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首页> 外文期刊>Clinical and Experimental Immunology: An Official Journal of the British Society for Immunology >Idiopathic histaminergic angioedema without wheals: a case series of 31 patients
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Idiopathic histaminergic angioedema without wheals: a case series of 31 patients

机译：不伴风湿性的特发性组胺能性血管性水肿：31例病例

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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 63 years. A history of allergy was found in 12 patients (387%), nine suffering from allergic rhinitis. The mean duration of attacks was 281 h. The AE attack was located in the upper respiratory tract in 548% 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 516% of patients (16 patients), twofold in 32% (10 patients) and three-fourfold in 161% (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.
机译：特发性组织胺能性获得性血管性水肿（IH-AAE）是复发性血管性水肿的常见原因，无任何风疹。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。这项研究的目的是描述IH-AAE患者的临床和流行病学特征。从2014年到2015年，在我们国家血管性水肿和/或荨麻疹的参考中心就诊了534名患者。其中，我们确定了31例特发性组织胺能性获得性无浮肿的血管性水肿（IH-AAE）患者。平均年龄为50岁的31例患者（15例男性和16例女性）符合IH-AAE标准。诊断的平均延迟为63年。在12名患者中发现了过敏史（387％），其中9名患有变应性鼻炎。平均攻击时间为281小时。 AE发作位于548％的病例（17例）中位于上呼吸道。在29％的患者中发现了舌侧位置。男性比女性更可能累及上呼吸道。没有关于插管或重症监护病房入院的报道。用于控制症状的抗组胺药的剂量在516％的患者（16例患者）中是推荐剂量的两倍，在32％（10例患者）中是两倍，在161％（5例患者）中是三倍。 IH-AAE的特点是诊断严重延迟，上呼吸道频繁受累以及发作期间的良性病程。与在CU中一样，为控制症状可能需要多达四倍剂量的H1-抗组胺药的试验。

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