Disease Information for Neonatal Lupus syndrome

Neonatal Lupus; Congenital Lupus; Congenital Lupus Erythematosus ; Neonatal Lupus Erythematosus; Neonatal Lupus Syndrome; Neonatal lupus is a rare autoimmune disorder that is present at birth (congenital); Affected infants often develop a characteristic red rash or skin eruption; In addition, infants with neonatal lupus may develop liver disease, a heart condition known as congenital heart block, and/or low numbers of circulating blood platelets that assist in blood clotting functions (thrombocytopenia); The symptoms associated with neonatal lupus, with the exception of congenital heart block, usually resolve within the first several months of life; The exact cause of neonatal lupus is unknown, although researchers speculate that specific antibodies that travel from a pregnant woman to her developing fetus via the placenta play a significant role; Neonatal lupus is not the infant form of lupus (systemic lupus erythematosus) although the skin rash resembles the one associated with lupus; Neonatal lupus is a separate disorder; [NORD]-------------------------------------

Neonatal Lupus ; Several lupus syndromes can occur in neonates, usually associated with maternal antibodies to Ro; Manifestations can include rash (the most frequent), congenital heart block, cardiomyopathy, hepatobiliary disease, and thrombocytopenia (the last of which is more strongly associated with maternal thrombocytopenia than anti-Ro); The rash consists of plaques of slightly raised erythema that clear spontaneously over several weeks as maternal IgG is metabolized; Congenital heart block can be fatal; in children who survive neonatal cardiac lupus, subsequent cardiomyopathy or total heart block are common; Therefore, patients with SLE who are pregnant should be screened for anti-Ro, and fetal heart rates should be closely monitored in women with anti-Ro so that early delivery and cardiac pacing can be offered if heart block occurs; Treatment of antibody-positive women with prior fetuses with congenital heart block, or evidence of cardiac distress in a current fetus, has been reported; Glucocortoids (dexamethasone or betamethasone), IV gamma globulin, or plasmapheresis have been administered in an attempt to prevent congenital heart block; It is likely that the therapies are effective in suppressing fetal myocarditis as well as fetal pleural effusions, ascites, and hydrops, but chances of reversing heart block, once established, are probably poor; Thrombocytopenia can occur in newborns, probably from maternal antiplatelet antibodies crossing the placenta; The mother may or may not have thrombocytopenia; It is short-lived in the newborn but may be associated with bleeding; Treatment of maternal thrombocytopenia with glucocorticoids is recommended; [KELLY rheumatology 2005]

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