Paediatrics Publications

Title

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

Authors

Angelo Ravelli, Università degli Studi di Genova
Francesca Minoia, Università degli Studi di Genova
Sergio Davì, Università degli Studi di Genova
Annacarin Horne, Karolinska Universitetssjukhuset
Francesca Bovis, Università degli Studi di Genova
Angela Pistorio, Università degli Studi di Genova
Maurizio Aricò
Tadej Avcin, University Children's Hospital, Ljubljana
Edward M. Behrens, The Children's Hospital of Philadelphia
Fabrizio De Benedetti, IRCCS Ospedale Pediatrico Bambino Gesù
Lisa Filipovic, Cincinnati Children's Hospital Medical Center
Alexei A. Grom, Cincinnati Children's Hospital Medical Center
Jan Inge Henter, Karolinska Universitetssjukhuset
Norman T. Ilowite, The Childen's Hospital at Montefiore
Michael B. Jordan, Cincinnati Children's Hospital Medical Center
Raju Khubchandani, Jaslok Hospital and Research Centre
Toshiyuki Kitoh, Aichi Medical University
Kai Lehmberg, Universitätsklinikum Hamburg-Eppendorf
Daniel J. Lovell, Cincinnati Children's Hospital Medical Center
Paivi Miettunen, University of Calgary
Kim E. Nichols, St. Jude Children's Research Hospital
Seza Ozen, Hacettepe Üniversitesi
Jana Pachlopnik Schmid, Kinderspital Zürich
Athimalaipet V. Ramanan, Bristol Royal Hospital for Children
Ricardo Russo, Fundacion Hospital de Pediatria Professor Dr. Juan P. Garrahan
Rayfel Schneider, Hospital for Sick Children University of Toronto
Gary Sterba, Mount Sinai Medical Center Miami Beach
Yosef Uziel, Meir Medical Center
Carol Wallace, University of Washington

Document Type

Article

Publication Date

3-1-2016

Journal

Arthritis and Rheumatology

Volume

68

Issue

3

First Page

566

Last Page

576

URL with Digital Object Identifier

10.1002/art.39332

Abstract

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.

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