neonatal lupus erythematosus

Etiology

• occurs in infants of mothers with systemic lupus erythematosus or Sjogren's syndrome
• asymptomatic mothers generally develop one of these disorders

Epidemiology

rare

Clinical manifestations

• rash (head, neck & trunk)
• congenital heart block
• hepatitis
• hemolytic anemia

Laboratory

• mother has anti-SSA/Ro, anti-SSB/La or both
  • necessary, but not sufficient for disease
  • 25% of pregnant with these antibodies have babies with neonatal lupus rash & 5% have babies with congenital heart block
  • maternal antibodies to RAB11FIP5
• increased liver function tests (LFTs)
• complete blood count (CBC)
  • thrombocytopenia
  • hemolytic anemia

Management

• the rash resolves when maternal IgG levels fall
• congenital heart block
  • 1/3 of infants die before age 3
  • most of the remainder require permanent pacemakers

More general terms

• systemic lupus erythematosus
• neonatal disorder or disease

Additional terms

• anti-SSA/Ro
• anti-SSB/La
• Sjogren's syndrome (autoimmune epitheliitis)