Neonatal lupus erythematosus - Cardiac manifestations and atrio-ventricular conduction block

Neonatal lupus is a rare disease, with a prevalence of 1:17,000-20,000 child births. The disease is attributed to auto-antibodies such as anti-Ro and anti-La. These antibodies are a common finding in Systemic Lupus Erythematosus and Sjogren disease patients. The clinical manifestations include dermatological, hematological, hepatic, neurological and cardiac involvement. Cardiac disease may be presented as atrioventricular conduction block, cardiomyopathy or endocardial fibroelastosis. If the fetus's heart is affected, the death rates may be as high as thirty percent, and dilated cardiomyopathy may evolve. About 60% of infants will require a permanent pacemaker. Routine screening and early detection are crucial for the initiation of effective treatment. Nevertheless, preventive treatment for all mothers is not recommended due to the low prevalence of the disorder and the common side effects. This article reviews the current knowledge on pathophysiology, and treatment options in neonatal lupus erythematosus.