Neonatal lupus with congenital atrioventricular block and myocarditis

This is a case of a child with neonatal lupus and congenital atrioventricular (AV) block, born to a mother with asymptomatic, systemic lupus erythematosus (SLE). The child, despite pacemaker insertion, died of septicemia and myocarditis at the age of three months. Although the association of neonatal lupus with congenital AV block is well-recognized, there are only few pathologic studies of the conduction system reported in the literature. This is such a study in which we emphasize that, due to an altered immune system in the child, septicemia may be the cause of death in some cases.