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抗中性粒细胞胞浆抗体阴性的嗜酸性肉芽肿性多血管炎中的多发性脑梗死和心内膜炎

Multiple brain infarctions and endomyocarditis in ANCA-negative eosinophilic granulomatosis with polyangiitis.

作者信息

Tashiro Takumi, Fujiwara Urara, Kira Yuichi, Karashima Chihiro, Maeda Norihisa

机构信息

Department of Neurology, National Hospital Organization Beppu Medical Center, Japan.

Department of Cardiology, National Hospital Organization Beppu Medical Center, Japan.

出版信息

Heliyon. 2023 Jan 7;9(1):e12881. doi: 10.1016/j.heliyon.2023.e12881. eCollection 2023 Jan.

Abstract

Eosinophilic granulomatosis with polyangiitis (EGPA) is a small vessel necrotizing vasculitis characterized by asthma and eosinophilia. Ischemic stroke is a rare complication of the disease. We herein report a case involving a 77-year-old woman with sinusitis who developed embolic stroke and splenic infarctions. Laboratory tests revealed hypereosinophilia and elevated troponin-T and N-terminal pro-brain natriuretic peptide. Antineutrophil cytoplasmic antibodies (ANCA) studies were negative. Skin biopsy showed infiltration of eosinophils into the arterial walls. These clinicopathological findings led to the diagnosis of EGPA. We also found the evidence of endomyocarditis as revealed by multimodality cardiac imaging. The patient underwent continuous immunosuppressive and anticoagulation therapy, and the infarctions did not recur. This report highlights the importance of histologically proven vasculitis with eosinophil infiltration and careful examination for cardiac involvement, especially in ANCA-negative patients.

摘要

嗜酸性肉芽肿性多血管炎(EGPA)是一种以哮喘和嗜酸性粒细胞增多为特征的小血管坏死性血管炎。缺血性中风是该疾病的一种罕见并发症。我们在此报告一例涉及一名77岁患鼻窦炎女性的病例,该患者发生了栓塞性中风和脾梗死。实验室检查显示嗜酸性粒细胞增多以及肌钙蛋白-T和N末端脑钠肽前体升高。抗中性粒细胞胞浆抗体(ANCA)检查为阴性。皮肤活检显示嗜酸性粒细胞浸润动脉壁。这些临床病理表现导致了EGPA的诊断。我们还通过多模态心脏成像发现了心内膜炎的证据。该患者接受了持续的免疫抑制和抗凝治疗,梗死未复发。本报告强调了组织学证实的伴有嗜酸性粒细胞浸润的血管炎以及仔细检查心脏受累情况的重要性,尤其是在ANCA阴性患者中。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4531/9860291/8704a3aa6dff/gr1.jpg

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