Neonatal Lupus Erythematosus (NLE)

Neonatal lupus erythematosus (NLE) or neonatal lupus syndrome (NLS) is a rare syndrome seen in 1-2% of neonates with auto-antibodies to SSA/Ro, SSB/La and or U1 RNP, passively transferred transplacentally from the mother who is either asymptomatic or having manifestations of Sjogren’s syndrome, SLE or other systemic rheumatic disease, characterized by cutaneous, cardiac or rarely both clinical manifestations. The skin manifestations are seen at least in 30% of these patients, in the form of periorbital annular erythematous plaques later spreading to other areas of face, scalp, trunk and extremities which is non-scarring and non-atrophic. This is usually transient lasting for days to months. But, the cardiac manifestations are seen in up to 60% of the patients is mainly in the form of complete congenital heart block (CHB) which is irreversible and is associate with cardiomyopathy in at least 10% of the cases. Cardiomyopathy is associated with increased morbidity and mortality. Almost all the patients having cardiac lupus require permanent pacemaker. The recurrence rate of neonatal lupus is as much as 25% in the subsequent pregnancies. There has been better understanding of aetiopathogenesis of the disease which is due to the rapid advances in field of medicine [Buyon, 2001, 2007].