Rheumatology Advance Access originally published online on July 10, 2007
Rheumatology 2007 46(8):1221-1222; doi:10.1093/rheumatology/kem148
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© The Author 2007. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oxfordjournals.org
EDITORIALS |
Predicting autoimmune congenital heart block: is it feasible and how?
Department of Pathophysiology, Medical School, University of Athens, Greece
Correspondence to: A. G. Tzioufas. E-mail: agtzi@med.uoa.gr
| The first 150 words of the full text of this article appear below. |
Autoimmune congenital heart block (CHB) is the most serious complication of neonatal lupus syndrome (NLS), a rare disorder afflicting fetuses of mothers with circulating autoantibodies to Ro/SSA and La/SSB ribonucleoproteins. The syndome is considered a model of passively acquired autoimmunity since during pregnancy maternal autoantibodies are transferred across the placenta, bind onto tissues of the developing fetus and eventually produce the pathology of NLS. NLS develops in 2–5% of fetuses of Ro/SSA and La/SSB autoantibody-positive pregnant women. Two thirds of mothers have Sjögren's syndrome, systemic lupus erythematosus or undifferentiated connective tissue disease and the remainder are asymptomatic. Although NLS can affect the skin, liver, blood cells and heart, almost all extra-cardiac manifestations subside when the maternal autoantibodies are cleared from the circulation of the offspring [1]. NLS has certain characteristics that distinguish it from other autoimmune diseases. These include a unique causative factor (antibodies against Ro/SSA and La/SSB),
Functional studies
Immunological studies