Authors
Angelo Ravelli, Francesca Minoia, Sergio Davì, AnnaCarin Horne, Francesca Bovis, Angela Pistorio, Maurizio Aricò, Tadej Avcin, Edward M Behrens, Fabrizio De Benedetti, Lisa Filipovic, Alexei A Grom, Jan‐Inge Henter, Norman T Ilowite, Michael B Jordan, Raju Khubchandani, Toshiyuki Kitoh, Kai Lehmberg, Daniel J Lovell, Paivi Miettunen, Kim E Nichols, Seza Ozen, Jana Pachlopnik Schmid, Athimalaipet V Ramanan, Ricardo Russo, Rayfel Schneider, Gary Sterba, Yosef Uziel, Carol Wallace, Carine Wouters, Nico Wulffraat, Erkan Demirkaya, Hermine I Brunner, Alberto Martini, Nicolino Ruperto, Randy Q Cron, Paediatric Rheumatology International Trials Organisation, the Childhood Arthritis and Rheumatology Research Alliance, the Pediatric Rheumatology Collaborative Study Group, and the Histiocyte Society, Mario Abinun, Amita Aggarwal, Jonathan Akikusa, Sulaiman M Al‐Mayouf, Maria Alessio, Jordi Anton, Maria Teresa Apaz, Itziar Astigarraga, Nuray A Ayaz, Patrizia Barone, Blanca Bica, Isabel Bolt, Luciana Breda, Vyacheslav Chasnyk, Rolando Cimaz, Fabrizia Corona, Ruben Cuttica, Gianfranco D'Angelo, Zane Davidsone, Carmen De Cunto, Jaime De Inocencio, Eli Eisenstein, Sandra Enciso, Graciela Espada, Michel Fischbach, Michael Frosch, Romina Gallizzi, Maria Luz Gamir, Yi‐Jin Gao, Thomas Griffin, Soad Hashad, Teresa Hennon, Gerd Horneff, Zeng Huasong, Adam Huber, Norman Ilowite, Antonella Insalaco, Maka Ioseliani, Marijia Jelusic‐Drazic, Michael Jeng, Agneza Kapovic, Ozgur Kasapcopur, Toshiyuki Kitoh, Isabelle Kone‐Paut, Sheila Knupp Feitosa de Oliveira, Bianca Lattanzi, Loredana Lepore, Caifeng Li, Jeffrey M Lipton, Silvia Magni‐Manzoni, Despoina Maritsi, Deborah McCurdy, Rosa Merino, Velma Mulaosmanovic, Susan Nielsen, Priyankar Pal, Sampath Prahalad, Donato Rigante, Ingrida Rumba‐Rozenfelde, Claudia Saad Magalhaes, Helga Sanner, Sujata Sawhney, Wafaa M Sewairi, Bita Shakoory, Susan Shenoi, Artur Silva Clovis, Valda Stanevicha, Kimo C Stine, Gordana Susic, Flavio Sztajnbok, Syuji Takei, Hasan Tezer, Ralf Trauzeddel, Elena Tsitsami, Erbil Unsal, Olga Vougiouka, Lehn K Weaver, Jennifer Weiss, Sheila Weitzman, Mabruka Zletni
Publication date
2016/3
Journal
Arthritis & rheumatology
Volume
68
Issue
3
Pages
566-576
Description
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 …
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