Korean J Med.  2004 Nov;67(Suppl 3):S887-S891.

A case of complete atrioventricular block in neonatal lupus erythematosus

• Jung KS
• Shin SH
• Choi CW
• Lee JH
• Kim SI

Abstract

Neonatal Lupus Erythematosus (NLE) is a disease of the newborn defined by presence of maternal autoantibodies and characteristic clinical features (congenital complete atrioventricular block, skin lesion, etc.). Although the autoantibodies often are not associated with clinical disease in the mother, NLE is likely the result of fetal or neonatal tissue damage caused by maternally transmitted IgG autoantibodies. The majority of morbidity and mortality of NLE results from congenital complete atrioventricular block. Most of the neonates with NLE have maternally transmitted anti-SSA/Ro antibodies. Anti-SSB/La antibodies are found in 40 to 50 percent of patients. Anti-SSA/Ro antibodies are the most important autoantibodies in the pathophysiology of NLE. Noncardiac manifestations of neonatal lupus are transient, resolving at about 6 months of life, coinciding with the disappearance of maternal autoantibodies from neonatal circulation. Congenital complete atrioventricular block is irreversible, and permanent pacemaker insertion is needed. We report a case of NLE with congenital complete atrioventricular block with literature reviews.

Keyword

Neonatal lupus erythematosus; Congenital complete atrioventricular block

MeSH Terms

Antibodies
Atrioventricular Block*
Autoantibodies
Humans
Immunoglobulin G
Infant, Newborn
Mortality
Mothers
Skin
Antibodies
Autoantibodies
Immunoglobulin G