Hyper-IgE Scoring Systems

  • NIH-HIES score > 30: 87.5% sensitivity, 80.6% specificity
  • NIH-HIES score > 20: possible
  • NIH-HIES score > 40: probable
  • Possible criteria
  • STAT3 score > 30
  • IgE >= 1000 IU/L
  • Probable criteria
  • Meets possible criteria
  • Lack of Th17 cells or HIES family history
  • Definitive criteria
  • Meets probably criteria
  • Dominant negative STAT3 mutation
  • Criteria: 91.4% sensitivity, 87.5% specificity
  • DOCK8 score > 30
  • NIH-HIES score > 20
  • DOCK8 score > STAT3 score
  • References