Neonatal lupus erythematosus with cholestatic hepatitis

Sheng-Chieh Lin, Shyh-Dar Shyur, Li-Hsin Huang, Jiunn-Yi Wu, Hsun-Teh Chuo, Hung-Chang Lee
Department of Pediatrics, Mackay Memorial Hospital, Taipei, Taiwan, ROC

Received: May 12, 2003 Revised: June 18, 2003 Accepted: August 15, 2003

Corresponding author: Dr. Shyh-Dar Shyur, Department of Pediatrics, Mackay Memorial Hospital, 92, Section 2, Chung-Shan North Road, Taipei, Taiwan 104, ROC. E-mail:

Neonatal lupus erythematosus is an uncommon passive autoimmune disease in which there is transplacental passage of anti-Ro/SSA and/or anti-La/SSB or anti-U1RNP maternal autoantibodies. Its common clinical manifestations include cardiac disease, notably congenital heart block, cutaneous lupus lesions, and hematologic problems. During the past decade, it has become clear that hepatobiliary disease may also occur as a manifestation of neonatal lupus erythematosus. We report a case of neonatal lupus erythematosus in a male infant who had lupus hepatitis with jaundice in addition to cutaneous lupus, anemia, and thrombocytopenia. Other diseases in the differential diagnosis of conjugated hyperbilirubinemia, including metabolic, infectious, and inherited anatomic conditions were all ruled out. The infant had a high titer of antinuclear antibodies (titer 1:640) with a speckled pattern, anti-Ro/SSA and anti-La/SSB antibodies, and no anti-dsDNA antibodies. Treatment with prednisolone (2 mg/kg/day) for 14 days resulted in dramatic improvement of the thrombocytopenia. Hemoglobin and bilirubin returned to normal 2 months later, and transaminases were normal by 10 months of age.

Key words: Cholestasis, hepatitis, lupus erythematosus, newborn infant

J Microbiol Immunol Infect 2004;37:131-134.