| Summary: | Hereditary angioedema (HAE) is an autosomal dominant disorder, mostly due to C1 esterase inhibitor (C1-INH) deficiency, known by recurring angioedema attacks that are nonpruritic, not accompanying with urticaria, and involve the dermis, intestinal submucosa, and upper respiratory system. The angioedema attacks are not responsive to epinephrine, glucocorticoids, or antihistamine treatments. Whereas HAE patients formerly had a few therapeutic options accessible such as anabolic androgens and antifibrinolytics. Nowadays in many parts of the world there has been remarkable progress in HAE treatment for the last two decades and clinically confirmed medications are presented for prophylactic and attack treatment. Alternatives in attack therapy contain two plasma-derived C1-INH concentrates, a recombinant C1-INH product, a kallikrein inhibitor, and a bradykinin β2 receptor antagonist. Options in prophylactic therapy include other than two plasma-derived C1-INH concentrates, subcutaneous C1-INH replacement and newest subcutaneous plasma kallikrein inhibitor Lanadelumab. In spite of these progresses, HAE patients still run into some challenges of an arduous disorder that can yield to devastating angioedema attacks related with important expenses for patients and the public. Better education of HAE patients and implementation of the self-management policy for 'on-demand' therapy will recuperate patients' life quality and negative effects of the disease. Herein the existing and promising therapeutic options are reviewed in the HAE management.
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