title: Activation of the complement, coagulation, fibrinolytic and kallikrein-kinin systems during attacks of hereditary angioedema.
abstract: Five patients with hereditary angioedema (HAE) were studied during attacks and remission as were healthy controls. The high levels of C1/C1-INH complexes, low C4 and high ratio C4 activation products (C4bc)/C4 also differed significantly during remission compared to controls. During attacks C4bc/C4 increased (922-2007; P = 0.022, remission versus attacks, median values throughout), C2 and CH50 dropped (111-31%; P = 0.043 and 110-36%; P = 0.016, respectively), TCC (C5b-9) increased (0.88-1.23 AU/ml; P = 0.028). Cleavage of HK increased to be almost complete during attacks (20-90%; P = 0.009). While factor XIa/serpin-complexes did not increase, a more than twofold rise in thrombin/antithrombin-complexes (0.20-0.50 microgram/l; P = 0.009) and in plasmin/alpha-2-antiplasmin-complexes (7.3-17 nmol/l; P = 0.028) was observed. For the first time cascade activation in HAE was studied simultaneously, and corroborates that attacks lead to activation of the kallikrein-kinin system, fibrinolysis and early part of the classical complement pathway. In addition, the authors present novel data of terminal complement and coagulation activation, the latter apparently not via FXIa.
authors.affiliation: Department of Anaesthesiology, Nordland Central Hospital, Bodø, Norway.
authors.forename: E W
authors.initials: EW
authors.lastname: Nielsen
authors.forename: H T
authors.initials: HT
authors.lastname: Johansen
authors.forename: K
authors.initials: K
authors.lastname: Høgåsen
authors.forename: W
authors.initials: W
authors.lastname: Wuillemin
authors.forename: C E
authors.initials: CE
authors.lastname: Hack
authors.forename: T E
authors.initials: TE
authors.lastname: Mollnes
mesh_terms: D000799:Angioedema / Q000097:blood / Q000276:immunology*; D001777:Blood Coagulation; D003167:Complement Activation; D003176:Complement C3 / Q000378:metabolism; D003182:Complement C5 / Q000378:metabolism; D005342:Fibrinolysis; D006801:Humans; D007610:Kallikreins / Q000378:metabolism*; D007705:Kinins / Q000378:metabolism*
chemical_list: D003176:Complement C3; D003182:Complement C5; D007705:Kinins; D007610:Kallikreins