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Neonatal Systemic Lupus Erythematosus Syndrome: a Comprehensive Review

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Abstract

Neonatal lupus erythematosus is an uncommon syndrome, which is caused by transplacental passage of maternal autoantibodies to Sjögren’s syndrome A or B autoantigens. The clinical presentation includes distinctive cutaneous lesions resembling those seen in systemic lupus erythematosus, hepatobiliary disease, and cytopenias, which disappear with the clearance of maternal autoantibodies. The most severe presentation is a total atrioventricular heart block, which begins during the second trimester of gestation and is irreversible. The risk of having a child with neonatal lupus erythematosus in mothers who test positive for autoantibodies to Sjögren’s syndrome autoantigens is approximately 2% for first pregnancies or if previous babies were healthy. The risk increases by approximately tenfold if a previous child had neonatal lupus erythematosus syndrome. The diagnosis of neonatal lupus erythematosus is made when the mother has autoantibodies to Sjögren’s syndrome autoantigens, and the fetus or newborn develops atrioventricular heart block, or the newborn develops the typical rash or hepatic or hematologic manifestations in the absence of other explanation. Fetal echocardiography from the 16th to the 26th week of gestation is advised in mothers with autoantibodies to Sjögren autoantigens. The detection of a slow fetal heart rate or the postnatal diagnosis of atrioventricular heart block warrants immediate maternal testing for these autoantibodies if not previously tested.

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Acknowledgments

The authors thank the families for permitting to publish clinical images with recognizable features of their children.

The authors would like to thank Dr. Alec Villa for his assistance in the linguistic revision.

Funding

This work was supported in part by the Fondazione E. Balli and the Swiss Research Network of Clinical Pediatric Hubs.

Dr. Gregorio P. Milani is the current recipient of research grants from the Fondazione G. E D. de Marchi ONLUS.

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Correspondence to Gregorio P. Milani.

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The present article does not contain any studies with human participants or animals performed by any of the authors.

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Vanoni, F., Lava, S.A.G., Fossali, E.F. et al. Neonatal Systemic Lupus Erythematosus Syndrome: a Comprehensive Review. Clinic Rev Allerg Immunol 53, 469–476 (2017). https://doi.org/10.1007/s12016-017-8653-0

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  • DOI: https://doi.org/10.1007/s12016-017-8653-0

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