Magerl M, Doumoulakis G, Kalkounou I, Weller K, Church M K, Kreuz W, Maurer M
Department of Dermatology and Allergy, Charité-Universitätsmedizin Berlin, Berlin, Germany.
Clin Exp Dermatol. 2014 Apr;39(3):298-303. doi: 10.1111/ced.12285.
Hereditary angio-oedema (HAE) due to C1-inhibitor (C1-INH) deficiency is a rare autosomal dominant disease. It predisposes affected patients to attacks of disfiguring, painful angio-oedema, which, in cases of involvement of the upper airways, can be life-threatening. Frequently, prodromal symptoms occur hours to days before an attack, but their predictive value is uncertain.
To characterize the spectrum of prodromal symptoms in patients with HAE in Germany.
A questionnaire asking about the frequency, type and time of occurrence of prodromal symptoms, and the interval until the onset of an attack of HAE was sent to 808 German patients with HAE. Answers from 365 participating patients were analysed.
The survey showed that 79% of patients with HAE had experienced ≥ 1 prodromal symptom before an attack of angio-oedema. The most commonly reported prodromal symptoms (67% of which occurred within 6 h before an attack) were fatigue, malaise and short temper. Significantly more women than men reported having prodromes (83% vs. 73%, P < 0.05). Over 90% of the patients with prodromes reported that they were able to predict the onset of an attack with a certainty of ≥ 50%. In addition, there was a significant correlation between the occurrence of skin rashes and delay in the diagnosis of HAE.
The results of this survey may aid the management of C1-HAE by recognizing that prodromal symptoms are of value in predicting the onset of an attack of angio-oedema and in diagnosing the condition.
由于C1抑制物（C1-INH）缺乏引起的遗传性血管性水肿（HAE）是一种罕见的常染色体显性疾病。它使受影响的患者易患毁容性、疼痛性血管性水肿发作，在上呼吸道受累的情况下，可能会危及生命。前驱症状通常在发作前数小时至数天出现，但其预测价值尚不确定。
描述德国HAE患者前驱症状的范围。
向808名德国HAE患者发送了一份问卷，询问前驱症状的发生频率、类型和时间，以及直到HAE发作的间隔时间。对365名参与患者的回答进行了分析。
调查显示，79%的HAE患者在血管性水肿发作前经历过≥1种前驱症状。最常报告的前驱症状（其中67%在发作前6小时内出现）是疲劳、不适和脾气暴躁。报告有前驱症状的女性明显多于男性（83%对73%，P<0.05）。超过90%有前驱症状的患者报告说他们能够以≥50%的确定性预测发作的开始。此外，皮疹的出现与HAE诊断延迟之间存在显著相关性。
这项调查的结果可能有助于C1-HAE的管理，因为认识到前驱症状在预测血管性水肿发作的开始和诊断该病方面具有价值。
