Hereditary angioedema (HAE) with C1-inhibitor deficiency belongs to bradykinin-mediated angioedemas. It is characterized by recurrent subcutaneous and/or submucosal swelling episodes (HAE attacks) and erythema marginatum skin rash as a pre-attack (prodromal) phase. HAE attacks were shown to be accompanied by peripheral blood neutrophilia. We aimed to find molecular mechanisms that may explain the distinct role of neutrophil granulocytes in HAE. Plasma levels of blood cells and factors related to neutrophil activation (cytokines, chemokines, chemotactic factors, enzymes, and neutrophil extracellular trap) were measured in plasma samples obtained from patients during symptom-free periods (n = 77), during prodromal phase (n = 8) and attacks (n = 14), during a spontaneously resolved attack (n = 1), and in healthy controls (n = 79). Higher counts of white blood cells, lymphocytes, and neutrophil granulocytes were found in symptom-free patients compared with controls; these cell counts were elevated further during HAE attacks. The level of chemokine (C–C motif) ligand 5, monocyte chemoattractant protein-1, and myeloperoxidase were also higher in the symptom-free patients than in the controls. Levels of monocyte chemoattractant protein-1, leukotriene B4, neutrophil elastase, and myeloperoxidase were elevated during attacks. During erythema marginatum, white blood cells and monocyte count and levels of interleukin 8 were elevated compared with symptom-free period. Similar changes were detected during the attack follow-up. We conclude that the activation of NGs in symptom-free periods and a further increase observed during attacks suggests that NGs may be involved in the pathomechanism of HAE with C1-INH deficiency.

C1 抑制剂缺乏的遗传性血管性水肿 (HAE) 属于缓激肽介导的血管性水肿。它的特点是复发性皮下和/或粘膜下肿胀发作（HAE 发作）和边缘红斑皮疹作为发作前（前驱）阶段。HAE 发作被证明伴有外周血中性粒细胞增多症。我们旨在寻找可以解释中性粒细胞在 HAE 中的独特作用的分子机制。在无症状期（n  = 77）、前驱期（n  = 8) 和攻击 ( n = 14)、自发缓解的发作期间 ( n  = 1) 和健康对照 ( n = 79）。与对照组相比，无症状患者的白细胞、淋巴细胞和中性粒细胞计数更高；在 HAE 攻击期间，这些细胞计数进一步升高。无症状患者的趋化因子（C-C 基序）配体 5、单核细胞趋化蛋白 1 和髓过氧化物酶的水平也高于对照组。单核细胞趋化蛋白 1、白三烯 B4、中性粒细胞弹性蛋白酶和髓过氧化物酶的水平在发作期间升高。在边缘性红斑期间，与无症状期相比，白细胞和单核细胞计数以及白细胞介素 8 水平升高。在攻击后续过程中检测到类似的变化。