Endocardial fibroelastosis associated with maternal anti-Ro and anti-La antibodies in the absence of atrioventricular block.

Abstract

OBJECTIVES:

This study was designed to document the association of endocardial fibroelastosis (EFE) and maternal autoantibodies.

BACKGROUND:

Neonatal lupus erythematosus is associated with the transplacental passage of maternal anti-Ro and anti-La antibodies, leading to complete atrioventricular block (CAVB). In some cases, CAVB is associated with EFE. Isolated EFE may be independently related to maternal anti-Ro and anti-La antibodies.

METHODS:

We identified three cases (one fetus and two infants, all female) of isolated EFE in infants born to autoantibody-positive mothers in the absence of CAVB. Demographics, echocardiograms, and pathology were reviewed. Immunohistochemical analyses for immunoglobulin (Ig)G, IgM, IgA, T-cell, B-cell, and terminal deoxynucleoleotidyl transferase-mediated dUTP-biotin nick end-labeling (TUNEL) (test for cell apoptosis) staining were performed on multiple sections of the heart in each case and compared with negative controls.

RESULTS:

Two cases died and one received a cardiac transplant. All three cases had histologically confirmed EFE. All cases demonstrated significant diffuse IgG infiltration. To a lesser extent, myocardial tissue was also positive for IgM, CD43, and Granzyme B. None of the specimens were TUNEL positive.

CONCLUSIONS:

These are the first reported cases of isolated EFE associated with maternal anti-Ro and anti-La antibodies in the absence of CAVB. The diffuse deposition of IgG and the presence of a T-cell infiltrate throughout the myocardium suggest that the transplacental passage of maternal autoantibodies induces an immune reaction within the myocardium, leading to isolated EFE. Autoantibody-mediated EFE may be an etiologic factor in cases of fetal and neonatal "idiopathic" dilated cardiomyopathy.

• Authors: Lynne E Nield, Earl D Silverman, Jeffrey F Smallhorn, Glenn P Taylor, J Brendan M Mullen, Leland N Benson, Lisa K Hornberger
• Journal: Journal of the American College of Cardiology (J Am Coll Cardiol) Vol. 40 Issue 4 Pg. 796-802 (Aug 21 2002) ISSN: 0735-1097 [Print] United States
• PMID: 12204513 (Publication Type: Case Reports, Journal Article, Research Support, Non-U.S. Gov't)

Chemical References

• Antibodies, Antinuclear
• Autoantibodies
• Autoantigens
• Ribonucleoproteins
• SS-A antibodies
• SS-B antigen

Topics

• Antibodies, Antinuclear
• Autoantibodies
• Autoantigens (immunology)
• Endocardial Fibroelastosis (immunology, pathology)
• Female
• Fetus (immunology)
• Humans
• Infant
• Lupus Erythematosus, Systemic (immunology)
• Maternal-Fetal Exchange
• Myocardium (immunology, pathology)
• Pregnancy
• Ribonucleoproteins (immunology)