Neonatal lupus erythematosus infants and their mothers: a 10-year retrospective study

Abstract

Neonatal lupus erythematosus (NLE) is a rare disease associated with transplacental transfer of maternal anti-Ro/anti-Sjögren syndrome A antibodies. The most common manifestations are cutaneous erythema and congenital heart block. Mothers of infants with NLE are either asymptomatic or diagnosed with autoimmune disease. The goal of this study is to investigate the clinical manifestation, prognosis, and association with autoimmune disease in NLE babies and their mothers in Taiwan. Medical records of newborns with NLE and their mothers from two hospitals in Taiwan between January 1999 and January 2009 were reviewed. Twenty-five newborns (one set of twins) and 24 mothers of infants with NLE were included in the study. Clinical data, including characteristics of skin manifestations, the onset of symptoms, and infants' antibody titers, were collected. A diagnosis of NLE was made if the baby had heart block or characteristic skin lesions and maternal antibodies to Sjögren syndrome A/Ro, Sjögren syndrome B/La, or U1 ribonucleoproteins. Questionnaires were used to determine the mother's health status at the time of delivery and in subsequent years. Of the infants, 84% had typical cutaneous manifestations of NLE, and 16% presented with congenital heart block without skin changes. The cutaneous lesions appeared on average at 21.7 weeks. Thirteen mothers were initially asymptomatic, and the other 11 remained asymptomatic over a mean follow-up period of 3.9 years. Two mothers developed lupus nephritis. There was a lower incidence of observed congenital heart block compared to previously reported NLE studies among Caucasian populations. Most mothers were asymptomatic before the birth of their NLE child, and few developed autoimmune diseases. However, continued follow-up of asymptomatic NLE mothers is recommended due to the chances of developing a life-threatening, systemic autoimmune disease.