Naslov
Hereditary and non-hereditary angioedema

Autori
Peternel, Sandra

Vrsta, podvrsta i kategorija rada
Sažeci sa skupova, neobjavljeni rad, stručni

Izvornik
European Academy of Allergy and Clinical Immunology - Skin Allergy Club / - Zürich, 2012

Skup
European Academy of Allergy and Clinical Immunology - Skin Allergy Club

Mjesto i datum
Zürich, Švicarska, 24.03.2012. - 25.03.2012

Vrsta sudjelovanja
Predavanje

Vrsta recenzije
Međunarodna recenzija

Ključne riječi
Angioedema; Bradykinin; C1 esterase inhibitor; Ecallantide; Icatibant

Sažetak
Angioedema is characterized by attack(s) of submucosal or subcutaneous swellings affecting various locations (face, genitalia, extremities, gastrointestinal tract and larynx). These commonly occur in association with urticaria, and usually respond well to the use of antihistamines and corticosteroids. In contrast, recurrent attacks of angioedema without urticaria are mediated by excessive levels of bradykinin, and therefore, do not respond to the aforementioned therapy. These bradykinin-induced attacks of angioedema can be associated with hereditary or acquired deficiency of the C1 esterase inhibitor, mutation of FXII, use of angiotensin-converting enzyme inhibitors, or may occur without identifiable cause (idiopathic). Hereditary angioedema is a rare genetic disorder resulting from an inherited deficiency (type I, 85% of cases) or dysfunction (type II, 15% of cases) of the C1 inhibitor. It usually presents in late childhood or adolescence, with familial history positive in approximately 75% of cases. Angioedema with acquired C1 inhibitor deficiency usually develops in older patients with B-cell lymphoproliferative or autoimmune disease. Management of hereditary angioedema with C1 inhibitor deficiency has advanced in the recent years. It involves both prophylactic strategies to prevent attacks of angioedema (trigger avoidance, attenuated androgens, tranexamic acid, C1 inhibitor replacement therapy) as well as use of drugs in the treatment of acute attacks (C1 inhibitor replacement therapy, ecallantide and icatibant). In patients with acquired C1 inhibitor deficiency, control of the underlying disease generally results in reduced severity of angioedema attacks. Treatment of acute attacks is the same as in hereditary form, although the needed dose of C1 inhibitor may be higher.

Izvorni jezik
Engleski

Znanstvena područja
Kliničke medicinske znanosti

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