Neonatal hemochromatosis associated with maternal autoantibodies against Ro/SS-A and La/SS-B ribonucleoproteins.
Liver disease in the fetus and neonate may be associated with maternal Sjögren's syndrome (neonatal lupus erythematosus). The infant of a mother with Sjögren's syndrome and high anti-Ro/SS-A and anti-La/SS-B antibody titers presented at 2 1/2 weeks of age with overwhelming hepatic insufficiency and died at 6 weeks of age. Autopsy confirmed the antemortem diagnosis of neonatal hemochromatosis. We discuss the possibility of a relationship between these two conditions.
Schoenlebe
J
J
Institut fuer Pathologie Georg Schmorl, Staedtisches Klinikum, Dresden, Germany.
Buyon
J P
JP
Zitelli
B J
BJ
Friedman
D
D
Greco
M A
MA
Knisely
A S
AS
D000328:Adult; D000974:Antibodies, Antinuclear / Q000097:blood*; D001324:Autoantigens / Q000276:immunology; D017809:Fatal Outcome; D005260:Female; D006432:Hemochromatosis / Q000276:immunology* / Q000473:pathology; D006801:Humans; D007231:Infant, Newborn; D011247:Pregnancy; D011248:Pregnancy Complications / Q000276:immunology*; D020733:RNA, Small Cytoplasmic; D012261:Ribonucleoproteins / Q000276:immunology; D012859:Sjogren's Syndrome / Q000276:immunology*; D000097585:SS-B Antigen; D000099207:SS-A Antigen
D000974:Antibodies, Antinuclear; D001324:Autoantigens; D020733:RNA, Small Cytoplasmic; D012261:Ribonucleoproteins; D000097585:SS-B Antigen; D000099207:SS-A Antigen; C116006:RO60 protein, human