NEONATAL LUPUS LIVER-DISEASE

Neonatal lupus erythematosus (NLE) is an autoimmune disease characteri zed by complete congenital heart block and/or transient skin lesions o f subacute cutaneous lupus erythematosus. We report that in approximat ely 10% of cases of NLE with heart block or skin disease, liver diseas e also occurs (4 of 35 cases in our series). Cholestasis was the major feature in our cases. Although the cholestasis may be severe, the dis ease process appears to be transient and surviving babies have been he althy 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-speci fic autoantibodies. No liver-specific autoantibodies were found. Rathe r, the maternal autoantibodies too were the ubiquitous Ro/SSA-associat ed autoantigens. The autoantibodies bound the 60 kDa SSA/Ro ribonuclea r protein (three of three sera), the 52 kDa SSA/Ro protein (two of thr ee sera) and the SSB/LA ribonuclear protein (two of three sera).