Jensen Katharine V, Brochez Nicholas, Spence Christopher, Livingston Joel, Khoury Michael, McColl Jeanine
Department of Pediatrics, Faculty of Medicine & Dentistry, University of Alberta, Edmonton, AB, Canada.
Division of General Pediatrics, Stollery Children's Hospital, Edmonton, AB, Canada.
SAGE Open Med Case Rep. 2024 Dec 19;12:2050313X241309966. doi: 10.1177/2050313X241309966. eCollection 2024.
Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare systemic necrotizing vasculitis marked by eosinophilia and extravascular granulomas, predominantly affecting the respiratory tract. This report details a unique EGPA case in a 6-year-old girl with extensive cardiac involvement, featuring an atypical intracardiac mass suggestive of endomyocardial fibrosis and a concomitant thrombus. The clinical course unfolded in three phases: an initial prodrome with asthma; subsequent peripheral hypereosinophilia; and ultimately systemic vasculitis. Cardiac involvement, notably an intracardiac mass in the right ventricular apex extending into the interventricular septum, underscored the diverse nature of EGPA. The patient fulfilled sufficient criteria outlined by the American College of Rheumatology and the European Alliance of Associations for Rheumatology for an EGPA diagnosis, displaying hypereosinophilia, obstructive airway disease, and biopsy-confirmed inflammation predominantly characterized by extravascular eosinophils. Treatment included high-dose methylprednisolone and cyclophosphamide, which resulted in clinical improvement and inflammatory marker normalization. To halt right ventricular thrombus progression, therapeutic unfractionated heparin was initiated, and she was transitioned to warfarin, which resulted in complete resolution of the cardiac mass. This case highlights the necessity of a multidisciplinary approach for managing complex EGPA manifestations, particularly in pediatrics, and emphasizes the importance of timely intervention in mitigating the impact of cardiac complications associated with EGPA.
嗜酸性肉芽肿性多血管炎（EGPA）是一种罕见的系统性坏死性血管炎，其特征为嗜酸性粒细胞增多和血管外肉芽肿，主要累及呼吸道。本报告详细介绍了一名6岁女孩的独特EGPA病例，该病例有广泛的心脏受累，其特征是存在提示心内膜纤维化的非典型心内肿块及并发血栓形成。临床病程分为三个阶段：最初为哮喘前驱期；随后出现外周血嗜酸性粒细胞增多；最终发展为系统性血管炎。心脏受累，尤其是右心室心尖部延伸至室间隔的一个心内肿块，突显了EGPA的多样性。该患者符合美国风湿病学会和欧洲抗风湿病联盟协会概述的EGPA诊断的充分标准，表现为嗜酸性粒细胞增多、阻塞性气道疾病以及活检证实的以血管外嗜酸性粒细胞为主的炎症。治疗包括大剂量甲基泼尼松龙和环磷酰胺，治疗后临床症状改善且炎症标志物恢复正常。为阻止右心室血栓进展，开始使用普通肝素进行治疗，并将其转换为华法林，最终心脏肿块完全消退。该病例突出了采用多学科方法管理复杂EGPA表现的必要性，尤其是在儿科患者中，并强调了及时干预对于减轻与EGPA相关的心脏并发症影响的重要性。
