Epidemiology, etiology, detection, and treatment of autoantibody-associated congenital heart block in neonatal lupus.

Neonatal lupus syndrome is a model of passively acquired autoimmunity in which the pregnant woman's serum contains specific antibodies to 52 or 60 kd SSA/Ro and/or 48 kd SSB/La, which cross the placenta and are associated with the development of congenital heart block in the fetus and/or a transient rash or various liver and blood cell abnormalities in the newborn. To date, congenital heart block is a permanent condition that entails significant morbidity and mortality, with nearly all affected infants requiring pacemakers and with an 80% cumulative probability of survival at 3 years of age. An intensive search is on for the specific etiopathophysiology and for new clinical tools to approach and treat this disease.