title: The safety of treatments for angioedema with hereditary C1 inhibitor deficiency.
abstract: BACKGROUND
  Angioedema is a localized and self-limiting edema of the subcutaneous and submucosal tissue. Hereditary angioedema with C1 inhibitor deficiency (C1-INH-HAE) is the best characterized form of hereditary angioedema. In C1-INH-HAE, the reduced plasma levels of C1-INH cause instability of the contact system with release of bradykinin, the key mediator of angioedema. C1-INH-HAE is characterized by recurrent skin swelling, abdominal pain, and potentially life-threatening upper airways obstruction. Knowledge of the molecular mechanisms leading from C1-INH deficiency to angioedema allowed the development of several therapies.
  
  
  METHODS
  The aim of this review article is to discuss the safety of currently available treatments of C1-INH-HAE. The authors give an insight on the mechanism of action and safety profile of drugs for treatment of acute attacks and for short- and long-term prophylaxis. Evidence from systematic reviews, clinical trials, retrospective studies, and case reports is summarized in this review.
  
  
  CONCLUSIONS
  C1-INH-HAE is a disabling, life-threatening condition that lasts life-long. Different therapeutic approaches with different drugs provide significant benefit to patients. Safety profiles of these therapies are critical for optimal therapeutic decision and need to be known by C1-INH-HAE treating physicians for appropriate risk/benefit evaluation.
authors.affiliation: a University of Milan, Luigi Sacco Hospital, Department of Biomedical and Clinical Sciences "Luigi Sacco" , Milan, Italy +39 02 50 31 98 29 ; +39 02 50 31 98 28 ; marco.cicardi@unimi.it.
authors.forename: Andrea
authors.initials: A
authors.lastname: Zanichelli
authors.affiliation: a University of Milan, Luigi Sacco Hospital, Department of Biomedical and Clinical Sciences "Luigi Sacco" , Milan, Italy +39 02 50 31 98 29 ; +39 02 50 31 98 28 ; marco.cicardi@unimi.it.
authors.forename: Maddalena Alessandra
authors.initials: MA
authors.lastname: Wu
authors.affiliation: a University of Milan, Luigi Sacco Hospital, Department of Biomedical and Clinical Sciences "Luigi Sacco" , Milan, Italy +39 02 50 31 98 29 ; +39 02 50 31 98 28 ; marco.cicardi@unimi.it.
authors.forename: Arnaldo
authors.initials: A
authors.lastname: Andreoli
authors.affiliation: a University of Milan, Luigi Sacco Hospital, Department of Biomedical and Clinical Sciences "Luigi Sacco" , Milan, Italy +39 02 50 31 98 29 ; +39 02 50 31 98 28 ; marco.cicardi@unimi.it.
authors.forename: Marta
authors.initials: M
authors.lastname: Mansi
authors.forename: Marco
authors.initials: M
authors.lastname: Cicardi
mesh_terms: D015746:Abdominal Pain / Q000209:etiology; D054179:Angioedemas, Hereditary / Q000188:drug therapy* / Q000503:physiopathology; D000818:Animals; D001920:Bradykinin / Q000378:metabolism*; D050718:Complement C1 Inhibitor Protein / Q000378:metabolism; D006801:Humans; D013997:Time Factors
chemical_list: D050718:Complement C1 Inhibitor Protein; D001920:Bradykinin
keywords: C1 inhibitor; androgens; angioedema; ecallantide; icatibant; tranexamic acid