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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
Author(s) -
Ravelli Angelo,
Minoia Francesca,
Davì Sergio,
Horne AnnaCarin,
Bovis Francesca,
Pistorio Angela,
Aricò Maurizio,
Avcin Tadej,
Behrens Edward M.,
De Benedetti Fabrizio,
Filipovic Lisa,
Grom Alexei A.,
Henter JanInge,
Ilowite Norman T.,
Jordan Michael B.,
Khubchandani Raju,
Kitoh Toshiyuki,
Lehmberg Kai,
Lovell Daniel J.,
Miettunen Paivi,
Nichols Kim E.,
Ozen Seza,
Pachlopnik Schmid Jana,
Ramanan Athimalaipet V.,
Russo Ricardo,
Schneider Rayfel,
Sterba Gary,
Uziel Yosef,
Wallace Carol,
Wouters Carine,
Wulffraat Nico,
Demirkaya Erkan,
Brunner Hermine I.,
Martini Alberto,
Ruperto Nicolino,
Cron Randy Q.
Publication year - 2016
Publication title -
arthritis and rheumatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 4.106
H-Index - 314
eISSN - 2326-5205
pISSN - 2326-5191
DOI - 10.1002/art.39332
Subject(s) - rheumatology , league , rheumatism , medicine , macrophage activation syndrome , arthritis , juvenile , physical therapy , family medicine , physics , astronomy , biology , genetics
Objective To develop criteria for the classification of macrophage activation syndrome (MAS) in patients with systemic juvenile idiopathic arthritis (JIA). Methods 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. Results 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). Conclusion 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.