Naslov
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 Collaborat

Autori
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 ; Lovel, lDaniel 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. ; Abinun, Mario ; Aggarwal, Amita ; Akikusa, Jonathan ; Al‐Mayouf, Sulaiman M. ; Alessio, Maria ; Anton, Jordi ; Apaz, Maria Teresa ; Astigarraga, Itziar ; Ayaz, Nuray A. ; Barone, Patrizia ; Bica, Blanca ; Bolt, Isabel ; Breda, Luciana ; Chasnyk, Vyacheslav ; Cimaz, Rolando ; Corona, Fabrizia ; Cuttica, Ruben ; D'Angelo, Gianfranco ; Davidsone, Zane ; De Cunto, Carmen ; De Inocencio, Jaime ; Eisenstein, Eli ; Enciso, Sandra ; Espada, Graciela ; Fischbach, Michel ; Frosch, Michael ; Gallizzi, Romina ; Luz Gamir, Maria ; Gao, Yi‐Jin ; Griffin, Thomas ; Hashad, Soad ; Hennon, Teresa ; Horneff, Gerd ; Huasong, Zeng ; Huber, Adam ; Ilowite, Norman ; Insalaco, Antonella ; Ioseliani, Maka ; Jelusic‐Drazic, Marija ; Jeng, Michael ; Kapovic, Agneza ; Kasapcopur, Ozgur ; Kitoh, Toshiyuki ; Kone‐Paut, Isabelle ; de Oliveira, Sheila Knupp Feitosa ; Lattanzi, Bianca ; Lepore, Loredana ; Li, Caifeng ; Lipton, Jeffrey M. ; Magni‐Manzoni, Silvia ; Maritsi, Despoina ; McCurdy, Deborah ; Merino, Rosa ; Mulaosmanovic, Velma ; Nielsen, Susan ; Pal, Priyankar ; Prahalad, Sampath ; Rigante, Donato ; Rumba‐Rozenfelde, Ingrida ; Saad Magalhaes, Claudia ; Sanner, Helga ; Sawhney, Sujata ; Sewairi, Wafaa M. ; Shakoory, Bita ; Shenoi, Susan ; Clovis, Artur Silva ; Stanevicha, Valda ; Stine, Kimo C. ; Susic, Gordana ; Sztajnbok, Flavio ; Takei, Syuji ; Tezer, Hasan ; Trauzeddel, Ralf ; Tsitsami, Elena ; Unsal, Erbil ; Vougiouka, Olga ; Weaver, Lehn K. ; Weiss, Jennifer ; Weitzman, Sheila ; Zletni, Mabruka

Izvornik
Arthritis & Rheumatology (2326-5191) 68 (2016), 3; 566-576

Vrsta, podvrsta i kategorija rada
Radovi u časopisima, članak, znanstveni

Ključne riječi
Classification Criteria ; Macrophage Activation Syndrome ; Systemic Juvenile Idiopathic Arthritis ; European League Against Rheumatism/American College of Rheumatology ; Paediatric Rheumatology International Trials Organisation Collaborative Initiative

Sažetak
Objective.To develop criteria for the classificationof macrophage activation syndrome (MAS) in patientswith systemic juvenile idiopathic arthritis (JIA).Methods.A multistep process, based on a combi-nation of expert consensus and analysis of real patientdata, was conducted. A panel of 28 experts was firstasked to classify 428 patient profiles as having or nothaving MAS, based on clinical and laboratory featuresat the time of disease onset. The 428 profiles comprised161 patients with systemic JIA–associated MAS and267 patients with a condition that could potentially beconfused with MAS (active systemic JIA without evi-dence of MAS, or systemic infection). Next, the abilityof candidate criteria to classify individual patients ashaving MAS or not having MAS was assessed by evalu-ating the agreement between the classification yieldedusing the criteria and the consensus classification ofthe experts. The final criteria were selected in a consen-sus conference.Results.Experts achieved consensus on the clas-sification 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 obtainedfrom the literature were evaluated at the consensus con-ference. During the conference, 82% consensus amongexperts was reached on the final MAS classification cri-teria. In validation analyses, these criteria had a sensitiv-ity of 0.73 and a specificity of 0.99. Agreement betweenthe classification (MAS or not MAS) obtained using thecriteria and the original diagnosis made by the treatingphysician was high (k50.76).Conclusion.We have developed a set of classifi-cation criteria for MAS complicating systemic JIA andprovided preliminary evidence of its validity. Use ofthese criteria will potentially improve understanding ofMAS in systemic JIA and enhance efforts to discovereffective therapies, by ensuring appropriate patientenrollment in studies.

Izvorni jezik
Engleski

Znanstvena područja
Kliničke medicinske znanosti

POVEZANOST RADA