Neonatal Lupus Erythematosis, NLE (SLE, Congenital Heart Block)
Liver, skin, hematologic, neuro, renal
Transient skin rash, IUGR
Approximately 15% of infants who have NLE have hepatic involvement either alone or in association with cardiac, skin, or hematologic abnormalities.
Typically, affected infants exhibit hepatomegaly, biochemical evidence of cholestasis, and possibly elevation of transaminases.
Despite this, there do not appear to be long-term consequences of neonatal liver disease.
Thrombocytopenia, occasionally accompanied by anemia or neutropenia, occurs in as many as 10% of affected infants. Platelet numbers generally normalize over the first few weeks of life, and treatment with high-dose corticosteroids only occasionally is required. An unusual complication caused by thrombocytopenia is intracerebral hemorrhage.
Glomerulonephritis only rarely has been associated with NLE and is transient.
Treatment: Usually observe; can try short course of steroids/ or exchange transfusion
McCauliffe DP. Neonatal lupus erythematosus. In: Harper J, Oranje A,
Prose N, eds. Textbook of Pediatric Dermatology. Malden, Mass:
Blackwell Science, Ltd; 2000:111-116
Rennebohm RM. Inflammatory "noninfectious" cardiovascular diseases.
In: Emmanouilides GC, Riemenschneider TA, Allen HD, Gutgesell HP, eds.
Moss and Adams' Heart Disease in Infants, Children, and Adolescents:
Including the Fetus and Young Adult. 5th ed. Baltimore, Md: Williams &
Silverman ED, Laxer RM. Neonatal lupus erythematosus. Rheum Dis Clin
North Am. 1997;23:599-618