[Pathogenesis of hereditary angioedema]

The hereditary angioedema (HAE) is an autosomal dominant transduced illness. Patients suffer from severe attacks with circumscribed swellings of the skin, or of the gastro-intestinal mucosa, or of whole organs. Laryngeal edema is responsible for airway obstruction and often for sudden death. Very often the abdominal symptoms are leading to false diagnosis and treatment. The diagnosis is proved by estimation of lowered C1-inhibitor (C1-INH) activity. The defect of C1-INH is responsible for the activation of the start phases of the complement system and of the kinin system. The liberation of vasoactive peptides and kinins induces the edematous swellings and severe pain. The acute symptoms of HAE are promptly resolved by intravenous application of C1-inhibitor concentrate.