New findings in neonatal lupus syndrome
L. A. Lee and W. L. Weston
Neonatal lupus is a syndrome characterized by cutaneous lupus and/or
congenital heart block (CHB). This report reviews our original observations
on patients with neonatal lupus during the past five years: (1) Sicca
syndrome (SS-A) (Ro) autoantibodies were found in the serum of the mothers
and infants, were of maternal origin, and constituted a marker for the
syndrome. (2) SS-A autoantibodies were found in the majority of the cases
of "idiopathic" CHB and may have been the most common cause of all CHBs.
(3) Mothers who had one child affected were at risk for having a second
child affected. (4) Mothers were often asymptomatic. (5) HLA associations
in this syndrome were HLA-DR3, HLA-B8, HLA-MB2, and HLA-MT2, and these
occurred in mothers but not infants. Thus, the HLA association was with
autoantibody production rather than tissue injury, a finding that may help
clarify genetic and environmental roles in autoantibody-mediated disease.
