一个难以捉摸的诊断：心包积液和高嗜酸性粒细胞增多揭示血清阴性嗜酸性肉芽肿性多血管炎：一例病例报告。
An elusive diagnosis: pericardial effusion and hypereosinophilia revealing seronegative eosinophilic granulomatosis with polyangitis: a case report.
文献信息
| PMID | 42866709 |
|---|---|
| 原文 | 在 PubMed 查看原文 ↗ |
| 发表日期 | 2026 |
| 作者 | Vishal Akula |
| 作者单位 | Mary Washington Healthcare Internal Medicine Resident, Fredericksburg, Virginia, USA. |
| 期刊 | Annals of medicine and surgery (2012) |
| SCI 分区 | Q2 |
| IF | 1.8 |
| 研究类型 | 临床研究 · 临床 |
| 所属专科 | 鼻科 |
中文摘要
背景： 嗜酸性肉芽肿性多血管炎（EGPA）是一种小血管和中血管坏死性血管炎，以哮喘、嗜酸性粒细胞增多和多器官受累为特征。心脏表现虽不常见，但在抗中性粒细胞胞浆抗体（ANCA）阴性患者中更为普遍，并伴有显著的发病率。由于表现多样且缺乏确定性检测，诊断可能具有挑战性。
方法： 我们报告一名60岁女性，患有哮喘和慢性鼻窦炎，出现进行性呼吸困难，并被发现有大心包积液伴填塞生理、双肺浸润和显著嗜酸性粒细胞增多。她接受了紧急心包开窗术和肺楔形切除术。肺活检显示嗜酸性血管炎和嗜酸性肺炎。广泛的感染和自身免疫检查，包括ANCA检测，均为阴性。在开始皮质类固醇治疗后，她表现出快速的临床改善。
结果： 哮喘、鼻窦疾病、嗜酸性粒细胞增多、肺浸润和活检结果的组合符合2022年ACR-EULAR的EGPA分类标准。她出院时服用高剂量类固醇，后来开始使用美泊利单抗进行维持治疗。
结论： 本病例突出了EGPA的诊断复杂性，特别是在具有危及生命特征（如心脏填塞）的ANCA阴性患者中。它强调了在系统性嗜酸性粒细胞增多患者中保持广泛鉴别诊断的重要性。早期识别、及时免疫抑制治疗和多学科护理对于改善预后和预防严重并发症至关重要。
英文摘要
Eosinophilic granulomatosis with polyangiitis (EGPA) is a small- and medium-vessel necrotizing vasculitis characterized by asthma, eosinophilia, and multiorgan involvement. Cardiac manifestations, though uncommon, are more prevalent in antineutrophil cytoplasmic antibody (ANCA)-negative patients and carry significant morbidity. Diagnosis can be challenging due to variable presentations and the lack of a definitive test. We present a 60-year-old woman with asthma and chronic rhinosinusitis who developed progressive dyspnea and was found to have a large pericardial effusion with tamponade physiology, bilateral pulmonary infiltrates, and marked eosinophilia. She underwent an urgent pericardial window and a lung wedge resection. Lung biopsy revealed eosinophilic vasculitis and eosinophilic pneumonia. Extensive infectious and autoimmune workups, including ANCA testing, was negative. She demonstrated rapid clinical improvement following the initiation of corticosteroids. The combination of asthma, sinus disease, eosinophilia, pulmonary infiltrates, and biopsy findings met the 2022 ACR-EULAR classification criteria for EGPA. She was discharged on high-dose steroids and was later started on mepolizumab for maintenance. This case highlights the diagnostic complexity of EGPA, particularly in ANCA-negative patients with life-threatening features such as cardiac tamponade. It underscores the importance of maintaining a broad differential in patients with systemic eosinophilia. Early recognition, timely immunosuppressive therapy, and multidisciplinary care are critical for improving outcomes and preventing severe complications.