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. Issue 3 (9th February 2016)

• Record Type: Journal Article
• Authors: 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, Jan‐Inge; 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.;
• Other Names: Abinun Mario investigator.; Aggarwal Amita investigator.; Akikusa Jonathan investigator.; Al‐Mayouf Sulaiman M. investigator.; Alessio Maria investigator.; Anton Jordi investigator.; Apaz Maria Teresa investigator.; Astigarraga Itziar investigator.; Ayaz Nuray A. investigator.; Barone Patrizia investigator.; Bica Blanca investigator.; Bolt Isabel investigator.; Breda Luciana investigator.; Chasnyk Vyacheslav investigator.; Cimaz Rolando investigator.; Corona Fabrizia investigator.; Cuttica Ruben investigator.; D'Angelo Gianfranco investigator.; Davidsone Zane investigator.; Cunto Carmen De investigator.; Inocencio Jaime De investigator.; Eisenstein Eli investigator.; Enciso Sandra investigator.; Espada Graciela investigator.; Fischbach Michel investigator.; Frosch Michael investigator.; Gallizzi Romina investigator.; Gamir Maria Luz investigator.; Gao Yi‐Jin investigator.; Griffin Thomas investigator.; Hashad Soad investigator.; Hennon Teresa investigator.; Horneff Gerd investigator.; Huasong Zeng investigator.; Huber Adam investigator.; Ilowite Norman investigator.; Insalaco Antonella investigator.; Ioseliani Maka investigator.; Jelusic‐Drazic Marijia investigator.; Jeng Michael investigator.; Kapovic Agneza investigator.; Kasapcopur Ozgur investigator.; Kitoh Toshiyuki investigator.; Kone‐Paut Isabelle investigator.; de Oliveira Sheila Knupp Feitosa investigator.; Lattanzi Bianca investigator.; Lepore Loredana investigator.; Li Caifeng investigator.; Lipton Jeffrey M. investigator.; Magni‐Manzoni Silvia investigator.; Maritsi Despoina investigator.; McCurdy Deborah investigator.; Merino Rosa investigator.; Mulaosmanovic Velma investigator.; Nielsen Susan investigator.; Pal Priyankar investigator.; Prahalad Sampath investigator.; Rigante Donato investigator.; Rumba‐Rozenfelde Ingrida investigator.; Magalhaes Claudia Saad investigator.; Sanner Helga investigator.; Sawhney Sujata investigator.; Sewairi Wafaa M. investigator.; Shakoory Bita investigator.; Shenoi Susan investigator.; Clovis Artur Silva investigator.; Stanevicha Valda investigator.; Stine Kimo C. investigator.; Susic Gordana investigator.; Sztajnbok Flavio investigator.; Takei Syuji investigator.; Tezer Hasan investigator.; Trauzeddel Ralf investigator.; Tsitsami Elena investigator.; Unsal Erbil investigator.; Vougiouka Olga investigator.; Weaver Lehn K. investigator.; Weiss Jennifer investigator.; Weitzman Sheila investigator.; Zletni Mabruka investigator.;
• Is Part Of: Arthritis & rheumatology. Volume 68:Issue 3(2016)
• Page Start: 566
• Page End: 576
• Publication Date: 2016-02-09
• Subjects: Arthritis -- Periodicals; Rheumatism -- Periodicals; 616.72
• DOI: 10.1002/art.39332
• Languages: English
• ISSNs: 2326-5191

Abstract

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.