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Published on: March 6, 2019
[Neonatal lupus syndromes]
1Département de Pédiatrie, Hôpital Edouard-Herrio, 69437 Lyon cedex 03, France. roland.cimaz@chu-lyon.fr
Insights
Neonatal lupus syndromes, caused by maternal autoantibodies (anti-SSA/Ro, anti-SSB/La), can affect newborns. Early detection and monitoring during pregnancy are crucial for managing potential heart conduction damage.
Area of Science:
- Immunology
- Pediatrics
- Obstetrics
Context:
- Maternal autoantibodies against SSA/Ro and/or anti-SSB/La ribonucleoproteins can be transmitted to the fetus.
- This can lead to neonatal lupus syndromes, characterized by heart conduction defects, skin rash, and hematologic abnormalities.
- Mothers may be asymptomatic, with diagnosis often occurring after the birth of an affected child.
Purpose:
- To summarize the clinical manifestations and prevalence of neonatal lupus syndromes.
- To highlight the increased risk of recurrence in subsequent pregnancies.
- To emphasize the importance of serial fetal monitoring in at-risk pregnancies.
Summary:
- Neonatal lupus is associated with maternal autoantibodies (anti-SSA/Ro, anti-SSB/La), leading to potential fetal complications like atrioventricular block (2% prevalence) and skin rash (20% prevalence).
- Laboratory abnormalities can occur in up to 40% of infants born to asymptomatic mothers.
- Recurrence risk for complete heart block is significantly higher in subsequent pregnancies.
Impact:
- Early detection via serial echocardiograms and sonograms from 16 weeks gestation in anti-Ro/SSA positive pregnancies can identify fetal abnormalities.
- Prompt intervention may prevent complete atrioventricular block, improving fetal outcomes.
- Understanding the role of transplacental IgG passage and potential fetal/environmental factors aids in risk assessment and management.
Abstract:
Children born from mothers positive for autoantibodies against SSA/Ro and/or anti-SSB/La ribonucleoproteins may develop heart conduction tissue damage resulting in atrioventricular block and/or transient skin rash, liver enzyme abnormalities and anaemia/thrombocytopenia. Additional transient electrocardiographic abnormalities (sinus bradycardia, QT interval prolongation) have been reported. Such clinical and laboratory manifestations are included in the so-called neonatal lupus syndromes, independently whether the mother is suffering from a systemic autoimmune disease or is totally asymptomatic. The prevalence of the congenital heart block is around 2%, of neonatal rash around 20%, while laboratory abnormalities in asymptomatic babies can be detected in up to 40% of cases. The risk of recurrence of complete heart block is almost 10 times higher in the following pregnancies. Most of the mothers are asymptomatic at delivery and are identified only by the birth of an affected child. Their long-term outcome is generally more reassuring than previously assumed. Serial echocardiograms and obstetric sonograms, performed at least every 2 weeks, starting from 16 weeks gestation, are recommended in anti-Ro/SSA positive pregnant women: the goal is to detect early fetal abnormalities, that might precede complete atrioventricular block and that might be a target for preventive therapy. Transplacental passage of maternal anti-SSA/Ro -SSB/La IgG is thought to be pivotal in inducing tissue damage. However, the discordant appearance of the syndrome in twins does suggest a role also for fetal or environmental factors.