Gr T Popa University of Medicine and Pharmacy Iaşi, Department of Medicine II-Pulmonary Disease, Pulmonary Disease University Hospital, Iaşi, Romania.
Clin Rev Allergy Immunol. 2011 Aug;41(1):114-22. doi: 10.1007/s12016-011-8254-2.
Hereditary angioedema (HAE) is characterized by acute attacks of edema with multiple localizations, the laryngeal angioedema being the most potentially lethal. In HAE, C1-INH impairments cause episodic increase in kallikrein activity leading to attacks of angioedema. Several therapies have recently become available to treat or to prevent HAE attacks, and others are under evaluation for this indication. Plasma-derived C1-INH, bradykinin receptor antagonists (icatibant), kallikrein inhibitors (ecallantide), or recombinant C1-INH is authorized on the market for HAE attack therapy or prophylaxis. Some of these compounds can be used exclusively to treat HAE attacks, whereas others can also be used as prophylactic therapies. Such therapies, although not available worldwide, can improve disease outcome due to their different mechanisms of action.
遗传性血管性水肿 (HAE) 的特征是急性水肿发作，具有多种定位，其中喉血管性水肿最具潜在致命性。在 HAE 中，C1-INH 功能障碍导致激肽释放酶活性的阵发性增加，导致血管性水肿发作。最近有几种治疗方法可用于治疗或预防 HAE 发作，还有其他方法正在为此适应症进行评估。市场上有用于 HAE 发作治疗或预防的血浆衍生 C1-INH、缓激肽受体拮抗剂（依卡替班）、激肽释放酶抑制剂（艾卡替班）或重组 C1-INH。这些化合物中的一些可专门用于治疗 HAE 发作，而另一些也可作为预防性治疗。由于这些药物具有不同的作用机制，因此尽管并非在全球范围内都可获得，但它们可以改善疾病结局。
