title: Strong correlation of high EBNA-1-IgG levels with edematous attacks involving upper airway mucosa in hereditary angioedema due to C1-inhibitor deficiency.
abstract: Elevated level of IgG-type antibodies against Type 1 nuclear antigen (anti-EBNA-1-IgG) of the Epstein-Barr virus is a strong risk factor for certain autoimmune diseases. We measured anti-EBNA-1 IgG titers in 107 patients with hereditary angioedema due to C1-inhibitor deficiency (HAE-C1-INH). In the sera from 33 longitudinally tested patients, we found a very strong correlation (R>0.75, p=0.0005) between anti-EBNA-1-IgG titers measured in 2004 and 2010, respectively. High (>200 U/ml) anti-EBNA-1 levels were strongly correlated with the frequency of upper airway attacks (p=0.003) and the dose requirement of C1-inhibitor concentrate (p=0.008), while no significant association with the frequency of subcutaneous and abdominal attacks was found. These novel findings indicate that the underlying/triggering mechanisms of upper airway attacks in HAE-C1-INH may differ from that of other types of attacks and measurement of the anti-EBNA-1 IgG levels may be suitable for the prediction of upper airway attacks and C1-inhibitor concentrate requirement in HAE-C1-INH patients.
authors.affiliation: 3rd Department of Internal Medicine, Semmelweis University, Budapest, Hungary. csukadorka@gmail.com
authors.forename: Dorottya
authors.initials: D
authors.lastname: Csuka
authors.forename: Lilian
authors.initials: L
authors.lastname: Varga
authors.forename: Henriette
authors.initials: H
authors.lastname: Farkas
authors.forename: George
authors.initials: G
authors.lastname: Füst
mesh_terms: D000328:Adult; D054179:Angioedemas, Hereditary / Q000276:immunology*; D001327:Autoimmune Diseases / Q000276:immunology / Q000378:metabolism; D016022:Case-Control Studies; D050718:Complement C1 Inhibitor Protein / Q000276:immunology*; D019309:Epstein-Barr Virus Nuclear Antigens / Q000276:immunology*; D005260:Female; D004854:Herpesvirus 4, Human / Q000276:immunology; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood* / Q000276:immunology*; D008297:Male; D008875:Middle Aged; D020545:Respiratory Mucosa / Q000276:immunology*; D055815:Young Adult
chemical_list: D050718:Complement C1 Inhibitor Protein; D019309:Epstein-Barr Virus Nuclear Antigens; D007074:Immunoglobulin G; C417029:EBV-encoded nuclear antigen 1