immunology allergic disorders
Hereditary and Acquired Angioedema
Prevention
Drugs used for long-term prophylaxis of hereditary angioedema episodes include Plasma-derivedC1 inhibitor(human) Lanadelumab Berotralstat Attenuated androgens Antifibrinolytics (eg,tranexamic acid) Plasma-derivedC1 inhibitormay be given in regular IV infusions or subcutaneous injections as long-term prophylaxis. Patients can be taught to self-administer. Plasma-derivedC1 inhibitoris approved for long-term prevention of hereditary angioedema in the US, but recombinantC1 esterase inhibitoris not. Lanadelumabis a recombinant humanized monoclonal antibody that binds to plasma kallikrein and blocks its activity. Usual dosage is 300 mg subcutaneously every 2 weeks. Berotralstatis a synthetic small molecule developed to inhibit plasma kallikrein. Dosage is 150 mg orally once a day Attenuated androgens (eg, stanozolol 2 mg orally 3 times a day,danazol200 mg orally 3 times a day) are used to stimulate hepaticC1 inhibitorsynthesis. This treatment may be less effective for the acquired form. Antifibrinolytics (eg,tranexamic acid) have been used as second-line drugs for long-term prophylaxis in children and pregnant women. (plasma-derived or recombinant) is available, some experts advocate giving it 1 hour before high-risk procedures rather than attenuated androgens for short-term prophylaxis. Plasma products (eg, 2 units of fresh frozen plasma) before procedures are also an option ( Prevention reference ). 1.Prematta M, Gibbs JG, Pratt EL: Fresh frozen plasma for the treatment of hereditary angioedema.Ann Allergy Asthma Immunol98 (4):383–388, 2007. 