Chapter 6 Neonatal Lupus Syndromes

This chapter focuses on the clinical features, epidemiology, pathogenesis, prognosis, and treatments of neonatal lupus syndromes. Neonatal lupus is an in vivo model of acquired autoimmune disease in which autoantibodies are transmitted through the placental barrier from a mother to her fetus. Congenital heart block (CHB) is a rare disorder closely linked to transplacental transport of maternal antibodies anti-Ro/SSA and anti-La/SSB. The prevalence of complete CHB in newborns of prospectively followed women already known to be anti-Ro/SSA positive and with known connective tissue disease is ∼2%. Depending on the severity of the process, the fetus may die in utero or a few days after birth or survive to the perinatal period and have a near normal life; in most survivors a pace-maker must be implanted. Skin lesions, hematological disorders, and hepatic cholestasisare other transient clinical features of the syndrome. Most of the mothers are asymptomatic at delivery and are identified only by the birth of an affected child. Their long-term outcome is generally more reassuring than previously assumed and arthralgias and dry eyes are the commonest symptoms.