Maternal autoantibodies and pregnancy--II: The neonatal lupus syndrome

Abstract

NLE is manifested most typically as transient subacute cutaneous lupus lesions or isolated complete congenital heart block. Babies with NLE have maternal anti-Ro/SSA, anti-La/SSB, or anti-U1RNP autoantibodies. It is presumed, but not proven, that transmission of these autoantibodies through the placenta to the baby has resulted in disease. However, other factors such as inflammatory cells or complement activation may be necessary for disease to be expressed. About half of babies reported with NLE have had heart disease and about half have had skin disease. There have been a few reports of liver disease and a few of thrombocytopenia. Any combination of these findings is possible in a given infant. Possibly, other haematologic abnormalities, pneumonitis or neurological disease could occur, but the evidence that these other abnormalities are part of NLE is scant. Mortality in NLE has occurred in babies with severe cardiac disease. It is estimated that 10% or more of babies with cardiac NLE die in infancy. Of the remainder, perhaps half will require permanent pacemaker implantation. Thus, there is substantial morbidity and mortality with cardiac NLE. The skin disease, by contrast, is not serious and typically leaves little or no residua. Individuals who have had NLE may develop connective tissue disease in adulthood. Whether this is a common or an unusual occurrence is not yet known, since a large cohort of individuals with NLE has not yet been followed into adulthood. Mothers of babies with NLE are often initially asymptomatic. With time, they frequently develop connective tissue disease symptoms. In our experience, these have been largely symptoms of Sjögren's syndrome and have generally not been debilitating. Most babies of mothers with anti-Ro/SSA, anti-La/SSB, or anti-U1RNP autoantibodies do not develop NLE. There is no way to determine prospectively which fetus or infant will be affected and which of those affected will have life-threatening disease. Systemic therapies should be reserved for those infants who have life-threatening manifestations of NLE. It is not yet known whether treatment of the mother during gestation will be beneficial or harmful to fetuses with severe NLE cardiac disease.