Hereditary angioedema (HAE) is a rare genetic disease, characterized by recurrent and unexpected potentially life-threatening mucosal swelling. HAE may be further classified into HAE with C1‐inhibitor deficiency (C1‐INH‐HAE) and HAE with normal C1‐INH activity (nlC1‐INH‐HAE), mostly due to mutations leading to increased vascular permeability. Recent evidence implicates also the innate and adaptive immune responses in several aspects of angioedema pathophysiology. Monocytes/macrophages, granulocytes, lymphocytes, and mast cells contribute directly or indirectly to the pathophysiology of angioedema. Immune cells are a source of vasoactive mediators, including bradykinin, histamine, complement components, or vasoactive mediators, whose concentrations or activities are altered in both attacks and remissions of HAE. In turn, through the expression of various receptors, these cells are also activated by a plethora of molecules. Thereby, activated immune cells are the source of molecules in the context of HAE, and on the other hand, increased levels of certain mediators can, in turn, activate immune cells through the engagement of specific surface receptors and contribute to vascular endothelial processes that lead to hyperpemeability and tissue edema. In this review, we summarize recent developments in the putative involvement of the innate and adaptive immune system of angioedema.

遗传性血管性水肿 (HAE) 是一种罕见的遗传性疾病，其特征是反复出现和意外的潜在威胁生命的粘膜肿胀。HAE 可进一步分为 C1-抑制剂缺乏的 HAE（C1-INH-HAE）和 C1-INH 活性正常的 HAE（nlC1-INH-HAE），主要是由于突变导致血管通透性增加。最近的证据还涉及血管性水肿病理生理学的几个方面的先天性和适应性免疫反应。单核细胞/巨噬细胞、粒细胞、淋巴细胞和肥大细胞直接或间接促成血管性水肿的病理生理学。免疫细胞是血管活性介质的来源，包括缓激肽、组胺、补体成分或血管活性介质，其浓度或活性在 HAE 发作和缓解时都会发生改变。反过来，通过表达各种受体，这些细胞也被大量分子激活。因此，激活的免疫细胞是 HAE 背景下的分子来源，另一方面，某些介质水平的增加可以反过来通过特定表面受体的参与激活免疫细胞，并有助于导致血管内皮过程对高渗透性和组织水肿。在这篇综述中，我们总结了血管性水肿的先天性和适应性免疫系统的假定参与的最新进展。通过特定表面受体的参与激活免疫细胞，并促进导致高渗透性和组织水肿的血管内皮过程。在这篇综述中，我们总结了血管性水肿的先天性和适应性免疫系统的假定参与的最新进展。通过特定表面受体的参与激活免疫细胞，并促进导致高渗透性和组织水肿的血管内皮过程。在这篇综述中，我们总结了血管性水肿的先天性和适应性免疫系统的假定参与的最新进展。