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- Title
Acquired Angioedema Associated with Primary Antiphospholipid Syndrome in a Patient with Antithrombin III Deficiency.
- Authors
Széplaki, Gábor; Varga, Lilian; Szépvölgyi, Anikó; Simon, Kornél; Blaskó, Bernadett; Nagy, Eszter; Karádi, István; Füst, George; Farkas, Henriette
- Abstract
Acquired angioedema (AAE) due to the functional deficiency of the C1 inhibitor (C1-INH) is a rare disease characterized by recurrent bouts of edema that involve subcutaneous tissues, the larynx or the gastrointestinal tract. In the present paper, we report the case of a male patient with symptoms of AAE and recurrent deep venous and arterial thrombosis. As a trigger of AAE in the present patient, we revealed primary antiphospholipid syndrome accompanied by antithrombin III deficiency, along with malignancy in the history, and angiotensin-converting enzyme inhibitor therapy. Although anti-C1-INH titers (type I AAE) were normal initially, we observed a sharp increase in anti-C1-INH titers (suggestive of type II AAE) during follow-up. It seems that thrombosis might worsen angioedematous attacks in functional C1-INH deficiency. Thrombophilia should be considered a provoking factor of AAE and should be carefully sought for in these patients, as the key to successful management of AAE is the effective treatment of the underlying disease. Copyright © 2008 S. Karger AG, Basel
- Subjects
ANGIONEUROTIC edema; EDEMA; LARYNX; GASTROINTESTINAL system; THROMBOSIS; BODY fluid disorders
- Publication
International Archives of Allergy & Immunology, 2008, Vol 146, Issue 2, p164
- ISSN
1018-2438
- Publication type
Article
- DOI
10.1159/000113521