Neonatal lupus erythematosus in Japan: a review of the literature.

Neonatal lupus erythematosus (NLE) is an autoimmune disease associated with maternal anti-SS-A/Ro and anti-SS-B/La antibodies. NLE is characterized by cutaneous erythema, congenital heart block (CHB), hepatic dysfunction and hematological abnormalities. CHB is irreversible, usually requiring a pacemaker, but other symptoms are reversible and most disappear within 6 months in parallel with declining antibody levels. In Japan, 193 cases of NLE were reported between 1971 and 2008. Most showed erythema, and only 23% of cases presented with CHB. Conversely, antibody status had not been examined in many infants presenting with CHB during the same period. Most pregnant woman with anti-SS-A/Ro and anti-SS-B/La antibodies are asymptomatic, and antibody status is first indicated when their child shows symptoms of NLE. These women show a greater risk of delivering an infant with CHB than normal. CHB is important because the main morbidity and mortality of NLE is from CHB. All clinicians should be familiar with the characteristics of NLE. We believe all pregnant women should be screened for anti-SS-A/Ro and anti-SS-B/La antibodies.