Department of Internal Medicine, University Hospital, Grenoble, France.
Ann Allergy Asthma Immunol. 2013 Oct;111(4):290-4. doi: 10.1016/j.anai.2013.07.012. Epub 2013 Aug 6.
Hereditary angioedema (HAE) is a rare and potentially life-threatening disease. New specific treatments are available.
To identify patients' features and patients' best therapeutic option.
A 1-year, multicenter, retrospective study was performed. The primary objective was to examine the clinical presentation of HAE. Secondary objectives included patient characteristics, management of HAE over 12 months, and health-related quality of life using the SF-36v2 questionnaire.
One hundred ninety-three patients were included, and 69.4% were women. In the 12-month period, the mean number of HAE attacks was 7.6. Among the 568 reported attacks, localizations were the abdomen (57.1%), peripheral limbs (42.5%), upper airway (7.9%), and face (6.9%); 31.6% of attacks were severe and occurred statistically more often in women (P < .02). Compared with a population of allergic patients, all age- and sex-adjusted scores were significantly lower in patients with HAE (P < .05) except for the physical component summary. Health-related quality of life negatively correlated with the annual number of attacks and was markedly altered for patients having more than 5 attacks per year (P < .05 for all dimensions).
HAE is a severe disease that places a heavy burden on quality of life.
遗传性血管性水肿（HAE）是一种罕见且可能危及生命的疾病。目前已有新的特效治疗方法。
明确患者的特征和最佳治疗选择。
一项为期 1 年的多中心回顾性研究。主要目的是研究 HAE 的临床表现。次要目的包括患者特征、12 个月内 HAE 的管理以及使用 SF-36v2 问卷评估健康相关生活质量。
共纳入 193 例患者，其中 69.4%为女性。在 12 个月期间，HAE 发作的平均次数为 7.6 次。在报告的 568 次发作中，定位为腹部（57.1%）、外周肢体（42.5%）、上呼吸道（7.9%）和面部（6.9%）；31.6%的发作严重，女性中发生率更高（P <.02）。与过敏患者人群相比，HAE 患者的所有年龄和性别调整评分均明显较低（P <.05），除生理成分综合评分外。健康相关生活质量与每年发作次数呈负相关，每年发作次数超过 5 次的患者健康相关生活质量明显改变（所有维度 P <.05）。
HAE 是一种严重的疾病，严重影响生活质量。
