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.