title: Hereditary angioedema with normal C1 inhibitor in a French cohort: Clinical characteristics and response to treatment with icatibant.
abstract: The clinical characteristics and icatibant-treatment outcomes of patients with hereditary angioedema with normal C1 inhibitor (HAE-nC1 INH) are limited.
  
  
  
  We retrospectively analyzed data from French HAE patients enrolled in the Icatibant Outcome Survey registry (from July 2009 to September 2013) to compare disease characteristics and the effectiveness and safety of acute icatibant-treated angioedema attacks in patients with HAE-nC1 INH, HAE with C1 INH deficiency (type I), or dysfunction (type II).
  
  
  
  One center in Grenoble contributed 22 patients with HAE-nC1 INH and a family history of HAE while 15 centers across France contributed 153 patients with HAE type I and seven patients with HAE type II. Patients with HAE-nC1 INH compared to HAE type I, respectively, were more likely to be female (88.1% vs. 63.4%), older at median age of disease onset (21 years vs. 15 years), and have a greater rate of abdominal (80% vs. 61%) and laryngeal (23% vs. 14%) attacks. Icatibant was effective in both groups though the median time to resolution of attack was significantly longer in the HAE-nC1 INH group (20.0 h, 37 attacks) versus the HAE type I group (14.0 h, 67 attacks). Icatibant was self-administered for 96.1% of attacks in patients with HAE-nC1 INH and 75.8% in patients with HAE type I. No serious adverse side effects related to icatibant were reported.
  
  
  
  These data help further define the disease characteristics of HAE-nC1 INH in the French population and extend the limited data reporting the safe and effective use of icatibant in acute treatment of angioedema in French patients diagnosed with HAE-nC1 INH.
authors.affiliation: Department of Internal Medicine National Reference Centre for Angioedema (CREAK) Université Grenoble Alpes, Joint Unit 1036 INSERM-CNRS-CEA CHU Grenoble France.
authors.forename: Laurence
authors.initials: L
authors.lastname: Bouillet
authors.affiliation: Department of Internal Medicine National Reference Centre for Angioedema (CREAK) Université Grenoble Alpes, Joint Unit 1036 INSERM-CNRS-CEA CHU Grenoble France.
authors.forename: Isabelle
authors.initials: I
authors.lastname: Boccon-Gibod
authors.affiliation: U995 Lille Inflammation Research International Center (LIRIC)University of LilleLilleFrance; Inserm, U995LilleFrance; département de médecine interne et immunologie cliniqueCHU LilleLilleFrance; National Reference Centre for Angioedema (CREAK)LilleFrance.
authors.forename: David
authors.initials: D
authors.lastname: Launay
authors.affiliation: Gynaecology and Endocrinology Unit Paris Descartes University, Cochin-Port Royal Hospital Paris France.
authors.forename: Anne
authors.initials: A
authors.lastname: Gompel
authors.affiliation: EA 7299 Internal Medicine, Clinical Immunology and Allergology Lorraine University, University Hospital Vandoeuvre-lès-Nancy France.
authors.forename: Gisele
authors.initials: G
authors.lastname: Kanny
authors.affiliation: Shire Zug Switzerland.
authors.forename: Vincent
authors.initials: V
authors.lastname: Fabien
authors.affiliation: Department of Internal Medicine Saint-Antoine Hospital (AP-HP) Paris 6 University, DHUi2B Paris France.
authors.forename: Oliver
authors.initials: O
authors.lastname: Fain
mesh_terms: D000328:Adult; D054179:Angioedemas, Hereditary / Q000188:drug therapy* / Q000378:metabolism; D001920:Bradykinin / Q000031:analogs & derivatives* / Q000627:therapeutic use; D065094:Bradykinin B2 Receptor Antagonists / Q000627:therapeutic use*; D050718:Complement C1 Inhibitor Protein / Q000378:metabolism*; D005260:Female; D005602:France; D006801:Humans; D008297:Male; D008875:Middle Aged; D016896:Treatment Outcome
chemical_list: D065094:Bradykinin B2 Receptor Antagonists; D050718:Complement C1 Inhibitor Protein; C469952:SERPING1 protein, human; C065679:icatibant; D001920:Bradykinin
keywords: hereditary angioedema; icatibant; normal C1 INH