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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 (<jats:italic>n<\/jats:italic>\u2009=\u200977), during prodromal phase (<jats:italic>n<\/jats:italic>\u2009=\u20098) and attacks (<jats:italic>n<\/jats:italic>\u2009=\u200914), during a spontaneously resolved attack (<jats:italic>n<\/jats:italic>\u2009=\u20091), and in healthy controls (<jats:italic>n<\/jats:italic>\u2009=\u200979). 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\u2013C 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.<\/jats:p>","DOI":"10.1007\/s12016-021-08847-4","type":"journal-article","created":{"date-parts":[[2021,2,19]],"date-time":"2021-02-19T21:45:16Z","timestamp":1613771116000},"page":"383-395","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":10,"title":["Pathways of Neutrophil Granulocyte Activation in Hereditary Angioedema with C1 Inhibitor Deficiency"],"prefix":"10.1007","volume":"60","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-0739-2193","authenticated-orcid":false,"given":"Erika","family":"Kajd\u00e1csi","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7647-2173","authenticated-orcid":false,"given":"N\u00f3ra","family":"Veszeli","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9750-1682","authenticated-orcid":false,"given":"Blanka","family":"Mez\u0151","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Zs\u00f3fia","family":"Jandrasics","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7381-6866","authenticated-orcid":false,"given":"Kinga Vikt\u00f3ria","family":"K\u0151halmi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1839-8873","authenticated-orcid":false,"given":"Anne Lise","family":"Ferrara","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0166-8697","authenticated-orcid":false,"given":"L\u00e1szl\u00f3","family":"Cervenak","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5484-364X","authenticated-orcid":false,"given":"Lilian","family":"Varga","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Henriette","family":"Farkas","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2021,2,19]]},"reference":[{"issue":"12","key":"8847_CR1","doi-asserted-by":"publisher","first-page":"1136","DOI":"10.1056\/NEJMra1808012","volume":"382","author":"PJ Busse","year":"2020","unstructured":"Busse PJ, Christiansen SC (2020) Hereditary angioedema. 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LV received travel grants from CSL Behring and Shire Human Genetic Therapies Inc. HF received honoraria and travel grants from CSL Behring, Shire\/Takeda, Swedish Orphan Biovitrum, Octapharma, Kalvista and Pharming; and\/or served as a consultant for these companies and has participated in clinical trials\/registries for BioCryst, CSL Behring, Pharming, and Shire. Other authors have declared no conflict of interest.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Conflict of Interest"}}]}}