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neonatal lupus erythematosus
Etiology:
1) occurs in infants of mothers with systemic lupus erythematosus or Sjogren's syndrome
2) asymptomatic mothers generally develop one of these disorders
Epidemiology: rare
Clinical manifestations:
1) rash (head, neck & trunk)
2) congenital heart block
3) hepatitis
4) hemolytic anemia
Laboratory:
1) mother has anti-SSA/Ro, anti-SSB/La or both
a) necessary, but not sufficient for disease
b) 25% of pregnant with these antibodies have babies with neonatal lupus rash & 5% have babies with congenital heart block
c) maternal antibodies to RAB11FIP5
2) increased liver function tests (LFTs)
3) complete blood count (CBC)
a) thrombocytopenia
b) hemolytic anemia
Management:
1) the rash resolves when maternal IgG levels fall
2) congenital heart block
a) 1/3 of infants die before age 3
b) most of the remainder require permanent pacemakers
Related
anti-SSA/Ro
anti-SSB/La
Sjogren's syndrome (autoimmune epitheliitis)
General
systemic lupus erythematosus
neonatal disorder or disease
References
- Medical Knowledge Self Assessment Program (MKSAP) 11,15
American College of Physicians, Philadelphia 1998, 2009