嗜酸性肉芽肿性多血管炎患者出现心脏压塞和眶周肿胀的非典型表现：一例报告
An atypical presentation of cardiac tamponade and periorbital swelling in a patient with eosinophilic granulomatosis with polyangiitis: a case report.
作者信息
Keefe Alexandra C, Hymas Joseph C, Emerson Lyska L, Ryan John J
机构信息
Department of Medicine, University of Utah School of Medicine, Salt Lake City, UT, USA.
General Cardiology, St. Luke's Hospital, Twin Falls, ID, USA.
出版信息
J Med Case Rep. 2017 Sep 24;11(1):271. doi: 10.1186/s13256-017-1434-9.
BACKGROUND
Eosinophilic granulomatosis with polyangiitis is a rare, necrotizing systemic vasculitis associated with asthma and hypereosinophilia. Its cause and pathophysiology are still being elucidated.
CASE PRESENTATION
We report a case of eosinophilic granulomatosis with polyangiitis in a 50-year-old Caucasian woman who presented with chest pain, dyspnea at rest, fever, and periorbital swelling. She was found to have significant hypereosinophilia and cardiac tamponade physiology. A biopsy confirmed extensive infiltration of both lungs and pericardium by eosinophils. She did not have any anti-neutrophil cytoplasmic antibodies.
CONCLUSIONS
Eosinophilic granulomatosis with polyangiitis diagnosis does not require the presence of anti-neutrophil cytoplasmic antibodies. Anti-neutrophil cytoplasmic antibody-positive and anti-neutrophil cytoplasmic antibody-negative eosinophilic granulomatosis with polyangiitis may present with different clinical phenotypes, perhaps suggesting two distinct disease etiologies and distinct pathophysiology.
背景
嗜酸性肉芽肿性多血管炎是一种罕见的坏死性系统性血管炎，与哮喘和嗜酸性粒细胞增多有关。其病因和病理生理学仍在研究中。
病例报告
我们报告一例50岁白种女性的嗜酸性肉芽肿性多血管炎病例，该患者表现为胸痛、静息时呼吸困难、发热和眶周肿胀。发现她有明显的嗜酸性粒细胞增多和心脏压塞的体征。活检证实双肺和心包均有大量嗜酸性粒细胞浸润。她没有任何抗中性粒细胞胞浆抗体。
结论
嗜酸性肉芽肿性多血管炎的诊断并不需要抗中性粒细胞胞浆抗体的存在。抗中性粒细胞胞浆抗体阳性和抗中性粒细胞胞浆抗体阴性的嗜酸性肉芽肿性多血管炎可能表现出不同的临床表型，这或许提示两种不同的疾病病因和病理生理学。
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