Abstract
The review focused on managing of patients with hereditary angioedema by administering C1-INH concentrate therapy. Hereditary angioedema is an orphan disorder characterized by recurrent episodes of angioedema, usually localized to the skin/subcutaneous tissue or mucous membranes of the upper respiratory tract and gastrointestinal tract and does not respond to usual treatment with antihistamines, corticosteroids, or adrenaline. Suspicion of HAE І or HAE II should be the basis for requesting laboratory tests to confirm the diagnosis. Determination of the function of C1-INH, C1-INH protein and C4 in serum/plasma is used to diagnose HAE І or HAE II. Own clinical case of management of the patients with HAE was proposed. Typical complex therapy includes a number of measures to avoid triggers of exacerbations and the development of a clear drug treatment plan with the patient (treatment of the acute condition, short-term (pre-procedural) and long-term prophylaxis). The importance of a multidisciplinary approach in the management of the patients with HAE is an important step towards timely verification of this rare/orphan disease.