The clinical spectrum of neonatal lupus

Neonatal lupus is an uncommon condition associated with maternal anti-Ro autoantibodies. Findings may include cutaneous lupus lesions, third-degree heart block, cardiomyopathy, hepatobiliary disease, and/or thrombocytopenia or other hematologic cytopenias. It is common for only one organ to be affected, but any combination of organ involvement may occur. Recent studies have raised the possibility that the central nervous system may also be affected, but if it is, it is generally apparently asymptomatic. The most common severe manifestation of neonatal lupus is third-degree heart block, which usually begins during the second trimester of gestation. Attempts have been made to prevent the development of heart block, most often by treating the mother with systemic corticosteroids during pregnancy. There is not yet consensus as to the value of intervention during pregnancy. The neonatal lupus disease process is transient, although third-degree heart block, once established, is permanent. Cutaneous lesions tend to resolve completely and affected individuals tend to be healthy later in childhood. There does appear to be an increased risk for children who have had neonatal lupus to develop autoimmune diseases later in childhood or adulthood. The magnitude of that risk is uncertain. Mothers, who are often asymptomatic at the time of delivery of a baby with neonatal lupus, tend eventually to develop signs and symptoms of autoimmune disease.