John Libbey Eurotext

European Journal of Dermatology

Hereditary angioedema with normal C1 inhibitor: clinical characteristics and treatment response with plasma-derived human C1 inhibitor concentrate (Berinert®) in a French cohort Volume 27, numéro 2, March-April 2017

Illustrations

  • Figure 1

Tableaux

Auteurs
1 Univ. Grenoble Alpes,
Joint unit 1036 INSERM-CEA-CNRS,
CREAK,
Department of Internal Medicine,
PPMGC,
CHU Grenoble,
Grenoble,
France
2 National Reference Centre for Angioedema,
CREAK,
France
3 Endocrine Gynaecology, Paris Descartes University, University Hospital Centre, Paris, France
4 Intensive Care Unit,
Edouard Herriot Hospital,
Hospices Civils de Lyon,
Lyon,
France
5 Department of Dermatology,
CHU Angers,
Angers,
France
6 Department of Internal Medicine,
CH Niort,
France
7 Université Lille Nord de France,
Department of Internal Medicine,
National Reference Centre for Autoimmune and Systemic Rare Diseases,
Hospital Claude-Huriez,
CHRU Lille,
France
8 Department of Internal Medicine,
Saint Antoine University Hospital,
AP-HP,
Paris,
France
* Reprints
  • Mots-clés : Berinert®, hereditary angioedema, pharmaco-epidemiological, C1 inhibitor, drug safety, registry, off label
  • DOI : 10.1684/ejd.2016.2948
  • Page(s) : 155-9
  • Année de parution : 2017

Background

Hereditary angioedema (HAE) is a rare genetic disorder characterised by episodes of swelling without urticaria. Berinert® (CSL Behring) is a plasma-derived human C1 inhibitor (C1-INH) concentrate, approved for the treatment of HAE with C1-INH deficiency (C1-INH-HAE), however, it is often used off-label in Europe to treat HAE with normal C1-INH.