2016 Classification Criteria for Macrophage Activation Syndrome Complicating Systemic Juvenile Idiopathic Arthritis: A European League Against Rheumatism/American College of Rheumatology/Paediatric Rheumatology International Trials Organisation Collaborative Initiative

TY - JOUR

T1 - 2016 Classification Criteria for Macrophage Activation Syndrome Complicating Systemic Juvenile Idiopathic Arthritis: A European League Against Rheumatism/American College of Rheumatology/Paediatric Rheumatology International Trials Organisation Collaborative Initiative

AU - Ravelli, Angelo

AU - Minoia, Francesca

AU - Davì, Sergio

AU - Horne, AnnaCarin

AU - Bovis, Francesca

AU - Pistorio, Angela

AU - Aricò, Maurizio

AU - Avcin, Tadej

AU - Behrens, Edward M

AU - De Benedetti, Fabrizio

AU - Filipovic, Lisa

AU - Grom, Alexei A

AU - Henter, Jan-Inge

AU - Ilowite, Norman T

AU - Jordan, Michael B

AU - Khubchandani, Raju

AU - Kitoh, Toshiyuki

AU - Lehmberg, Kai

AU - Lovell, Daniel J

AU - Miettunen, Paivi

AU - Nichols, Kim E

AU - Ozen, Seza

AU - Pachlopnik Schmid, Jana

AU - Ramanan, Athimalaipet V

AU - Russo, Ricardo

AU - Schneider, Rayfel

AU - Sterba, Gary

AU - Uziel, Yosef

AU - Wallace, Carol

AU - Wouters, Carine

AU - Wulffraat, Nico

AU - Demirkaya, Erkan

AU - Brunner, Hermine I

AU - Martini, Alberto

AU - Ruperto, Nicolino

AU - Cron, Randy Q

AU - Paediatric Rheumatology International Trials Organisation, the Childhood Arthritis and Rheumatology Research Alliance, the Pediatric Rheumatology Collaborative Study Group, and the Histiocyte Society

N1 - Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/

PY - 2016/3

Y1 - 2016/3

N2 - To develop criteria for the classification of macrophage activation syndrome (MAS) in patients with systemic juvenile idiopathic arthritis (JIA). A multistep process, based on a combination of expert consensus and analysis of real patient data, was conducted. A panel of 28 experts was first asked to classify 428 patient profiles as having or not having MAS, based on clinical and laboratory features at the time of disease onset. The 428 profiles comprised 161 patients with systemic JIA-associated MAS and 267 patients with a condition that could potentially be confused with MAS (active systemic JIA without evidence of MAS, or systemic infection). Next, the ability of candidate criteria to classify individual patients as having MAS or not having MAS was assessed by evaluating the agreement between the classification yielded using the criteria and the consensus classification of the experts. The final criteria were selected in a consensus conference. Experts achieved consensus on the classification of 391 of the 428 patient profiles (91.4%). A total of 982 candidate criteria were tested statistically. The 37 best-performing criteria and 8 criteria obtained from the literature were evaluated at the consensus conference. During the conference, 82% consensus among experts was reached on the final MAS classification criteria. In validation analyses, these criteria had a sensitivity of 0.73 and a specificity of 0.99. Agreement between the classification (MAS or not MAS) obtained using the criteria and the original diagnosis made by the treating physician was high (κ=0.76). We have developed a set of classification criteria for MAS complicating systemic JIA and provided preliminary evidence of its validity. Use of these criteria will potentially improve understanding of MAS in systemic JIA and enhance efforts to discover effective therapies, by ensuring appropriate patient enrollment in studies.

AB - To develop criteria for the classification of macrophage activation syndrome (MAS) in patients with systemic juvenile idiopathic arthritis (JIA). A multistep process, based on a combination of expert consensus and analysis of real patient data, was conducted. A panel of 28 experts was first asked to classify 428 patient profiles as having or not having MAS, based on clinical and laboratory features at the time of disease onset. The 428 profiles comprised 161 patients with systemic JIA-associated MAS and 267 patients with a condition that could potentially be confused with MAS (active systemic JIA without evidence of MAS, or systemic infection). Next, the ability of candidate criteria to classify individual patients as having MAS or not having MAS was assessed by evaluating the agreement between the classification yielded using the criteria and the consensus classification of the experts. The final criteria were selected in a consensus conference. Experts achieved consensus on the classification of 391 of the 428 patient profiles (91.4%). A total of 982 candidate criteria were tested statistically. The 37 best-performing criteria and 8 criteria obtained from the literature were evaluated at the consensus conference. During the conference, 82% consensus among experts was reached on the final MAS classification criteria. In validation analyses, these criteria had a sensitivity of 0.73 and a specificity of 0.99. Agreement between the classification (MAS or not MAS) obtained using the criteria and the original diagnosis made by the treating physician was high (κ=0.76). We have developed a set of classification criteria for MAS complicating systemic JIA and provided preliminary evidence of its validity. Use of these criteria will potentially improve understanding of MAS in systemic JIA and enhance efforts to discover effective therapies, by ensuring appropriate patient enrollment in studies.

U2 - 10.1136/annrheumdis-2015-208982

DO - 10.1136/annrheumdis-2015-208982

M3 - SCORING: Journal article

C2 - 26865703

VL - 75

SP - 481

EP - 489

JO - ANN RHEUM DIS

JF - ANN RHEUM DIS

SN - 0003-4967

IS - 3

ER -