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Review
. 2021 Jan 18;49(5):529-538.
doi: 10.1515/jpm-2020-0543. Print 2021 Jun 25.

Neonatal lupus erythematosus - practical guidelines

Julia Marta Derdulska  1 , Lidia Rudnicka  1 , Agata Szykut-Badaczewska  1 , Dorota Mehrholz  2 , Roman J Nowicki  2 , Wioletta Barańska-Rybak  2 , Aleksandra Wilkowska  2
Affiliations
Review

Neonatal lupus erythematosus - practical guidelines

Julia Marta Derdulska et al. J Perinat Med. .

Abstract

Background: Neonatal lupus erythematosus is an autoimmune disease acquired during fetal life as a result of transplacental passage of maternal anti-Sjögren's-syndrome-related antigen A (anti-SSA/Ro), anti-Sjögren's-syndrome-related antigen B (anti-SSB/La) or anti-U1 ribonucleoprotein (anti-U1-RNP) antinuclear autoantibodies.

Contents: Clinical manifestations include skin lesions, congenital heart block, hepatobiliary involvement and cytopenias. Most of the disorders disappear spontaneously after clearance of maternal antibodies. Cardiac symptoms, however, are not self-resolving and often pacemaker implantation is required. Diagnosis is based on clinical presentation and the presence of typical antibodies in the mother's or infant's serum.

Outlook: Neonatal lupus erythematosus may develop in children born to anti-SSA/Ro or anti-SSB/La women with various systemic connective tissue diseases. However, in half of the cases, the mother is asymptomatic, which may delay the diagnosis and have negative impact on the child's prognosis. Testing for antinuclear antibodies should be considered in every pregnant woman since early treatment with hydroxychloroquine or intravenous immunoglobulin (IVIG) has proven to be effective in preventing congenital heart block.

Keywords: antinuclear antibodies; congenital heart block; neonatal lupus erythematosus; newborn; pediatric dermatology.

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References

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