title: Congenital heart block and immune mediated sensorineural hearing loss: possible cross reactivity of immune response.
abstract: Immune-mediated sensorineural hearing loss may complicate systemic autoimmune diseases. We have previously reported the presence of antibodies directed against inner ear antigens in patients with Cogan syndrome, a disease characterized by sudden hearing loss and interstitial keratitis. Such autoantibodies cross-react with an epitope of SSA/Ro60 protein. Anti-Ro/SSA antibodies in pregnant women cross the placenta and reach the fetal tissues inducing an immune-mediated damage of the cardiac conduction system. We wanted to evaluate whether mothers with anti-Ro/SSA antibodies who gave birth to children with congenital heart block have antibodies directed against inner ear antigens and whether these antibodies are connected with the presence of immune-mediated sensorineural hearing loss. We did not find anti-inner ear antibodies in the majority of the mothers. On the contrary a 13-year-old boy with congenital heart block and sensorineural hearing loss was positive for the presence of anti-inner ear antigens antibodies. Moreover his serum was positive for the presence of anti-Ro60 peptide antibodies but did not recognize the entire protein Ro60 (TROVE2), a behaviour similar to that of sera from patients with Cogan syndrome. In conclusion the data obtained so far show that anti-inner ear antibodies do not recognize the entire protein TROVE2 and do not support the hypothesis that such antibodies may be involved in the pathogenesis of congenital heart block.
authors.affiliation: 1 Department of Medicine, University of Verona, Verona, Italy.
authors.forename: C
authors.initials: C
authors.lastname: Bason
authors.affiliation: 2 Rheumatology Unit, Anna Meyer Children's Hospital and University of Florence, Florence, Italy.
authors.forename: I
authors.initials: I
authors.lastname: Pagnini
authors.affiliation: 3 Internal Medicine, Ospedali Riuniti di Bergamo, Bergamo, Italy.
authors.forename: A
authors.initials: A
authors.lastname: Brucato
authors.affiliation: 3 Internal Medicine, Ospedali Riuniti di Bergamo, Bergamo, Italy.
authors.forename: S
authors.initials: S
authors.lastname: Maestroni
authors.affiliation: 4 Immunology Area, Ospedale Pediatrico Bambin Gesù, Rome, Italy.
authors.forename: A
authors.initials: A
authors.lastname: Puccetti
authors.affiliation: 1 Department of Medicine, University of Verona, Verona, Italy.
authors.forename: C
authors.initials: C
authors.lastname: Lunardi
authors.affiliation: 2 Rheumatology Unit, Anna Meyer Children's Hospital and University of Florence, Florence, Italy.
authors.forename: R
authors.initials: R
authors.lastname: Cimaz
mesh_terms: D000293:Adolescent; D000974:Antibodies, Antinuclear / Q000276:immunology*; D001323:Autoantibodies / Q000276:immunology*; D001324:Autoantigens / Q000276:immunology; D055952:Cogan Syndrome / Q000276:immunology; D003429:Cross Reactions / Q000276:immunology; D000939:Epitopes / Q000276:immunology; D005260:Female; D006319:Hearing Loss, Sensorineural / Q000276:immunology*; D006327:Heart Block / Q000151:congenital* / Q000276:immunology; D006801:Humans; D008297:Male; D009035:Mothers
chemical_list: D000974:Antibodies, Antinuclear; D001323:Autoantibodies; D001324:Autoantigens; D000939:Epitopes; C035356:SS-A antibodies
keywords: Anti-SSa/Ro antibodies; Sjögren’s syndrome; cross-reactive antibodies; inner ear antigens