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Published on: January 27, 2019
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[Neonatal lupus syndrome: Literature review]
N Morel1, S Georgin-Lavialle2, K Levesque3
1Centre de référence maladies auto-immunes et systémiques rares, service de médecine interne pôle médecine, hôpital Cochin, AP-HP, université René-Descartes Paris V, 27, rue du Faubourg-Saint-Jacques, 75679 Paris cedex 14, France.
Summary
Neonatal lupus syndrome, caused by maternal antibodies, can lead to congenital heart block (CHB) in infants. Early detection and monitoring during pregnancy are crucial for managing this serious condition.
Area of Science:
- Obstetrics and Gynecology
- Pediatrics
- Immunology
Context:
- Neonatal lupus syndrome (NLS) arises from maternal autoantibodies crossing the placenta.
- Key antibodies involved are anti-SSA/Ro and anti-SSB/La.
- Affected infants present with diverse symptoms, including cardiac, cutaneous, hematological, and hepatic manifestations.
Purpose:
- To summarize the clinical features, prevalence, and management of neonatal lupus syndrome, with a focus on cardiac manifestations.
- To highlight the importance of maternal antibody screening and fetal monitoring in at-risk pregnancies.
- To review current and emerging therapeutic strategies for NLS.
Summary:
- Congenital heart block (CHB) is a significant cardiac complication of NLS, occurring in 1-2% of newborns from mothers with connective tissue disease and anti-Ro/SSA antibodies.
- While skin and systemic lesions are typically transient, CHB is permanent, leading to high morbidity (18% mortality, 2/3 requiring pacemakers).
- Maternal hydroxychloroquine use may reduce recurrent CHB risk, and ongoing research is evaluating its efficacy.
Impact:
- Emphasizes the need for vigilant prenatal screening and monitoring in pregnancies involving anti-SSA/Ro positive mothers.
- Informs clinical practice regarding the management of NLS and its cardiac complications.
- Highlights potential preventative strategies like maternal hydroxychloroquine use and the need for further research.
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