Neonatal Lupus Erythematosus

Excerpt

Neonatal lupus erythematosus (NLE) is an acquired autoimmune disorder caused by transplacental passage of maternal immunoglobulin G (IgG) autoantibodies, predominantly Sjögren syndrome antigens A (Ro/SSA) and B (La/SSB), leading to clinical manifestations in the neonate. This pathogenesis parallels other neonatal autoimmune conditions, including antiphospholipid antibody syndrome, Graves disease, immune thrombocytopenic purpura, myasthenia gravis, and autoimmune blistering diseases. Bridge and Foley first described NLE in 1954, after observing transplacental transmission of the maternal lupus erythematosus factor to newborns.

That same year, a lupus rash was reported in a 6-week-old infant whose mother was later diagnosed with systemic lupus erythematosus (SLE). In 1957, congenital heart block was documented in an infant born to a mother with SLE. Recognition of NLE as a multisystem disorder has broadened its clinical spectrum to include hepatic enzyme elevations and transient cytopenias. The clinical spectrum of NLE ranges from transient hematological and dermatological abnormalities to severe, irreversible cardiac manifestations, especially congenital heart block. Notably, a substantial proportion of affected neonates are born to mothers without clinically apparent autoimmune disease, highlighting the importance of maternal antibody screening during pregnancy.