Neonatal lupus syndrome: review of the literature

Neonatal lupus syndrome include skin lesions, hematological and hepatic disorders, and congenital heart block (CHB) in the absence of severe cardiac malformation. This rare disorder is closely linked to transplacental transport of anti-SSA/Ro and anti-SSB/La maternal antibodies. The prevalence of CHB in newborns of anti-Ro/SSA positive women with known connective tissue disease is 2% and the risk of recurrence ranges from 10 to 17%. Skin and systemic lesions are transient, whereas CHB is definitive and is associated with significant morbidity and mortality (estimated at 16-19%). A pacemaker must be implanted in 2/3 of cases. Myocarditis may be associated or may appeared secondarily. Mothers of children with CHB are usually asymptomatic or have Gougerot-Sjögren, or undifferentiated connective tissue disease. Mothers of children with cutaneous manifestations may present with more severe disease and systemic lupus erythematosus. In anti-Ro/SSA positive pregnant women, echocardiograms should be performed at least every 2 weeks from 16 to 24 weeks gestation. Electrocardiogram should be performed for all children. The efficiency of prophylactic treatment of CHB is not established. Therapy for CHB detected in utero is not standardized and involves fluorinated steroids (especially betamethasone).