Angioedema Due to Acquired Deficiency of C1-Inhibitor: a Cohort Study in Spain and a Comparison With Other series.

Background Data on acquired angioedema due to C1-inhibitor deficiency (C1-INH-AAE) from 4 European countries (France, Italy, Germany, and Hungary) were recently published. Objective To report data from a group of 50 patients with acquired C1-INH deficiency from Spain, of whom 46 had angioedema, and compare them to other European series. Methods We performed a retrospective observational study of 46 patients with C1-INH-AAE and 4 asymptomatic patients. Clinical and biological characteristics and associated diseases were assessed and compared to other European series. Results Women accounted for 73.9% of cases. The prevalence of C1-INH-AAE related to hereditary forms was 1/10.1. Overall, 8.7% patients were aged Conclusions This study emphasizes the possibility of C1-INH-AAE in patients younger than 40 and in autoimmune diseases other than SLE such as autoimmune thyroiditis. N: 243.