title: Distinct conditions support a novel classification for bradykinin-mediated angio-oedema.
abstract: BACKGROUND
  Angio-oedema (AO) can be attributable to bradykinin (BK) accumulation, as is the case for prototypical hereditary AO (HAO) due to C1 inhibitor (C1-INH) deficiency. However, our clinical experience in a reference centre has shown that some patients display a clinical history suggestive of HAO, but exhibit normal C1-INH function, have no mutation in the causative genes associated with HAO (SERPING1, F12), and report no intake of drugs known to promote AO.
  
  
  OBJECTIVE
  We sought to determine the frequency and distribution of different AO subtypes suspected to be BK-mediated AO (BK-AO) and defined by clinical, history and biological criteria (enzyme activities implicated in BK formation and catabolism).
  
  
  METHODS
  The files of all patients referred to our centre for suspected BK-AO were retrospectively analysed.
  
  
  RESULTS
  The distribution of patients (n = 162) was 16 and 4% with a hereditary deficiency of C1-INH or a gain of factor XII function, respectively, 29% with iatrogenic BK-AO, 21% with non-iatrogenic defective kininase activity and 30% with idiopathic increased kinin formation.
  
  
  CONCLUSIONS
  BK-AO may be caused by multiple inherited or acquired factors triggering BK accumulation. Therefore, we propose a novel typology for BK-AO based on the imbalance of production/catabolism of BK.
authors.affiliation: Department of Dermatology, Angers Hospital, L'UNAM University, Angers, France.
authors.forename: Panteha
authors.initials: P
authors.lastname: Dessart
authors.forename: Federica
authors.initials: F
authors.lastname: Defendi
authors.forename: Hélène
authors.initials: H
authors.lastname: Humeau
authors.forename: Brigitte
authors.initials: B
authors.lastname: Nicolie
authors.forename: Marie-Elodie
authors.initials: ME
authors.lastname: Sarre
authors.forename: Delphine
authors.initials: D
authors.lastname: Charignon
authors.forename: Denise
authors.initials: D
authors.lastname: Ponard
authors.forename: Sven
authors.initials: S
authors.lastname: Cichon
authors.forename: Christian
authors.initials: C
authors.lastname: Drouet
authors.forename: Ludovic
authors.initials: L
authors.lastname: Martin
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000581:Amidohydrolases / Q000378:metabolism; D000626:Aminopeptidases / Q000235:genetics / Q000378:metabolism; D000799:Angioedema / Q000145:classification* / Q000209:etiology / Q000378:metabolism*; D057911:Angiotensin Receptor Antagonists / Q000009:adverse effects; D000806:Angiotensin-Converting Enzyme Inhibitors / Q000009:adverse effects; D001920:Bradykinin / Q000378:metabolism*; D002648:Child; D002675:Child, Preschool; D003174:Complement C1 Inactivator Proteins / Q000235:genetics; D050718:Complement C1 Inhibitor Protein / Q000378:metabolism*; D005174:Factor XII / Q000235:genetics; D005260:Female; D056829:Hereditary Angioedema Types I and II / Q000150:complications / Q000201:enzymology / Q000235:genetics; D006728:Hormones / Q000009:adverse effects; D006801:Humans; D001121:Lysine Carboxypeptidase / Q000378:metabolism; D008297:Male; D008875:Middle Aged; D007703:Peptidyl-Dipeptidase A / Q000378:metabolism; D020641:Polymorphism, Single Nucleotide; D012008:Recurrence; D012189:Retrospective Studies; D014581:Urticaria / Q000209:etiology; D055815:Young Adult
chemical_list: D057911:Angiotensin Receptor Antagonists; D000806:Angiotensin-Converting Enzyme Inhibitors; D003174:Complement C1 Inactivator Proteins; D050718:Complement C1 Inhibitor Protein; D006728:Hormones; C469952:SERPING1 protein, human; D005174:Factor XII; D000626:Aminopeptidases; C022750:X-Pro aminopeptidase; D007703:Peptidyl-Dipeptidase A; D001121:Lysine Carboxypeptidase; D000581:Amidohydrolases; C019665:amidase; D001920:Bradykinin