A case of complete atrioventricular block in neonatal lupus erythematosus.
- Author:
Kyung Sik JUNG
1
;
Sun Hye SHIN
;
Chul Woong CHOI
;
Jun Hee LEE
;
Sung Il KIM
Author Information
1. Department of Internal Medicine, Pusan National University, College of Medicine, Busan, Korea. ksimd@pusan.ac.kr
- Publication Type:Case Report
- Keywords:
Neonatal lupus erythematosus;
Congenital complete atrioventricular block
- MeSH:
Antibodies;
Atrioventricular Block*;
Autoantibodies;
Humans;
Immunoglobulin G;
Infant, Newborn;
Mortality;
Mothers;
Skin
- From:Korean Journal of Medicine
2004;67(Suppl 3):S887-S891
- CountryRepublic of Korea
- Language:Korean
-
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.
