Neonatal Lupus Erythematosus

Neonatal lupus is a passively acquired autoimmune disease that occurs in offspring of mothers with anti-SSA/Ro and/or anti-SSB/ La antibodies. The primary clinical features are a photosensitive rash that is usually found on the scalp and periorbital areas, congenital heart block with or without cardiomyopathy, cytopenias, disseminated intravascular coagulation, and neonatal cholestasis with or without elevated transaminases. The diagnosis is usually made in utero by detection of a slow fetal heart rate and subsequent fetal echocardiographic confirmation of heart block and/or cardiomyopathy. Prenatal treatment with fluorinated glucocorticoids beginning as soon after detection has favourable outcome for mothers of fetuses with second degree heart block, is of no value for mothers of fetuses with third degree heart block, and controversial for mothers of fetuses with first degree heart block. Prenatal glucocorticoids can be used also in presence of cardiomyopathy associated with neonatal lupus. Hydroxychloroquine has been used in pregnant women who have anti-SSA/Ro antibodies and who have previously given birth to a child with cardiac manifestations. First and second degree block, detected in utero or at birth, can progress to complete heart block. Infants with complete heart block usually require a pacemaker with an excellent prognosis, although development of heart failure may occur.