Abstract
One of the strongest clinical associations with autoantibodies against components of the SSA/Ro–SSB/La ribonucleoprotein complex is the development of congenital heart block in an offspring, an alarming prospect facing 2% of primigravid mothers with these reactivities. This risk is increased tenfold in women who have had a previous child with congenital heart block. Accumulated evidence suggests that anti-SSA/Ro and anti-SSB/La antibodies are necessary but insufficient for fetal disease. Basic and clinical research is heavily focused on identifying fetal and environmental factors that convert disease susceptibility to disease development. A disturbing observation that has emerged from current research efforts is the rapidity of disease progression, with advanced heart block and life-threatening cardiomyopathy being observed less than 2 weeks after detection of a normal sinus rhythm. Once third-degree block is unequivocally identified, reversal has never been achieved, despite dexamethasone treatment. Accordingly, strategies aimed at preventing disease before irrevocable scarring ensues assume a high priority. One approach has been the implementation of serial echocardiography to monitor for a prolonged PR interval. Intravenous immunoglobulin is being evaluated as a potential prophylactic approach in mothers who have previously had an affected child.
Key Points
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Mothers with anti-SSA/Ro antibodies face a 2% risk of having a child with congenital heart block if it is a first pregnancy or if previous babies have all been healthy
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A previous child with congenital heart block raises the risk of having another by almost tenfold
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Normal sinus rhythm can progress to complete block in 7 days; thus, frequent monitoring of a pregnancy in a mother with anti-SSA/Ro antibodies is appropriate
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A mechanical PR interval of greater than 150 ms is consistent with first-degree block, and warrants an immediate discussion regarding the use of a fluorinated steroid to potentially reverse the situation
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Intravenous immunoglobulin is currently being evaluated as a prophylactic therapy
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Acknowledgements
This work was funded by an NIH-NIAMS grant (Maternal Autoantibodies: Pathogenesis of Neonatal Lupus), an NIH contract (Research Registry for Neonatal Lupus) and an NIH-NIAMS grant (the PRIDE study) to Dr Buyon, and an American Heart Association, Heritage Affiliate grant to Dr Clancy.
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Buyon, J., Clancy, R. & Friedman, D. Cardiac manifestations of neonatal lupus erythematosus: guidelines to management, integrating clues from the bench and bedside. Nat Rev Rheumatol 5, 139–148 (2009). https://doi.org/10.1038/ncprheum1018
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DOI: https://doi.org/10.1038/ncprheum1018
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