Systemic Lupus Erythematosus and Congenital Anomalies,Focusing on Neonatal Lupus Erythematosus and Anti-SS-A/SS-B Antibodies*

SLE is a representative autoimmune disease which develops preferentially in women of childbearing age. Its frequent occurrence makes the coincidence of SLE and pregnancy an important clinical problem. SLE is thought to be multifactorial disease. A familial prevalence was found to be 3% which was significantly higher when compared with Japanese prevalence of SLE. When women with SLE become pregnant, the fetus is at high risk and is adversely affected showing the high prevalence of fetal loss. Antiphospholipid antibodies were thought to be one of the causative factors. Ne-onetal lupus erythematosus (NLE) including lupus like skin lesions and congenital complete heart block (CCHB) is associated with maternal anti-SS-A and SS-B antibodies. The disease resolves with normalization of the maternal antibodies by the 8th month after delivery with the exception of the CCHB which is almost universally irriversible. When the sera from 4 maternal patients with NLE and 32 patients with normal delivery were analyzed by immunoblotting using recombinant 60 and 52 kD SS-A, and SS-B purified from calf thymus extract, the frequency of anti-52 kD SS-A and SS-B in patients with NLE was significantly higher than that of patients with normal delivery. Anti-SS-A/SS-B antibodies may react with the SS-A/SS-B antigens in fetal heart tissue as fetal levels of IgG increases which is almost simultaneously as when the SS-A/SS-B antigens appear in the fetal heart tissue. The pathogenesis of tissue damage mediated by autoimmune mechanisms specifically dependent on the biology of pregnancy is recognized to be due to passively acquired autoimmune injury. congenital complete heart block, anti-SS-A antibodies, anti-SS-B antibodies.