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 1.8×10(9)/L neutrophils (sensitivity 85%, specificity 83%) and 90 mg/L C-reactive protein (74%, 89%) as cutoff values. Soluble CD25<7900 U/L (79%, 76%) indicated MAS/sJIA rather than FHL/VA-HLH. Platelet (-59%) and white blood cell count (-46%) displayed a significant decrease, and neutrophil count (-35%) and fibrinogen (-28%) showed a trend during the development of MAS. However, a substantial portion of patients had values at diagnosis of MAS within or above the normal range for white blood cells (84%), neutrophils (77%), platelets (26%), and fibrinogen (71%). 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.
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 1.8×10(9)/L neutrophils (sensitivity 85%, specificity 83%) and 90 mg/L C-reactive protein (74%, 89%) as cutoff values. Soluble CD25<7900 U/L (79%, 76%) indicated MAS/sJIA rather than FHL/VA-HLH. Platelet (-59%) and white blood cell count (-46%) displayed a significant decrease, and neutrophil count (-35%) and fibrinogen (-28%) showed a trend during the development of MAS. However, a substantial portion of patients had values at diagnosis of MAS within or above the normal range for white blood cells (84%), neutrophils (77%), platelets (26%), and fibrinogen (71%). 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.
Authors: Thomas N Burn; Lehn Weaver; Julia E Rood; Niansheng Chu; Aaron Bodansky; Portia A Kreiger; Edward M Behrens Journal: Arthritis Rheumatol Date: 2019-12-15 Impact factor: 10.995
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Authors: Sebastian Fn Bode; Sandra Ammann; Waleed Al-Herz; Mihaela Bataneant; Christopher C Dvorak; Stephan Gehring; Andrew Gennery; Kimberly C Gilmour; Luis I Gonzalez-Granado; Ute Groß-Wieltsch; Marianne Ifversen; Jenny Lingman-Framme; Susanne Matthes-Martin; Rolf Mesters; Isabelle Meyts; Joris M van Montfrans; Jana Pachlopnik Schmid; Sung-Yun Pai; Pere Soler-Palacin; Uta Schuermann; Volker Schuster; Markus G Seidel; Carsten Speckmann; Polina Stepensky; Karl-Walter Sykora; Bianca Tesi; Thomas Vraetz; Catherine Waruiru; Yenan T Bryceson; Despina Moshous; Kai Lehmberg; Michael B Jordan; Stephan Ehl Journal: Haematologica Date: 2015-05-28 Impact factor: 9.941
Authors: R Lee Reinhardt; Hong-Erh Liang; Katherine Bao; April E Price; Markus Mohrs; Ben L Kelly; Richard M Locksley Journal: J Immunol Date: 2015-01-30 Impact factor: 5.422