Neonatal lupus erythematosus with cholestatic hepatitis

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: abc1016@ms2.mmh.org.tw Neonatal lupus erythematosus (NLE) is a passive autoimmune disease in which maternal autoantibodies are transferred across the placenta. These antibodies can be detected in the affected infant for the first few months of life. The major clinical manifestations of NLE are cutaneous lupus lesions, congenital heart block, and hematologic problems (e.g. anemia, thrombocytopenia and leukopenia). During the past decade, however, it has become clear that hepatobiliary disease may also occur as a manifestation of NLE [1]. The diagnosis of NLE liver disease is based on bilirubin levels and liver enzyme abnormalities consistent with cholestasis and hepatitis in a case in which metabolic, infectious, and inherited anatomic liver abnormalities have been ruled out. We report a case of NLE hepatitis in a 55-day-old male infant.