Ha Yura, Pyo Min Ju, Hong Ye Eun, Nam So Hye, Song Woo-Jung, Kwon Hyouk-Soo, Kim Tae-Bum, Cho Yoo Sook, Lee Ji-Hyang
Department of Medical Science, AMIST, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Republic of Korea.
Division of Rheumatology, Department of Internal Medicine, Keimyung University, Dongsan Medical Center, Daegu, Republic of Korea.
World Allergy Organ J. 2024 Oct 24;17(11):100981. doi: 10.1016/j.waojou.2024.100981. eCollection 2024 Nov.
Episodic angioedema with eosinophilia, also known as Gleich's syndrome, is a differential diagnosis in patients with recurrent angioedema with higher blood eosinophils. Meanwhile, less has been elucidated regarding non-episodic angioedema with eosinophilia (NEAE). This study aimed to examine the prevalence, clinical characteristics, and disease course of NEAE.
By reviewing the electronic medical records, we identified patients with NEAE among those referred to allergy clinics due to eosinophilia from January 2021 to December 2023 at Asan Medical Center.
Among 687 patients with eosinophilia, 58 (8.4%) were diagnosed with and treated for NEAE. All patients were females, with a mean age of 31.79 years. The mean absolute blood eosinophil count was 4468.76 cells/μL. All patients reported symmetric angioedema of the lower legs, and 37 (63.8%) had additional angioedema of the upper arms. Twenty-five (43.1%) patients reported a preceding event prior to onset of angioedema. Systemic corticosteroids (mean total dose 1745 ± 508.49 mg) were prescribed to all patients, with a treatment duration of approximately 40 days to achieve resolution. Following the resolution of angioedema, 6 patients experienced persistent arthralgia, 1 developed chronic spontaneous urticaria, and 1 developed hypereosinophilic syndrome.
NEAE is an essential differential diagnosis in young female patients with eosinophilia, particularly those presenting with symmetric peripheral angioedema.
伴有嗜酸性粒细胞增多的发作性血管性水肿，也称为格莱希综合征，是复发性血管性水肿且血液嗜酸性粒细胞增多患者的鉴别诊断之一。与此同时，关于非发作性嗜酸性粒细胞增多性血管性水肿（NEAE）的研究较少。本研究旨在探讨NEAE的患病率、临床特征和病程。
通过查阅电子病历，我们在2021年1月至2023年12月因嗜酸性粒细胞增多转诊至峨山医院过敏门诊的患者中识别出NEAE患者。
在687例嗜酸性粒细胞增多患者中，58例（8.4%）被诊断为NEAE并接受治疗。所有患者均为女性，平均年龄31.79岁。平均绝对血液嗜酸性粒细胞计数为4468.76个/μL。所有患者均报告小腿对称性血管性水肿，37例（63.8%）上臂有额外的血管性水肿。25例（43.1%）患者报告在血管性水肿发作前有前驱事件。所有患者均接受了全身糖皮质激素治疗（平均总剂量1745±508.49mg），治疗持续约40天以达到缓解。血管性水肿消退后，6例患者出现持续性关节痛，1例发展为慢性自发性荨麻疹，1例发展为高嗜酸性粒细胞综合征。
NEAE是嗜酸性粒细胞增多的年轻女性患者，尤其是出现对称性外周血管性水肿患者的重要鉴别诊断。
