AB0983 Possible Correlation Between Congenital Heart Block and Autoimmune Hearing Loss

2015 ◽  
Vol 74 (Suppl 2) ◽  
pp. 1227.2-1227
Author(s):  
I. Pagnini ◽  
C. Bason ◽  
G. Simonini ◽  
T. Giani ◽  
A. Brucato ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Heart Block ◽  
Congenital Heart ◽  
Congenital Heart Block ◽  
Autoimmune Hearing Loss

Congenital heart block and immune mediated sensorineural hearing loss: possible cross reactivity of immune response

Lupus ◽  
2016 ◽  
Vol 26 (8) ◽  
pp. 835-840 ◽  
Author(s):  
C Bason ◽  
I Pagnini ◽  
A Brucato ◽  
S Maestroni ◽  
A Puccetti ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Inner Ear ◽  
Heart Block ◽  
Congenital Heart ◽  
Sensorineural Hearing ◽  
Congenital Heart Block ◽  
Cardiac Conduction ◽  
Immune Mediated ◽  
Cogan Syndrome

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.

Surveillance of congenital heart block in highly specialised care – Authors' reply

2020 ◽  
Vol 2 (4) ◽  
pp. e204-e205 ◽  
Author(s):  
Nathalie Costedoat-Chalumeau ◽  
Nathalie Morel
Keyword(s):  
Heart Block ◽  
Congenital Heart ◽  
Congenital Heart Block

Apheresis in high risk antiphospholipid syndrome pregnancy and autoimmune congenital heart block

2015 ◽  
Vol 53 (3) ◽  
pp. 269-278 ◽  
Author(s):  
Amelia Ruffatti ◽  
Maria Favaro ◽  
Antonio Brucato ◽  
Veronique Ramoni ◽  
Myriam Facchinetti ◽  
...  
Keyword(s):  
High Risk ◽  
Antiphospholipid Syndrome ◽  
Heart Block ◽  
Congenital Heart ◽  
Congenital Heart Block

Congenital heart block: Natural history and management

1995 ◽  
Vol 4 (2) ◽  
pp. 63-65 ◽  
Author(s):  
Brian K. O'Connor ◽  
Christopher L. Case ◽  
Paul C. Gillette
Keyword(s):  
Natural History ◽  
Heart Block ◽  
Congenital Heart ◽  
Congenital Heart Block

scholarly journals QT interval prolongation in asymptomatic anti-SSA/Ro–positive infants without congenital heart block

2000 ◽  
Vol 43 (5) ◽  
pp. 1049 ◽  
Author(s):  
Rolando Cimaz ◽  
Marco Stramba-Badiale ◽  
Antonio Brucato ◽  
Luca Catelli ◽  
Paola Panzeri ◽  
...  
Keyword(s):  
Qt Interval ◽  
Heart Block ◽  
Congenital Heart ◽  
Congenital Heart Block ◽  
Interval Prolongation ◽  
Qt Interval Prolongation

ERV-3 Envelope Expression and Congenital Heart Block: What does a Physiological Knockout Teach Us

Autoimmunity ◽  
1999 ◽  
Vol 30 (2) ◽  
pp. 81-83 ◽  
Author(s):  
Nathalie De Parseval ◽  
Graham Forrest ◽  
Patrick J.W. Venables ◽  
Thierry Heidmann
Keyword(s):  
Heart Block ◽  
Congenital Heart ◽  
Congenital Heart Block

scholarly journals A shared idiotype among human anti-Ro/SSA autoantibodies.

1989 ◽  
Vol 169 (5) ◽  
pp. 1583-1588 ◽  
Author(s):  
K K Gaither ◽  
J B Harley
Keyword(s):  
Binding Site ◽  
Heart Block ◽  
Congenital Heart ◽  
Congenital Heart Block ◽  
Antigen Binding ◽  
Normal Donor ◽  
Neonatal Lupus ◽  
Antigen Binding Site ◽  
Fine Specificity ◽  
Low Level

Idiotypes and antiidiotypes are thought to be important immune regulators and have provided clues for the origin and pathogenicity of autoantibodies. Many lupus and Sjögren's syndrome patients, as well as most neonatal lupus infants with congenital heart block or dermatitis, have antibodies to the ribonucleoprotein Ro/SSA, which is one of a group of RNA-protein autoantigens commonly found in human lupus sera. To characterize the fine specificity of anti-Ro/SSA antibodies, a rabbit antidiotypic serum was prepared against polyclonal affinity purified anti-Ro/SSA F(ab')2. The resulting antiidiotype, anti-Id-Rol, is specific for the F(ab')2 fraction of the anti-Ro/SSA immunogen and its binding to anti-Ro/SSA is inhibited by purified Ro/SSA. These data indicate that the Id-Rol epitope on anti-Ro/SSA is associated with the antigen binding site of these same antibodies. The Id-Rol idiotype was present by ELISA in 3 of 12 additional anti-Ro/SSA preparations from precipitin-positive donor sera and in anti-Ro/SSA from one normal donor with low level antibody. This is the first shared idiotype to be found in the human autoantibodies binding to this RNA-protein antigen. Idiotypic differences between anti-Ro/SSA autoantibodies have the potential to explain the variation in pathologic associations found in individuals who develop this autoantibody specificity.

Management of a hydropic infant with congenital heart block

1990 ◽  
Vol 26 (3) ◽  
pp. 158-159 ◽  
Author(s):  
L.Y. SO ◽  
R.Y.T. SUNG ◽  
J.K.S. HO ◽  
T.F. FOK ◽  
Y.L. CHAN ◽  
...  
Keyword(s):  
Heart Block ◽  
Congenital Heart ◽  
Congenital Heart Block
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