RESUMO
Neonatal lupus has a rare incidence, distinct from systemic lupus erythematosus. This is an acquired autoimmune disease associated with maternal antibodies to proteins Ro / La (SSA /SSB), transferred by the placenta; it represents the prototype of passive transfer of antibodies from mother to child. The disease can affect the skin, heart, and rarely, the hepatobiliary or hematologic systems. Congenital complete heart block is the most severe form of neonatal lupus. In clinical practice it is important to distinguish in utero a complete from an incomplete atrioventricular block (AV) in order to render prompt care. We present the case of a new born female, who was diagnosed with an atrio-ventricular block at 26 weeksí gestation. When the baby was delivered at 38 weeksí gestation, she presented bradycardia (54 xí). On the suspicion of neonatal lupus, we required antinuclear antibodies, anti-Sm, anti-RNP, anti-SS-A and anti-SS-B, which were positive. A bicameral pacemaker was placed uneventfully.