Neonatal lupus is characterized by congenital heart block, dermatitis, hepatitis, myocarditis and hematologic disorders and is strongly associated with the passive transfer of maternal autoantibodies. Except for congenital heart block symptoms of neonatal lupus usually resolve by 6 months, paralleling the disappearance of the maternal antibodies.

We present 3 fatal cases of neonatal lupus; autopsies were performed at 7 weeks and 19 months; the third patient had a liver biopsy in infancy. Two mothers had lupus; 1 remains well but declined serologic testing. Two babies developed serologic, clinical and tissue evidence of active primary lupus without ever recovering completely from neonatal disease. The third case, a premature infant, lacked heart block and rash but did have severe systemic involvement, with serologic and tissue evidence of active lupus within the first several weeks of life. In all 3 cases we found that lupus during the newborn period can include features typical of primary lupus, including lupus nephritis, CNS symptoms, intracerebral calcifications, sterile valvular vegetations and polyserositis. Our cases demonstrate that SLE may be superimposed on neonatal disease induced by passively transferred antibodies.