Development and initial validation of the MS score for diagnosis of macrophage activation syndrome in systemic juvenile idiopathic arthritis

Pediatric Rheumatology international Trials Organization, the Childhood arthritis &Rheumatology Research alliance, the Pediatric Rheumatology Collaborative study Group and the Histiocyte society

Research output: Contribution to journalArticle

6 Citations (Scopus)

Abstract

Objective To develop and validate a diagnostic score that aids in identifying macrophage activation syndrome (MAS) in patients with systemic juvenile idiopathic arthritis (sJIA). Methods The clinical and laboratory features of 362 patients with sJIA-associated MAS and 404 patients with active sJIA without evidence of MAS were collected in a multinational collaborative project. Eighty percent of the study population was used to develop the score and the remaining 20% constituted the validation sample. A Bayesian Model Averaging approach was used to assess the role of each clinical and laboratory variables in the diagnosis of MAS and to obtain the coefficients of selected variables. The final score, named MAS/sJIA (MS) score, resulted from the linear combination of these coefficients multiplied by the values of each variable. The cut-off that best discriminated MAS from active sJIA was calculated by means of receiver operating characteristic (ROC) curve analysis. Score performance was evaluated in both developmental and validation samples. Results The MS score ranges from-8.4 to 41.8 and comprises seven variables: central nervous system dysfunction, haemorrhagic manifestations, active arthritis, platelet count, fibrinogen, lactate dehydrogenase and ferritin. A cut-off value ≥-2.1 revealed the best performance in discriminating MAS from active sJIA, with a sensitivity of 0.85, a specificity of 0.95 and a kappa value of 0.80. The good performance of the MS score was confirmed in the validation sample. Conclusion The MS score is a powerful and feasible tool that may assist practitioners in making a timely diagnosis of MAS in patients with sJIA.

Original languageEnglish
Pages (from-to)1357-1362
Number of pages6
JournalAnnals of the Rheumatic Diseases
Volume78
Issue number10
DOIs
Publication statusPublished - Jan 1 2019

Fingerprint

Macrophage Activation Syndrome
Juvenile Arthritis
Macrophages
Chemical activation
Neurology
Ferritins
Platelets
Platelet Count
L-Lactate Dehydrogenase
ROC Curve
Fibrinogen
Arthritis
Central Nervous System

Keywords

  • diagnostic score
  • hemophagocytic syndrome
  • macrophage activation syndrome
  • still's disease
  • systemic juvenile idiopathic arthritis

ASJC Scopus subject areas

  • Rheumatology
  • Immunology and Allergy
  • Immunology
  • Biochemistry, Genetics and Molecular Biology(all)

Cite this

Pediatric Rheumatology international Trials Organization, the Childhood arthritis &Rheumatology Research alliance, the Pediatric Rheumatology Collaborative study Group and the Histiocyte society (2019). Development and initial validation of the MS score for diagnosis of macrophage activation syndrome in systemic juvenile idiopathic arthritis. Annals of the Rheumatic Diseases, 78(10), 1357-1362. https://doi.org/10.1136/annrheumdis-2019-215211

Development and initial validation of the MS score for diagnosis of macrophage activation syndrome in systemic juvenile idiopathic arthritis. / Pediatric Rheumatology international Trials Organization, the Childhood arthritis &Rheumatology Research alliance, the Pediatric Rheumatology Collaborative study Group and the Histiocyte society.

In: Annals of the Rheumatic Diseases, Vol. 78, No. 10, 01.01.2019, p. 1357-1362.

Research output: Contribution to journalArticle

Pediatric Rheumatology international Trials Organization, the Childhood arthritis &Rheumatology Research alliance, the Pediatric Rheumatology Collaborative study Group and the Histiocyte society 2019, 'Development and initial validation of the MS score for diagnosis of macrophage activation syndrome in systemic juvenile idiopathic arthritis', Annals of the Rheumatic Diseases, vol. 78, no. 10, pp. 1357-1362. https://doi.org/10.1136/annrheumdis-2019-215211
Pediatric Rheumatology international Trials Organization, the Childhood arthritis &Rheumatology Research alliance, the Pediatric Rheumatology Collaborative study Group and the Histiocyte society. Development and initial validation of the MS score for diagnosis of macrophage activation syndrome in systemic juvenile idiopathic arthritis. Annals of the Rheumatic Diseases. 2019 Jan 1;78(10):1357-1362. https://doi.org/10.1136/annrheumdis-2019-215211
Pediatric Rheumatology international Trials Organization, the Childhood arthritis &Rheumatology Research alliance, the Pediatric Rheumatology Collaborative study Group and the Histiocyte society. / Development and initial validation of the MS score for diagnosis of macrophage activation syndrome in systemic juvenile idiopathic arthritis. In: Annals of the Rheumatic Diseases. 2019 ; Vol. 78, No. 10. pp. 1357-1362.
@article{7ac04178449e4ed89fb8d4b535f0dcb3,
title = "Development and initial validation of the MS score for diagnosis of macrophage activation syndrome in systemic juvenile idiopathic arthritis",
abstract = "Objective To develop and validate a diagnostic score that aids in identifying macrophage activation syndrome (MAS) in patients with systemic juvenile idiopathic arthritis (sJIA). Methods The clinical and laboratory features of 362 patients with sJIA-associated MAS and 404 patients with active sJIA without evidence of MAS were collected in a multinational collaborative project. Eighty percent of the study population was used to develop the score and the remaining 20{\%} constituted the validation sample. A Bayesian Model Averaging approach was used to assess the role of each clinical and laboratory variables in the diagnosis of MAS and to obtain the coefficients of selected variables. The final score, named MAS/sJIA (MS) score, resulted from the linear combination of these coefficients multiplied by the values of each variable. The cut-off that best discriminated MAS from active sJIA was calculated by means of receiver operating characteristic (ROC) curve analysis. Score performance was evaluated in both developmental and validation samples. Results The MS score ranges from-8.4 to 41.8 and comprises seven variables: central nervous system dysfunction, haemorrhagic manifestations, active arthritis, platelet count, fibrinogen, lactate dehydrogenase and ferritin. A cut-off value ≥-2.1 revealed the best performance in discriminating MAS from active sJIA, with a sensitivity of 0.85, a specificity of 0.95 and a kappa value of 0.80. The good performance of the MS score was confirmed in the validation sample. Conclusion The MS score is a powerful and feasible tool that may assist practitioners in making a timely diagnosis of MAS in patients with sJIA.",
keywords = "diagnostic score, hemophagocytic syndrome, macrophage activation syndrome, still's disease, systemic juvenile idiopathic arthritis",
author = "{Pediatric Rheumatology international Trials Organization, the Childhood arthritis &Rheumatology Research alliance, the Pediatric Rheumatology Collaborative study Group and the Histiocyte society} and Francesca Minoia and Francesca Bovis and Sergio Dav{\`i} and Horne, {Anna Carin} and Michel Fischbach and Michael Frosch and Adam Huber and Marija Jelusic and Sujata Sawhney and McCurdy, {Deborah K.} and Silva, {Cl{\'o}vis A.} and Donato Rigante and Erbil Unsal and Nicolino Ruperto and Alberto Martini and Cron, {Randy Q.} and Angelo Ravelli",
year = "2019",
month = "1",
day = "1",
doi = "10.1136/annrheumdis-2019-215211",
language = "English",
volume = "78",
pages = "1357--1362",
journal = "Annals of the Rheumatic Diseases",
issn = "0003-4967",
publisher = "BMJ Publishing Group",
number = "10",

}

TY - JOUR

T1 - Development and initial validation of the MS score for diagnosis of macrophage activation syndrome in systemic juvenile idiopathic arthritis

AU - Pediatric Rheumatology international Trials Organization, the Childhood arthritis &Rheumatology Research alliance, the Pediatric Rheumatology Collaborative study Group and the Histiocyte society

AU - Minoia, Francesca

AU - Bovis, Francesca

AU - Davì, Sergio

AU - Horne, Anna Carin

AU - Fischbach, Michel

AU - Frosch, Michael

AU - Huber, Adam

AU - Jelusic, Marija

AU - Sawhney, Sujata

AU - McCurdy, Deborah K.

AU - Silva, Clóvis A.

AU - Rigante, Donato

AU - Unsal, Erbil

AU - Ruperto, Nicolino

AU - Martini, Alberto

AU - Cron, Randy Q.

AU - Ravelli, Angelo

PY - 2019/1/1

Y1 - 2019/1/1

N2 - Objective To develop and validate a diagnostic score that aids in identifying macrophage activation syndrome (MAS) in patients with systemic juvenile idiopathic arthritis (sJIA). Methods The clinical and laboratory features of 362 patients with sJIA-associated MAS and 404 patients with active sJIA without evidence of MAS were collected in a multinational collaborative project. Eighty percent of the study population was used to develop the score and the remaining 20% constituted the validation sample. A Bayesian Model Averaging approach was used to assess the role of each clinical and laboratory variables in the diagnosis of MAS and to obtain the coefficients of selected variables. The final score, named MAS/sJIA (MS) score, resulted from the linear combination of these coefficients multiplied by the values of each variable. The cut-off that best discriminated MAS from active sJIA was calculated by means of receiver operating characteristic (ROC) curve analysis. Score performance was evaluated in both developmental and validation samples. Results The MS score ranges from-8.4 to 41.8 and comprises seven variables: central nervous system dysfunction, haemorrhagic manifestations, active arthritis, platelet count, fibrinogen, lactate dehydrogenase and ferritin. A cut-off value ≥-2.1 revealed the best performance in discriminating MAS from active sJIA, with a sensitivity of 0.85, a specificity of 0.95 and a kappa value of 0.80. The good performance of the MS score was confirmed in the validation sample. Conclusion The MS score is a powerful and feasible tool that may assist practitioners in making a timely diagnosis of MAS in patients with sJIA.

AB - Objective To develop and validate a diagnostic score that aids in identifying macrophage activation syndrome (MAS) in patients with systemic juvenile idiopathic arthritis (sJIA). Methods The clinical and laboratory features of 362 patients with sJIA-associated MAS and 404 patients with active sJIA without evidence of MAS were collected in a multinational collaborative project. Eighty percent of the study population was used to develop the score and the remaining 20% constituted the validation sample. A Bayesian Model Averaging approach was used to assess the role of each clinical and laboratory variables in the diagnosis of MAS and to obtain the coefficients of selected variables. The final score, named MAS/sJIA (MS) score, resulted from the linear combination of these coefficients multiplied by the values of each variable. The cut-off that best discriminated MAS from active sJIA was calculated by means of receiver operating characteristic (ROC) curve analysis. Score performance was evaluated in both developmental and validation samples. Results The MS score ranges from-8.4 to 41.8 and comprises seven variables: central nervous system dysfunction, haemorrhagic manifestations, active arthritis, platelet count, fibrinogen, lactate dehydrogenase and ferritin. A cut-off value ≥-2.1 revealed the best performance in discriminating MAS from active sJIA, with a sensitivity of 0.85, a specificity of 0.95 and a kappa value of 0.80. The good performance of the MS score was confirmed in the validation sample. Conclusion The MS score is a powerful and feasible tool that may assist practitioners in making a timely diagnosis of MAS in patients with sJIA.

KW - diagnostic score

KW - hemophagocytic syndrome

KW - macrophage activation syndrome

KW - still's disease

KW - systemic juvenile idiopathic arthritis

UR - http://www.scopus.com/inward/record.url?scp=85070296112&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=85070296112&partnerID=8YFLogxK

U2 - 10.1136/annrheumdis-2019-215211

DO - 10.1136/annrheumdis-2019-215211

M3 - Article

C2 - 31296501

AN - SCOPUS:85070296112

VL - 78

SP - 1357

EP - 1362

JO - Annals of the Rheumatic Diseases

JF - Annals of the Rheumatic Diseases

SN - 0003-4967

IS - 10

ER -