International Archives of Allergy and Immunology
Novel Insights from Clinical Practice
Acquired Angioedema Associated with Primary Antiphospholipid Syndrome in a Patient with Antithrombin III DeficiencySzéplaki G.a · Varga L.a · Szépvölgyi A.c · Simon K.c · Blaskó B.a · Nagy E.b · Karádi I.a · Füst G.a · Farkas H.aaThird Department of Internal Medicine, Faculty of Medicine, and bCentral Laboratory of Immunology, Semmelweis University, Budapest, and cDepartment of Internal Medicine, Siófok City Hospital and Outpatient Clinic, Siófok, Hungary
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Article / Publication Details
Received: April 02, 2007
Accepted: October 17, 2007
Published online: January 18, 2008
Issue release date: May 2008
Number of Print Pages: 5
Number of Figures: 1
Number of Tables: 1
ISSN: 1018-2438 (Print)
eISSN: 1423-0097 (Online)
For additional information: https://www.karger.com/IAA
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.
© 2008 S. Karger AG, Basel
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Article / Publication Details
Received: April 02, 2007
Accepted: October 17, 2007
Published online: January 18, 2008
Issue release date: May 2008
Number of Print Pages: 5
Number of Figures: 1
Number of Tables: 1
ISSN: 1018-2438 (Print)
eISSN: 1423-0097 (Online)
For additional information: https://www.karger.com/IAA
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