Kemp John G, Craig Timothy J
Department of Medicine, Section of Allergy, Asthma, and Immunology, Penn State University, College of Medicine, Hershey, Pennsylvania 17033, USA.
Allergy Asthma Proc. 2009 Sep-Oct;30(5):493-9. doi: 10.2500/aap.2009.30.3278.
Hereditary angioedema (HAE) is a rare disease caused by deficiency in the production or function of C1 inhibitor. It predisposes individuals to paroxysmal acute attacks causing painful, debilitating, disfiguring, and life-threatening angioedema. Prodromes occurring hours to days before attacks have been described in the literature; however, their significance as predictive signals of impending attacks is uncertain. Given the morbidity and mortality associated with HAE attacks and the increasing availability of therapeutic products for their treatment, identifying prodromes that accurately predict the onset of attacks could provide the basis for the development of a validated instrument to identify the onset of such attacks requiring abortive therapeutic intervention before the development of clinically significant angioedema. The aim of this study was to review the literature to identify the prodromes reported to occur with HAE attacks. A literature review of English language journal articles was performed using search terms hereditary angioedema, HAE, angioneurotic edema, prodrome, signs, and symptoms. Nineteen original English language articles that included both case reports and studies describing prodromes associated with HAE attacks were obtained. Our review indicates that there is significant variability in the expression, manifestation, prevalence, timing, and predictive reliability of the prodromes that have been described. There is considerable variability of the prodromal manifestations that may occur before or during HAE attacks. We have not found any evidence that their sensitivity and specificity for accurately predicting such attacks has been studied.
遗传性血管性水肿（HAE）是一种罕见疾病，由C1抑制剂产生或功能缺陷引起。它使个体易患阵发性急性发作，导致疼痛、使人衰弱、毁容且危及生命的血管性水肿。文献中已描述了发作前数小时至数天出现的前驱症状；然而，它们作为即将发作的预测信号的意义尚不确定。鉴于HAE发作相关的发病率和死亡率，以及用于治疗的治疗产品越来越多，识别能够准确预测发作开始的前驱症状可为开发一种经过验证的工具提供依据，该工具可识别在临床上显著的血管性水肿出现之前需要进行预防性治疗干预的此类发作的开始。本研究的目的是回顾文献，以识别据报道与HAE发作相关的前驱症状。使用搜索词遗传性血管性水肿、HAE、血管神经性水肿、前驱症状、体征和症状对英文期刊文章进行了文献综述。获得了19篇原创英文文章，其中包括病例报告和描述与HAE发作相关的前驱症状的研究。我们的综述表明，已描述的前驱症状在表达、表现、患病率、发作时间和预测可靠性方面存在显著差异。在HAE发作之前或期间可能出现的前驱表现存在相当大的差异。我们没有发现任何证据表明对它们准确预测此类发作的敏感性和特异性进行过研究。
