Neonatal Lupus

Neonatal lupus erythematosus (NLE) is a rare disorder caused by the transplacental passage of maternal autoantibodies that involves the presence of human leukocyte antigen B8 (HLA-B8) and human leukocyte antigen DR3 (HLA-DR3) in the mother which predisposes the infant to neonatal lupus erythematosus and congenital heart block. Congenital heart block can result in congestive heart failure and subsequent placement of a pacemaker. In one investigation, 57% of patients eventually required a pacemaker. Congenital heart block is associated with a 20-30% mortality rate in the neonatal period. Deaths may also occur later in life as a result of the failure of the pacemaker. Neonatal lupus erythematosus has been reported slightly more often in female infants than in male infants. The onset of neonatal lupus erythematosus occurs between birth and a few months of life.