Acquired angioedema associated with primary antiphospholipid syndrome in a patient with antithrombin III deficiency.
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.
Széplaki
Gábor
G
Third Department of Internal Medicine, Faculty of Medicine, Semmelweis University, Budapest, Hungary.
Varga
Lilian
L
Szépvölgyi
Anikó
A
Simon
Kornél
K
Blaskó
Bernadett
B
Nagy
Eszter
E
Karádi
István
I
Füst
George
G
Farkas
Henriette
H
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
D050718:Complement C1 Inhibitor Protein