Neonatal Lupus Erythematosus

Extracutaneous Findings Beyond Heart Block

One percent of NLE patients develop hepatic disease. Some infants present with severe liver failure at birth, some present in the first few weeks of life with cholestasis, while others develop mild transaminase elevation weeks to months after birth (Lee, Sokol, & Buyon, 2002). In evaluating a neonate with liver disease, several entities are in the differential diagnosis. Metabolic, infectious, and inherited anatomic (extrahepatic biliary atresia) conditions must be ruled out. This leaves a large subset of infants classified as "idiopathic neonatal hepatitis." The course of this disease is spontaneous resolution with full recovery by 6 to 12 months in 80% of cases (Laxer et al., 1990). In a recent study the authors did not find a significant number of mothers of infants classified as "idiopathic neonatal hepatitis" to be anti-Ro/La positive (manuscript submitted for publication).

Thrombocytopenia or neutropenia is observed in about 10% of cases. This resolves in 2 to 3 weeks, but can be severe. One reported case of GI hemorrhage occurred as a result of thrombocytopenia. Pancytopenia, aplastic anemia, and hemolytic anemia have been rarely reported (Hariharan, Manno, & Seri, 2000; Lee & Weston, 1995.

Central nervous system disease has been rarely reported in NLE. Two children were reported (Kaye, Butler, & Conley, 1987) with neonatal lupus and myelopathy. Four children from Spain were found to have vasculopathy in the gangliothalamic vasculature by cerebral ultrasound and color Doppler flow imaging (Cabanas, Pellicer, Valverde, Morales, & Quero, 1996). These children did not have any neurologic impairment during short-term followup.

Cite this: Neonatal Lupus Erythematosus - Medscape - Jun 01, 2002.

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