagnostic challenges with emphasis on meticulous history-taking (deep hyponasal voice, anosmia, and childhood asthma), supported by timely blood markers (peripheral eosinophilia and raised CRP), and multi-modal imaging (severe paranasal sinusitis on cranial magnetic resonance imaging and multiple lung infiltrates with small patches of ground-glass appearance on thoracic computed tomography), to reach a diagnosis of EGPA coronary vasculitis with particular reference to the American College of Rheumatology EGPA classification. Importantly, with prompt immunosuppression, his coronary lesions resolved completely without the need of any surgical or percutaneous revascularisation. He remained well and asymptomatic on maintenance immunosuppressants at 1 year follow-up.

DISCUSSION

This case highlighted the rare but recognized involvement of the coronary arteries in systemic EGPA vasculitis, which can sometimes mimic atherosclerotic coronary disease and acute coronary syndrome.

摘要

背景

嗜酸性肉芽肿性多血管炎(EGPA,曾称为变应性肉芽肿性血管炎)是一种罕见的自身免疫性疾病,其特征为中小血管炎症,通常在气道过敏性超敏反应之前出现系统性血管炎。

病例摘要

在此,我们报告一例极为罕见的急性ST段抬高型心肌梗死病例,患者为一名年轻健康男性,无心血管危险因素。其急诊冠状动脉造影显示冠状动脉疾病广泛且严重程度不成比例。我们描述了诊断过程中的挑战,重点强调了细致的病史采集(鼻腔深部声音低沉、嗅觉减退和儿童期哮喘),及时的血液指标(外周血嗜酸性粒细胞增多和C反应蛋白升高)以及多模态影像学检查(头颅磁共振成像显示严重的鼻窦炎,胸部计算机断层扫描显示肺部有多个小片状磨玻璃样改变的浸润影)的支持,以根据美国风湿病学会EGPA分类标准确诊为EGPA冠状动脉血管炎。重要的是,通过及时的免疫抑制治疗,其冠状动脉病变完全消退,无需任何外科手术或经皮血管重建治疗。在1年的随访中,他在维持免疫抑制剂治疗下情况良好且无症状。

讨论

该病例突出了系统性EGPA血管炎中冠状动脉罕见但已被认识到的受累情况,这种情况有时可模拟动脉粥样硬化性冠状动脉疾病和急性冠状动脉综合征。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d721/6177047/4b5565c5b953/yty075f1.jpg

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