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

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Differentiating macrophage activation syndrome in systemic juvenile idiopathic arthritis from other forms of hemophagocytic lymphohistiocytosis. / Lehmberg, Kai; Pink, Isabell; zu Eulenburg, Christine; Beutel, Karin; Maul-Pavicic, Andrea; Janka-Schaub, Gritta.

In: J PEDIATR-US, Vol. 162, No. 6, 01.06.2013, p. 1245-51.

Research output: SCORING: Contribution to journal › SCORING: Journal article › Research › peer-review

Harvard

Lehmberg, K, Pink, I, zu Eulenburg, C, Beutel, K, Maul-Pavicic, A & Janka-Schaub, G 2013, 'Differentiating macrophage activation syndrome in systemic juvenile idiopathic arthritis from other forms of hemophagocytic lymphohistiocytosis', J PEDIATR-US, vol. 162, no. 6, pp. 1245-51. https://doi.org/10.1016/j.jpeds.2012.11.081

APA

Lehmberg, K., Pink, I., zu Eulenburg, C., Beutel, K., Maul-Pavicic, A., & Janka-Schaub, G. (2013). Differentiating macrophage activation syndrome in systemic juvenile idiopathic arthritis from other forms of hemophagocytic lymphohistiocytosis. J PEDIATR-US, 162(6), 1245-51. https://doi.org/10.1016/j.jpeds.2012.11.081

Vancouver

Lehmberg K, Pink I, zu Eulenburg C, Beutel K, Maul-Pavicic A, Janka-Schaub G. Differentiating macrophage activation syndrome in systemic juvenile idiopathic arthritis from other forms of hemophagocytic lymphohistiocytosis. J PEDIATR-US. 2013 Jun 1;162(6):1245-51. https://doi.org/10.1016/j.jpeds.2012.11.081

Bibtex

@article{447f88f82734426697a6c119440bfcfa,
title = "Differentiating macrophage activation syndrome in systemic juvenile idiopathic arthritis from other forms of hemophagocytic lymphohistiocytosis",
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 1.8×10(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.",
keywords = "Arthritis, Juvenile, Biological Markers, Child, Diagnosis, Differential, Female, Humans, Lymphohistiocytosis, Hemophagocytic, Macrophage Activation Syndrome, Male",
author = "Kai Lehmberg and Isabell Pink and {zu Eulenburg}, Christine and Karin Beutel and Andrea Maul-Pavicic and Gritta Janka-Schaub",
note = "Copyright {\textcopyright} 2013 Mosby, Inc. All rights reserved.",
year = "2013",
month = jun,
day = "1",
doi = "10.1016/j.jpeds.2012.11.081",
language = "English",
volume = "162",
pages = "1245--51",
journal = "J PEDIATR-US",
issn = "0022-3476",
publisher = "Mosby Inc.",
number = "6",

}

RIS

TY - JOUR

T1 - Differentiating macrophage activation syndrome in systemic juvenile idiopathic arthritis from other forms of hemophagocytic lymphohistiocytosis

AU - Lehmberg, Kai

AU - Pink, Isabell

AU - zu Eulenburg, Christine

AU - Beutel, Karin

AU - Maul-Pavicic, Andrea

AU - Janka-Schaub, Gritta

N1 - Copyright © 2013 Mosby, Inc. All rights reserved.

PY - 2013/6/1

Y1 - 2013/6/1

N2 - 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.

AB - 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.

KW - Arthritis, Juvenile

KW - Biological Markers

KW - Child

KW - Diagnosis, Differential

KW - Female

KW - Humans

KW - Lymphohistiocytosis, Hemophagocytic

KW - Macrophage Activation Syndrome

KW - Male

U2 - 10.1016/j.jpeds.2012.11.081

DO - 10.1016/j.jpeds.2012.11.081

M3 - SCORING: Journal article

C2 - 23333131

VL - 162

SP - 1245

EP - 1251

JO - J PEDIATR-US

JF - J PEDIATR-US

SN - 0022-3476

IS - 6

ER -