Autoantibodies in mothers of children with neonatal liver disease.
OBJECTIVE
Neonatal lupus erythematosus (NLE) is associated with maternal anti-Ro/La autoantibodies. It is characterized by heart block and/or cutaneous skin lesions, and occasionally liver disease. This study was performed to determine whether idiopathic neonatal cholestasis (INC) represents NLE without its cardiac or cutaneous findings.


METHODS
Sera were obtained for autoantibody analysis from mothers of children with INC (N = 11), biliary atresia (N = 25), other liver disease excluding viral hepatitis (liver disease control subjects, N = 14), and healthy children (normal control subjects [NC], N = 22).


RESULTS
The characteristic serologic findings of NLE, high titer antibodies to Ro and/or La, were absent in mothers from all groups. An unexpected finding was the prevalence of autoantibodies in mothers of infants with liver disease of any type. The frequency of maternal antinuclear antibodies at > or = 1:120 dilution was greater than the estimated frequency in the general population (22% vs. 9%, P = 0.044). The frequency of maternal low titer autoantibodies to 52 kD Ro detected by ELISA was significantly greater than in the NC group (31% vs. 5%, P = 0.014).


CONCLUSIONS
The majority of cases of INC do not represent NLE. The frequent presence of autoantibodies in mothers of infants in all neonatal liver disease groups raises the possibility that maternal serologic autoimmunity is associated with neonatal liver disease.
Burch
Joanna M
JM
Department of Dermatology, University of Colorado School of Medicine, CO, USA.
Sokol
Ronald J
RJ
Narkewicz
Michael R
MR
Reichlin
Morris
M
Frank
Mark Barton
MB
MacKenzie
Todd
T
Lee
Lela A
LA
D000328:Adult; D000974:Antibodies, Antinuclear / Q000032:analysis / Q000097:blood; D001323:Autoantibodies / Q000032:analysis / Q000097:blood*; D001656:Biliary Atresia / Q000097:blood / Q000276:immunology*; D016022:Case-Control Studies; D002648:Child; D002675:Child, Preschool; D002779:Cholestasis / Q000097:blood / Q000276:immunology*; D004797:Enzyme-Linked Immunosorbent Assay; D005260:Female; D006801:Humans; D007112:Immunity, Maternally-Acquired; D007223:Infant; D007231:Infant, Newborn; D008107:Liver Diseases / Q000097:blood / Q000276:immunology; D008178:Lupus Erythematosus, Cutaneous / Q000097:blood / Q000276:immunology*; D011247:Pregnancy
D000974:Antibodies, Antinuclear; D001323:Autoantibodies; C035356:SS-A antibodies; C035357:SS-B antibodies