Connective-Tissue Disease, Antibodies to Ribonucleoprotein, and Congenital Heart Block

Abstract

The relation between congenital heart block and maternal connective-tissue disease was studied by antibody screening of serum samples obtained in connection with 45 cases of isolated congenital complete heart block. Serum was available from 41 mothers (17 who had connective-tissue disease and 24 who were healthy) and 21 children. Thirty-four mothers had antibody to a soluble tissue ribonucleoprotein antigen called Ro(SS-A), which was identified by immunodiffusion. Anti-Ro(SS-A) was found in seven of eight serum samples collected from affected children when they were less than three months old but in none of 13 samples obtained when these children were older. It appears that maternal anti-Ro(SS-A) antibody crosses the placenta and is a marker for risk of congenital complete heart block; its absence from maternal serum suggests that a child is unlikely to be affected. Anti-Ro(SS-A) or a related antibody is probably involved in the pathogenesis of congenital complete heart block.

Anti—Ro/SS‐a Antibodies in the Pathophysiology of Congenital Heart Block in Neonatal Lupus Syndrome, An Experimental Model

“…This group included 3 women with 4 normal children. Three patients had SS, 2 had S S L E overlap syndrome (1 of whom had an infant with cutaneous NLE and the other had an infant with idiopathic thrombocytopenic purpura and 2 occurrences of intrauterine fetal death), and 2 were asymptomatic. Group 111 was SLE patients (n = 14) with normal children, other serologic specificities, and no detectable antibodies to LdSS-B andor RolSS-A.…”

Neonatal lupus erythematosus: Haplotypic analysis of HLA class II alleles in child/mother pairs

“…Babies with NLE have maternal autoantibodies against Ro/SS-A and/or La/SS-B (1,2). Although previous immunogenetic studies have suggested that the frequencies of HLA-DR3 and DQ2 were increased among mothers of NLE infants in white and North American black populations (1,3), and a few investigators have questioned whether fetal HLA antigens are contributory, no prior study has addressed whether the maternal-fetal HLA relationship affects the immunopathogenesis of NLE.…”

Production of anti-Ro/SS-A and anti-La/SS-B Autoantibodies is Closely Coordinated in Systemic Lupus Erythematosus and Independent of anti-dsDNA Production

“…Anti-Ro/SS-A and anti-La/SS-B autoantibodies are amongst the few of their kind thought to be directly pathogenic: transplacental transfer of these antibodies from mother to unborn child damages the fetal cardiac conduction system resulting in congenital heart block [8,9]. Different mechanisms have been proposed to explain the development of non-organ specific autoantibodies.…”