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Gleich 综合征:系统评价。

Gleich syndrome: a systematic review.

机构信息

Dermatology Department, Faculty of Medicine, Balamand University, Saint Georges Hospital UMC, Beirut, Lebanon.

Pulmonary Medicine and Critical Care Department, Saint Georges Hospital UMC, Beirut, Lebanon.

出版信息

Int J Dermatol. 2020 Dec;59(12):1458-1465. doi: 10.1111/ijd.14963. Epub 2020 Jun 18.

Abstract

Characterized chiefly by hypereosinophilia and angioedema, Gleich syndrome is a rare disorder with unclear clinical and therapeutic findings. Other symptoms include increased IgM levels, weight gain, fever, and urticaria. Herein we review Gleich syndrome and assess clinical features, epidemiology, and treatment options. Thirty-two articles including case reports or case series of eosinophilic angioedema and Gleich syndrome were included. Data regarding patient age, gender, and history, clinical and biological manifestations, and treatment protocols were recorded. The most common clinical findings include recurrent or non-recurrent angioedema, fever, urticaria, weight gain, blood eosinophilia, and elevated immunoglobulin levels. Corticosteroid therapy is the mainstay of treatment. Gleich syndrome is a distinctive hypereosinophilic entity with a benign course and good response to systemic corticosteroids. More studies are needed to evaluate the pathophysiology of this syndrome and lead to better therapeutic options.

摘要

格莱希综合征的特征主要为嗜酸性粒细胞增多和血管性水肿,是一种罕见疾病,临床和治疗发现尚不清楚。其他症状包括 IgM 水平升高、体重增加、发热和荨麻疹。本文对格莱希综合征进行综述,并评估其临床特征、流行病学和治疗选择。纳入了 32 篇包括嗜酸性粒细胞性血管性水肿和格莱希综合征病例报告或病例系列的文章。记录了患者年龄、性别和病史、临床和生物学表现以及治疗方案的数据。最常见的临床发现包括复发性或非复发性血管性水肿、发热、荨麻疹、体重增加、血液嗜酸性粒细胞增多和免疫球蛋白水平升高。皮质类固醇治疗是主要的治疗方法。格莱希综合征是一种独特的高嗜酸性粒细胞实体,具有良性病程和对全身皮质类固醇的良好反应。需要更多的研究来评估该综合征的病理生理学,并提供更好的治疗选择。

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