Abstract
Background Hereditary angioedema due to C1 inhibitor deficiency (C1-INH-HAE) is a rare disease characterized by recurrent episodes of cutaneous, abdominal and laryngeal edema. Bradykinin is the mediator of increased vascular permeability and edema formation. Treatment of HAE attacks in Italy is based on the administration of human C1 inhibitor or bradykin receptor antagonist. A recombinant human C1 inhibitor (rhC1INH) is marketed in Italy since 2012 for the treatment of HAE in adults. Safety and efficacy of rhC1INH were documented in several phase III trials. This analysis reports characteristics and treatment outcomes of HAE attacks treated with rhC1INH in a real-life setting.
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