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 Vol. 9 No. 5, May 2008
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NeoReviews Vol.9 No.5 2008 e206
© 2008 American Academy of Pediatrics

Neonatal Lupus and Related Autoimmune Disorders of Infants

Jennifer Frankovich, MD*
Christy Sandborg, MD*
Pat Barnes, MD*
Susan Hintz, MD*
Eliza Chakravarty, MD*

* Lucile Salter Packard Children's Hospital, Stanford University School of Medicine, Stanford, Calif

Neonatal lupus syndromes are caused by maternal antibodies targeting proteins displayed on apoptotic blebs. Mothers frequently are healthy and unaware of their autoantibody status. Manifestations in infants include rashes, cytopenias, hepatobiliary disease, heart block, and rarely, cardiomyopathies. Cerebral dysmaturation, ventriculomegaly, and lenticulostriate vasculopathy are recently described manifestations. Rhizomelic chondrodysplasia punctata, pneumonitis, nephritis, and multiorgan failure are rare. Coexisting antithyroid and antiphospholipid antibodies may complicate the presentation. Symptoms typically disappear with the clearance of maternal antibodies from the neonatal circulation, except in cases where the disease is extensive or involves vulnerable tissues. Early diagnosis, close monitoring, and appropriate intervention with immunosuppressive treatment may subvert organ-threatening disease in select cases.

Abbreviations: ANA: antinuclear antibody • APL: antiphospholipid • CHB: congenital heart block • CNS: central nervous system • Ig: immunoglobulin • IVIg: intravenous immunoglobulin • NLS: neonatal lupus syndrome • RNP: ribonuclear protein • SLE: systemic lupus erythematosus • SSA/Ro: anti-Sjögren's syndrome A antibody (also known as anti-Ro antibody) • SSB/La: anti-Sjögren's syndrome B antibody (also known as anti-La antibody)






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Copyright © 2008 by the American Academy of Pediatrics.