Reduced perforin expression in systemic juvenile idiopathic arthritis is restored by autologous stem-cell transplantation.

@article{Wulffraat2003ReducedPE,
  title={Reduced perforin expression in systemic juvenile idiopathic arthritis is restored by autologous stem-cell transplantation.},
  author={N M Wulffraat and G T Rijkers and E Elst and Rik A Brooimans and W Kuis},
  journal={Rheumatology},
  year={2003},
  volume={42 2},
  pages={375-9}
}
  • N M Wulffraat, G T Rijkers, +2 authors W Kuis
  • Published 2003 in Rheumatology
OBJECTIVES Familial haemophagocytic lymphohistiocytosis (FHL) is a disorder characterized by deficient cytotoxic T-cell function and activated macrophages, owing to a defect in the perforin gene and absent perforin expression. Because symptoms of patients with systemic juvenile idiopathic arthritis (sJIA) are sometimes clinically very similar to those with FHL, we studied whether perforin expression in sJIA patients would be reduced also. METHODS We determined the perforin expression levels… CONTINUE READING