Table 3.

Performance of existing criteria for HLH and MAS diagnosis.

Cohort 1, n = 34Cohort 2, n = 41All, n = 76
Cohort 1 decision rule9085
Cohort 2 decision rule9063
PReS Lupus Working Group-proposed criteria for MAS in cSLE100171003210025
Familial HLH criteriaa5086441004793
  • – Estimation of sensitivity and specificity in these cohorts would be overfit, since these cohorts gave rise to the decision rule.

  • a Restricted to patients with bone marrow aspirates/biopsies (cohort 1: n = 20; cohort 2: n = 23). Soluble interleukin receptor and natural killer cell activity were not included in sensitivity and specificity calculations. cSLE: childhood-onset systemic lupus erythematosus; HLH: hemophagocytic lymphohistiocytosis; MAS: macrophage activation syndrome; PReS: Paediatric Rheumatology European Society.