Congenital heart block and immune mediated sensorineural hearing loss: possible cross reactivity of immune response. (July 2017)

• Record Type: Journal Article
• Authors: Bason, C; Pagnini, I; Brucato, A; Maestroni, S; Puccetti, A; Lunardi, C; Cimaz, R
• Is Part Of: Lupus. Volume 26:Number 8(2017)
• Page Start: 835
• Page End: 840
• Publication Date: 2017-07
• Subjects: Anti-SSa/Ro antibodies -- inner ear antigens -- cross-reactive antibodies -- Sjögren's syndrome; Systemic lupus erythematosus -- Periodicals; 616.772005
• DOI: 10.1177/0961203316682099
• Languages: English
• ISSNs: 0961-2033

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.