Differentiating macrophage activation syndrome in systemic juvenile idiopathic arthritis from other forms of hemophagocytic lymphohistiocytosis

Kai Lehmberg*, Isabell Pink, Christine Eulenburg, Karin Beutel, Andrea Maul-Pavicic, Gritta Janka

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review

56 Citations (Scopus)

Abstract

Objectives To identify measures distinguishing macrophage activation syndrome (MAS) in systemic juvenile idiopathic arthritis (sJIA) from familial hemophagocytic lymphohistiocytosis (FHL) and virus-associated hemophagocytic lymphohistiocytosis (VA-HLH) and to define appropriate cutoff values. To evaluate suggested dynamic measures differentiating MAS in patients with sJIA from sJIA flares.

Study design In a cohort of patients referred for evaluation of hemophagocytic lymphohistiocytosis, we identified 27 patients with sJIA and MAS (MAS/sJIA) fulfilling the criteria of the proposed preliminary diagnostic guideline for the diagnosis of MAS in sJIA. Ten measures at diagnosis were compared between the MAS/sJIA group and 90 patients with FHL and 42 patients with VA-HLH, and cutoff values were determined. In addition, 5 measures were analyzed for significant change from before MAS until MAS diagnosis.

Results Neutrophil count and C-reactive protein were significantly higher in patients with MAS/sJIA compared with patients with FHL and patients with VA-HLH, with 1.8 x 10(9)/L neutrophils (sensitivity 85%, specificity 83%) and 90 mg/L C-reactive protein (74%, 89%) as cutoff values. Soluble CD25

Conclusion Readily available measures can rapidly differentiate between MAS/sJIA and FHL/VA-HLH. The findings substantiate that a decline of measures may facilitate the distinction of MAS from flares of sJIA.

Original languageEnglish
Pages (from-to)1245-1251
Number of pages7
JournalThe Journal of Pediatrics
Volume162
Issue number6
DOIs
Publication statusPublished - Jun-2013
Externally publishedYes

Keywords

  • KILLER-CELL DYSFUNCTION
  • RHEUMATOID-ARTHRITIS
  • DISEASE
  • DISORDERS
  • CHILDREN
  • MULTICENTER
  • GUIDELINES
  • DIAGNOSIS
  • SAFETY
  • GENE

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