Neonatal lupus syndrome

Neonatal lupus syndrome is an autoimmune disease associated with transplacental passage of maternal autoantibodies to SSA/Ro and SSB/La antigens. Characterised by cutaneous lesions and cardiac involvement, the most important clinical manifestation is complete heart block. Rarely, hepatic and haematological abnormalities occur. Although occurring in only 2% of affected mothers, regular fetal monitoring is mandatory in all mothers with anti-SSA/Ro or anti-SSB/La antibodies from approximately 16 weeks of gestation to detect evidence of cardiac conduction defects. If present, maternal corticosteroid therapy may be indicated. Careful postnatal monitoring is essential to identify cardiac and other manifestations that may yet develop. Skin, hepatic and haematological manifestations are self-limiting and resolve. Cardiac disease can be fatal. Heart block is permanent, often requiring cardiac pacing. Mothers of infants with neonatal lupus syndrome may be asymptomatic but often develop autoimmune diseases, including systemic lupus erythematosus or Sjogren's syndrome. Affected infants may be at increased risk of subsequent autoimmune disease.