title: Novel Vasoregulatory Aspects of Hereditary Angioedema: the Role of Arginine Vasopressin, Adrenomedullin and Endothelin-1.
abstract: The elevation of bradykinin (BK) level during attacks of hereditary angioedema due to C1-Inhibitor deficiency (C1-INH-HAE) is well known. We previously demonstrated that endothelin-1 (ET-1) level also increases during C1-INH-HAE attacks. Although BK and ET-1 are both potent vasoactive peptides, the vasoregulatory aspect of the pathomechanism of C1-INH-HAE has not yet been investigated. Hence we studied the levels of vasoactive peptides in controls and in C1-INH-HAE patients, as well as evaluated their changes during C1-INH-HAE attacks. The levels of arginine vasopressin (AVP), adrenomedullin (ADM) and ET-1 were measured in the plasma of 100 C1-INH-HAE patients in inter-attack periods and of 111 control subjects, using BRAHMS Kryptor technologies. In 18 of the 100 C1-INH-HAE patients, the levels of vasoactive peptides were compared in blood samples obtained during attacks, or in inter-attack periods. AVP, ADM and ET-1 levels were similar in inter-attack samples from C1-INH-HAE patients and in the samples of controls, although cardiovascular risk has an effect on the levels of vasoactive peptides in both groups. The levels of all three vasoactive peptides increased during C1-INH-HAE attacks. Moreover, the levels of ET-1 and ADM as well as their changes during attacks were significantly correlated. This study demonstrated that vascular regulation by vasoactive peptides is affected during C1-INH-HAE attacks. Our results suggest that the cooperation of several vasoactive peptides may be necessary to counterbalance the actions of excess BK, and to terminate the attacks. This may reveal a novel pathophysiological aspect of C1-INH-HAE.
authors.affiliation: 3rd Department of Internal Medicine, Semmelweis University, Kútvölgyi út 4., Budapest, H-1125, Hungary. kajdacsi.erika@med.semmelweis-univ.hu.
authors.forename: Erika
authors.initials: E
authors.lastname: Kajdácsi
authors.affiliation: 3rd Department of Internal Medicine, Semmelweis University, Kútvölgyi út 4., Budapest, H-1125, Hungary. jani.peter.k@gmail.com.
authors.forename: Péter K
authors.initials: PK
authors.lastname: Jani
authors.affiliation: 3rd Department of Internal Medicine, Semmelweis University, Kútvölgyi út 4., Budapest, H-1125, Hungary. csuka.dorottya@med.semmelweis-univ.hu.
authors.forename: Dorottya
authors.initials: D
authors.lastname: Csuka
authors.affiliation: 3rd Department of Internal Medicine, Semmelweis University, Kútvölgyi út 4., Budapest, H-1125, Hungary. varga.lilian@med.semmelweis-univ.hu.
authors.forename: Lilian
authors.initials: L
authors.lastname: Varga
authors.affiliation: 3rd Department of Internal Medicine, Semmelweis University, Kútvölgyi út 4., Budapest, H-1125, Hungary. prohoz@kut.sote.hu.
authors.forename: Zoltán
authors.initials: Z
authors.lastname: Prohászka
authors.affiliation: 3rd Department of Internal Medicine, Semmelweis University, Kútvölgyi út 4., Budapest, H-1125, Hungary. farkas.henriette@med.semmelweis-univ.hu.
authors.forename: Henriette
authors.initials: H
authors.lastname: Farkas
authors.affiliation: 3rd Department of Internal Medicine, Semmelweis University, Kútvölgyi út 4., Budapest, H-1125, Hungary. cervenak.laszlo@med.semmelweis-univ.hu.
authors.forename: László
authors.identifier: http://orcid.org/0000-0003-0166-8697
authors.initials: L
authors.lastname: Cervenak
mesh_terms: D053607:Adrenomedullin / Q000097:blood / Q000378:metabolism*; D000328:Adult; D054179:Angioedemas, Hereditary / Q000175:diagnosis / Q000378:metabolism*; D001127:Arginine Vasopressin / Q000097:blood / Q000378:metabolism*; D002318:Cardiovascular Diseases / Q000209:etiology; D016022:Case-Control Studies; D050718:Complement C1 Inhibitor Protein / Q000378:metabolism; D018450:Disease Progression; D019332:Endothelin-1 / Q000097:blood / Q000378:metabolism*; D005260:Female; D056829:Hereditary Angioedema Types I and II / Q000175:diagnosis / Q000378:metabolism; D006801:Humans; D008297:Male; D008875:Middle Aged; D012307:Risk Factors; D055815:Young Adult
chemical_list: D050718:Complement C1 Inhibitor Protein; D019332:Endothelin-1; D001127:Arginine Vasopressin; D053607:Adrenomedullin
keywords: Hereditary angioedema due to C1-Inhibitor deficiency; adrenomedullin; arginine vasopressin; endothelin-1; oedematous attack; vasoactive peptides