Hepatobiliary disease in neonatal lupus: prevalence and clinical characteristics in cases enrolled in a national registry.

OBJECTIVE

To extend the information base on the hepatobiliary manifestations of neonatal lupus erythematosus (NLE) with regard to frequency of occurrence, clinical characteristics, and outcome.

METHODS

Review of records from the Research Registry for Neonatal Lupus.

RESULTS

Nineteen (9%) of 219 patients who had NLE and were enrolled in a national registry had probable or possible NLE hepatobiliary disease. In 16 cases, hepatobiliary disease occurred in addition to cardiac or cutaneous NLE. In 3 cases, hepatobiliary disease occurred as the sole clinical manifestation of NLE. Three clinical variants of hepatobiliary disease were observed: 1) severe liver failure present during gestation or in the neonatal period, often with the phenotype of neonatal iron storage disease; 2) conjugated hyperbilirubinemia with mild or no elevations of aminotransferases, occurring in the first few weeks of life; and 3) mild elevations of aminotransferases occurring at approximately 2 to 3 months of life. The prognosis for the children in the last 2 categories is excellent.

CONCLUSIONS

Hepatobiliary disease is a relatively common finding in NLE and can be the sole clinical manifestation of NLE. Clinicians should be aware of the broad range of hepatobiliary disease that may occur in children with NLE.