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Neonatal Lupus Liver Disease

Department of Dermatology, University of Oklahoma Medical School, Departemnt of Medicine/Arthritis-Immunology, University of Oklahoma Medical School, Department of Veterans Affairs Medical Center, Oklahoma Medical Research Foundation, Oklahoma Center for Molecular Medicine, Oklahoma City, OK

Morris Reichlin

Departemnt of Medicine/Arthritis-Immunology, University of Oklahoma Medical School, Oklahoma Medical Research Foundation

Stephanie Z. Ruyle

Department of Pathology, University of Colorado School cof Medicine, The Children's Hospital, Denver, CO, USA

William L. Weston

Departments of Dermatology, University of Colorado School of Medicine, Department of Pediatrics, University of Colorado School of Medicine, The Children's Hospital, Denver, CO, USA

Neonatal lupus erythematosus (NLE) is an autoimmune disease characterized by complete congenital heart block and/or transient skin lesions of subacute cutaneous lupus erythematosus. We report that in approximately 10% of cases of NLE with heart block or skin disease, liver disease also occurs (4 of 35 cases in our series). Cholestasis was the major feature in our cases. Although the cholestasis may be severe, the disease process appears to be transient and surviving babies have been healthy on follow-up. In one liver examined for antibody deposition, IgG antibody deposits, presumably of maternal origin, were present. Three maternal sera were examined for autoantibodies, including liver-specific autoantibodies. No liver-specific autoantibodies were found. Rather, the maternal autoantibodies too were the ubiquitous Ro/SSA-associated autoantigens. The autoantibodies bound the 60 kDa SSA/Ro ribonuclear protein (three of three sera), the 52kDa SSA/Ro protein (two of three sera) and the SSB/La ribonuclear protein (two of three sera).

Key Words: Neonatal lupus erythematosus • Anti-SSA/Ro autoantibodies • Liver disease

Lupus, Vol. 2, No. 5, 333-338 (1993)
DOI: 10.1177/096120339300200511


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