Neonatal hemochromatosis associated with maternal autoantibodies against Ro/SS-A and La/SS-B ribonucleoproteins

Am J Dis Child. 1993 Oct;147(10):1072-5. doi: 10.1001/archpedi.1993.02160340058014.


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.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Antibodies, Antinuclear / blood*
  • Autoantigens / immunology
  • Fatal Outcome
  • Female
  • Hemochromatosis / immunology*
  • Hemochromatosis / pathology
  • Humans
  • Infant, Newborn
  • Pregnancy
  • Pregnancy Complications / immunology*
  • RNA, Small Cytoplasmic*
  • Ribonucleoproteins / immunology
  • Sjogren's Syndrome / immunology*


  • Antibodies, Antinuclear
  • Autoantigens
  • RNA, Small Cytoplasmic
  • RO60 protein, human
  • Ribonucleoproteins
  • SS-A antigen
  • SS-B antigen