Introduction: 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.Areas covered: 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.Expert opinion: 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.
The safety of treatments for angioedema with hereditary C1 inhibitor deficiency / A. Zanichelli, M.A. Wu, A. Andreoli, M. Mansi, M. Cicardi. - In: EXPERT OPINION ON DRUG SAFETY. - ISSN 1474-0338. - 14:11(2015), pp. 1725-1736.
|Titolo:||The safety of treatments for angioedema with hereditary C1 inhibitor deficiency|
ZANICHELLI, ANDREA [Writing – Original Draft Preparation]
WU, MADDALENA ALESSANDRA [Writing – Review & Editing]
MANSI, MARTA [Investigation]
|Parole Chiave:||androgens; angioedema; C1 inhibitor; ecallantide; icatibant; tranexamic acid; Abdominal Pain; Angioedemas, Hereditary; Animals; Bradykinin; Complement C1 Inhibitor Protein; Humans; Time Factors; Pharmacology (medical)|
|Settore Scientifico Disciplinare:||Settore MED/09 - Medicina Interna|
|Data di pubblicazione:||2015|
|Digital Object Identifier (DOI):||http://dx.doi.org/10.1517/14740338.2015.1094053|
|Appare nelle tipologie:||01 - Articolo su periodico|