Faisant C, Boccon-Gibod I, Mansard C, Dumestre Perard C, Pralong P, Chatain C, Deroux A, Bouillet L
Department of Internal Medicine, Grenoble University Hospital.
Grenoble Alps University.
Clin Exp Immunol. 2016 Jul;185(1):81-5. doi: 10.1111/cei.12789. Epub 2016 Apr 13.
Idiopathic histaminergic acquired angioedema (IH-AAE) is a common cause of recurrent angioedema without wheals. It is a mast cell-mediated disease thought to belong to the same clinical entity as chronic urticaria (CU). The objective of this study was to describe the clinical and epidemiological characteristics of IH-AAE patients. From 2014 to 2015, 534 patients were seen at our national reference centre for angioedema and/or urticaria. Among them, we identified 31 patients with idiopathic histaminergic acquired angioedema without wheals (IH-AAE). Thirty-one patients (15 men and 16 women) with a mean age of 50 years met the criteria for IH-AAE. The average delay in diagnosis was 6·3 years. A history of allergy was found in 12 patients (38·7%), nine suffering from allergic rhinitis. The mean duration of attacks was 28·1 h. The AE attack was located in the upper respiratory tract in 54·8% of cases (17 patients). A lingual location was found in 29% of patients. Men were more likely than women to have an upper airway involvement. No intubations or admissions to intensive care units were reported. The dosage of anti-histamines to control the symptoms was onefold the recommended dose in 51·6% of patients (16 patients), twofold in 32% (10 patients) and three-fourfold in 16·1% (five patients). IH-AAE is characterized by an important delay in diagnosis, a frequent involvement of the upper airway and a benign course during attacks. As in CU, a trial of up to fourfold dose of H1-anti-histamines may be necessary to control symptoms.
特发性组胺能获得性血管性水肿（IH - AAE）是反复出现无风团性血管性水肿的常见原因。它是一种肥大细胞介导的疾病，被认为与慢性荨麻疹（CU）属于同一临床实体。本研究的目的是描述IH - AAE患者的临床和流行病学特征。2014年至2015年期间，我们国家血管性水肿和/或荨麻疹参考中心共诊治了534例患者。其中，我们确定了31例患有特发性组胺能获得性无风团血管性水肿（IH - AAE）的患者。31例患者（15名男性和16名女性），平均年龄50岁，符合IH - AAE的标准。诊断的平均延迟时间为6.3年。12例患者（38.7%）有过敏史，其中9例患有过敏性鼻炎。发作的平均持续时间为28.1小时。54.8%的病例（17例患者）血管性水肿发作位于上呼吸道。29%的患者发现有舌部受累。男性比女性更易出现上气道受累。未报告有插管或入住重症监护病房的情况。51.6%的患者（16例）控制症状所需的抗组胺药剂量是推荐剂量的1倍，32%（10例）是2倍，16.1%（5例）是3至4倍。IH - AAE的特点是诊断延迟时间长、上气道频繁受累且发作期病程良性。与慢性荨麻疹一样，可能需要试用高达4倍剂量的H1抗组胺药来控制症状。
