Acquired angioedema associated with primary antiphospholipid syndrome in a patient with antithrombin III deficiency

International Archives of Allergy and Immunology
Gábor SzéplakiHenriette Farkas

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.

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Citations

Mar 14, 2013·Expert Opinion on Pharmacotherapy·Henriette Farkas
Dec 31, 2009·The Journal of Rheumatology·Menachem Rottem, Reuven Mader
Dec 14, 2019·Clinical Reviews in Allergy & Immunology·Federica DefendiChantal Dumestre-Pérard
Jun 9, 2018·Allergy, Asthma, and Clinical Immunology : Official Journal of the Canadian Society of Allergy and Clinical Immunology·Arturo J BonninG Wendell Richmond
Nov 21, 2020·Journal of Internal Medicine·Zs PólaiH Farkas
Aug 7, 2021·Clinical Immunology : the Official Journal of the Clinical Immunology Society·Yiyun Shi, Chen Wang

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