Neonatal lupus syndromes

Congenital heart block is considered to be a model of passively acquired autoimmunity, whereby immune abnormalities in the mother lead to the production of autoantibodies that cross the placenta and presumably injure the otherwise normally developing fetus. The major targets of the maternal immune response are the SSA/Ro and SSB/La ribonucleoproteins. Other neonatal abnormalities affecting the skin, liver, and blood elements have also been reported to be associated with anti-SSA/Ro-SSB/La antibodies in the maternal and fetal circulation and are now grouped along with congenital heart block under the heading of the neonatal lupus syndromes. This review covers the histopathology, SSA/Ro-SSB/La antigen-antibody systems, immunogenetics, clinical manifestations, and diagnosis and management strategies of these syndromes.