title: Lack of increased prevalence of immunoregulatory disorders in hereditary angioedema due to C1-inhibitor deficiency.
abstract: Hereditary angioedema due to deficiency of C1-INH (HAE-C1-INH) is associated with enhanced consumption of the early complement components, which may predispose for autoimmune disease. We assessed the prevalence of such disorders among HAE- C1-INH patients and their impact on the natural course of HAE-C1-INH. Clinical data and immunoserological parameters of 130 HAE-C1-INH and 174 non-C1-INH-deficient patients with angioedema were analyzed. In our study, the incidence of immunoregulatory disorders was 11.5% in the population of HAE-C1-INH patients and 5.2% among non-C1-INH-deficient controls with angioedema. Immunoserology screening revealed a greater prevalence of anticardiolipin IgM (p=0.0118) among HAE-C1-INH patients, than in those with non-C1-INH-deficient angioedema. We did not find higher prevalence of immunoregulatory disorders among our HAE-C1-INH patients. However, in patients with confirmed immunoregulatory disorders, the latter influenced both the severity of HAE-C1-INH and the effectiveness of its long-term management. Appropriate management of the immunoregulatory disease thus identified improves the symptoms of HAE-C1-INH.
authors.affiliation: Semmelweis University, 3rd Department of Internal Medicine, Budapest, Hungary. farkash@kut.sote.hu
authors.forename: Henriette
authors.initials: H
authors.lastname: Farkas
authors.forename: Dorottya
authors.initials: D
authors.lastname: Csuka
authors.forename: Judit
authors.initials: J
authors.lastname: Gács
authors.forename: Ibolya
authors.initials: I
authors.lastname: Czaller
authors.forename: Zsuzsanna
authors.initials: Z
authors.lastname: Zotter
authors.forename: George
authors.initials: G
authors.lastname: Füst
authors.forename: Lilian
authors.initials: L
authors.lastname: Varga
authors.forename: Péter
authors.initials: P
authors.lastname: Gergely
mesh_terms: D000328:Adult; D000799:Angioedema / Q000097:blood / Q000150:complications / Q000276:immunology; D017153:Antibodies, Anticardiolipin / Q000097:blood; D001323:Autoantibodies / Q000097:blood; D001327:Autoimmune Diseases / Q000097:blood / Q000209:etiology* / Q000276:immunology; D016022:Case-Control Studies; D015984:Causality; D002446:Celiac Disease / Q000097:blood / Q000150:complications / Q000276:immunology; D050718:Complement C1 Inhibitor Protein / Q000378:metabolism*; D005260:Female; D056829:Hereditary Angioedema Types I and II / Q000097:blood / Q000150:complications* / Q000276:immunology*; D006801:Humans; D017098:IgA Deficiency / Q000097:blood / Q000150:complications / Q000276:immunology; D007075:Immunoglobulin M / Q000097:blood; D008297:Male; D008875:Middle Aged; D055815:Young Adult
chemical_list: D017153:Antibodies, Anticardiolipin; D001323:Autoantibodies; D050718:Complement C1 Inhibitor Protein; D007075:Immunoglobulin M