title: Acquired angioedema associated with primary antiphospholipid syndrome in a patient with antithrombin III deficiency.
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.
authors.affiliation: Third Department of Internal Medicine, Faculty of Medicine, Semmelweis University, Budapest, Hungary.
authors.forename: Gábor
authors.initials: G
authors.lastname: Széplaki
authors.forename: Lilian
authors.initials: L
authors.lastname: Varga
authors.forename: Anikó
authors.initials: A
authors.lastname: Szépvölgyi
authors.forename: Kornél
authors.initials: K
authors.lastname: Simon
authors.forename: Bernadett
authors.initials: B
authors.lastname: Blaskó
authors.forename: Eszter
authors.initials: E
authors.lastname: Nagy
authors.forename: István
authors.initials: I
authors.lastname: Karádi
authors.forename: George
authors.initials: G
authors.lastname: Füst
authors.forename: Henriette
authors.initials: H
authors.lastname: Farkas
mesh_terms: D000368:Aged; D000799:Angioedema / Q000145:classification / Q000175:diagnosis / Q000276:immunology*; D016736:Antiphospholipid Syndrome / Q000150:complications / Q000175:diagnosis / Q000276:immunology*; D020152:Antithrombin III Deficiency / Q000150:complications / Q000175:diagnosis / Q000276:immunology*; D050718:Complement C1 Inhibitor Protein / Q000235:genetics; D003937:Diagnosis, Differential; D006801:Humans; D008297:Male; D012008:Recurrence; D019851:Thrombophilia / Q000150:complications / Q000175:diagnosis / Q000276:immunology
chemical_list: D050718:Complement C1 Inhibitor Protein