title: Current treatment options for hereditary angioedema due to C1 inhibitor deficiency.
abstract: BACKGROUND
  Hereditary angioedema (HAE) usually results from C1 inhibitor (C1-INH) deficiency or dysfunction. It is a rare autosomal dominant disorder characterized by localized, non-pitting edema of the skin and submucosal tissues of the upper respiratory and gastrointestinal tracts, without significant wheals or pruritus, due to a temporary increase in vascular permeability. Other forms of HAE have been described, but therapies are approved only for HAE with C1-INH deficiency: hence, this review focuses on C1-INH-HAE.
  
  
  METHODS
  The aim of this review article is to present current available therapies for treatment of acute attacks as well as for short- and long-term prophylaxis of hereditary angioedema due to C1 inhibitor deficiency (C1-INH-HAE). The Authors highlight also critical issues on the management of C1-INH-HAE, which is continuously evolving thanks to evidence from clinical trials, post-marketing experience and ongoing studies.
  
  
  CONCLUSIONS
  In the last decade, the quality of life of C1-INH-HAE patients has significantly improved due to increased knowledge and awareness of the disease, improved patient support and major progress in pharmacotherapy. Ongoing research will probably provide patients with other new effective therapeutic agents in the near future.
authors.affiliation: a Department of Biomedical and Clinical Sciences "Luigi Sacco" , Luigi Sacco Hospital, University of Milan , Milan , Italy.
authors.forename: Maddalena Alessandra
authors.initials: MA
authors.lastname: Wu
authors.affiliation: a Department of Biomedical and Clinical Sciences "Luigi Sacco" , Luigi Sacco Hospital, University of Milan , Milan , Italy.
authors.forename: Andrea
authors.initials: A
authors.lastname: Zanichelli
authors.affiliation: a Department of Biomedical and Clinical Sciences "Luigi Sacco" , Luigi Sacco Hospital, University of Milan , Milan , Italy.
authors.forename: Marta
authors.initials: M
authors.lastname: Mansi
authors.affiliation: a Department of Biomedical and Clinical Sciences "Luigi Sacco" , Luigi Sacco Hospital, University of Milan , Milan , Italy.
authors.forename: Marco
authors.initials: M
authors.lastname: Cicardi
mesh_terms: D054179:Angioedemas, Hereditary / Q000188:drug therapy* / Q000517:prevention & control; D000933:Antifibrinolytic Agents / Q000627:therapeutic use; D065094:Bradykinin B2 Receptor Antagonists / Q000627:therapeutic use; D003174:Complement C1 Inactivator Proteins / Q000378:metabolism* / Q000627:therapeutic use; D050718:Complement C1 Inhibitor Protein; D019468:Disease Management; D006801:Humans; D010949:Plasma; D020842:Plasma Kallikrein / Q000037:antagonists & inhibitors; D011994:Recombinant Proteins / Q000627:therapeutic use
chemical_list: D000933:Antifibrinolytic Agents; D065094:Bradykinin B2 Receptor Antagonists; D003174:Complement C1 Inactivator Proteins; D050718:Complement C1 Inhibitor Protein; D011994:Recombinant Proteins; C469952:SERPING1 protein, human; D020842:Plasma Kallikrein
keywords: C1 inhibitor; angioedema; bradykinin; ecallantide; icatibant; kallikrein