Alam Loba, Lasam Glenmore, Fishberg Robert
Department of Medicine, Atlantic Health System-Overlook Medical Center, Summit, NJ 07901, United States.
Department of Cardiology, Icahn School of Medicine, Mount Sinai Heart at Mount Sinai Morningside, New York, NY 10025, United States.
World J Cardiol. 2020 Sep 26;12(9):460-467. doi: 10.4330/wjc.v12.i9.460.
Eosinophilic granulomatosis polyangiitis (EGPA) is a small vessel necrotizing vasculitis that commonly presents as peripheral eosinophilia and asthma; however, it can rarely manifest with cardiac involvement such as pericarditis and cardiac tamponade. Isolated pericardial tamponade presenting as the initial symptom of EGPA is exceedingly rare. Early diagnosis and appropriate treatment are crucial to prevent life-threatening outcomes.
52-year-old woman with no past medical history presented with progressive dyspnea and dry cough. On physical exam she had a pericardial friction rub and bilateral rales. Vital signs were notable for tachycardia at 119 beats per minute and hypoxia with 89% oxygen saturation. On laboratory exam, she had 45% peripheral eosinophilia, troponin elevation of 1.1 ng/mL and N-terminal prohormone of brain natriuretic peptide of 2101 pg/mL. TTE confirmed a large pericardial effusion and tamponade physiology. She underwent urgent pericardial window procedure. Pericardial and lung biopsy demonstrated eosinophilic infiltration. Based on the American College of Radiology guidelines, the patient was diagnosed with EGPA which manifested in its rare form of cardiac tamponade. She was treated with steroid taper and mepolizumab.
This case highlights that when isolated pericardial involvement occurs in EGPA, diagnosis is recognized by performing pericardial biopsy demonstrating histopathologic evidence of eosinophilic infiltration.
嗜酸性肉芽肿性多血管炎（EGPA）是一种小血管坏死性血管炎，通常表现为外周嗜酸性粒细胞增多和哮喘；然而，它很少表现为心脏受累，如心包炎和心脏压塞。以心脏压塞为初始症状的孤立性心包炎极为罕见。早期诊断和适当治疗对于预防危及生命的后果至关重要。
一名52岁无既往病史的女性出现进行性呼吸困难和干咳。体格检查时，她有心包摩擦音和双侧啰音。生命体征显示心动过速，每分钟119次，存在低氧血症，氧饱和度为89%。实验室检查显示外周嗜酸性粒细胞增多45%，肌钙蛋白升高至1.1 ng/mL，脑钠肽前体N末端为2101 pg/mL。经胸超声心动图（TTE）证实有大量心包积液和心脏压塞表现。她接受了紧急心包开窗术。心包和肺活检显示嗜酸性粒细胞浸润。根据美国放射学会指南，该患者被诊断为EGPA，表现为罕见的心脏压塞形式。她接受了激素减量治疗和美泊利珠单抗治疗。
该病例强调，当EGPA出现孤立的心包受累时，通过心包活检显示嗜酸性粒细胞浸润的组织病理学证据来进行诊断。
