Hereditary and acquired angioedemas : rare but potentially life-threatening

HAE, AAE and ACE inhibitor-induced angioedema are rare forms of angioedema that should be considered in patients with angioedema without urticaria. Although angioedema associated with allergic or chronic idiopathic urticaria generally resolves with antihistamine treatment of the associated urticaria, these other forms have no or poor response to antihistamines, corticosteroids and adrenaline, and require other management. In patients with HAE, danazol and tranexamic acid are appropriate for prophylaxis, and C1 INH concentrates (expensive and not PBS listed), icatibant (PBS listed) and ecallantide (not available in Australia) are appropriate for the treatment of severe episodes. In patients with AAE, treatment of the underlying disease generally relieves symptoms. In patients with ACE inhibitor-induced angioedema, withdrawal of the ACE inhibitor and supportive management is appropriate, with intubation if necessary. Angiotensin receptor antagonists may be used as an alternative when ACE inhibitors are withdrawn.