TY - JOUR T1 - Dissecting the Heterogeneity of Macrophage Activation Syndrome Complicating Systemic Juvenile Idiopathic Arthritis JF - The Journal of Rheumatology JO - J Rheumatol DO - 10.3899/jrheum.141261 SP - jrheum.141261 AU - Francesca Minoia AU - Sergio Davì AU - AnnaCarin Horne AU - Francesca Bovis AU - Erkan Demirkaya AU - Jonathan Akikusa AU - Nuray A. Ayaz AU - Sulaiman M. Al-Mayouf AU - Patrizia Barone AU - Bianca Bica AU - Isabel Bolt AU - Luciana Breda AU - Carmen De Cunto AU - Sandra Enciso AU - Romina Gallizzi AU - Thomas Griffin AU - Teresa Hennon AU - Gerd Horneff AU - Michael Jeng AU - Ageza M. Kapovic AU - Jeffrey M. Lipton AU - Silvia Magni Manzoni AU - Ingrida Rumba-Rozenfelde AU - Claudia Saad Magalhaes AU - Wafaa M. Sewairi AU - Kimo C. Stine AU - Olga Vougiouka AU - Lehn K. Weaver AU - Zane Davidsone AU - Jaime De Inocencio AU - Maka Ioseliani AU - Bianca Lattanzi AU - Hasan Tezer AU - Antonella Buoncompagni AU - Paolo Picco AU - Nicolino Ruperto AU - Alberto Martini AU - Randy Q. Cron AU - Angelo Ravelli Y1 - 2015/04/15 UR - http://www.jrheum.org/content/early/2015/04/09/jrheum.141261.abstract N2 - Objective To seek insights into the heterogeneity of macrophage activation syndrome (MAS) complicating systemic juvenile idiopathic arthritis (sJIA) through the analysis of a large patient sample collected in a multinational survey. Methods International pediatric rheumatologists and hemato-oncologists entered their patient data, collected retrospectively, in a Web-based database. The demographic, clinical, laboratory, histopathologic, therapeutic, and outcome data were analyzed in relation to (1) geographic location of caring hospital, (2) subspecialty of attending physician, (3) demonstration of hemophagocytosis, and (4) severity of clinical course. Results A total of 362 patients were included by 95 investigators from 33 countries. Demographic, clinical, laboratory, and histopathologic features were comparable among patients seen in diverse geographic areas or by different pediatric specialists. Patients seen in North America were given biologics more frequently. Patients entered by pediatric hemato-oncologists were treated more commonly with biologics and etoposide, whereas patients seen by pediatric rheumatologists more frequently received cyclosporine. Patients with demonstration of hemophagocytosis had shorter duration of sJIA at MAS onset, higher prevalence of hepatosplenomegaly, lower levels of platelets and fibrinogen, and were more frequently administered cyclosporine, intravenous immunoglobulin (IVIG), and etoposide. Patients with severe course were older, had longer duration of sJIA at MAS onset, had more full-blown clinical picture, and were more commonly given cyclosporine, IVIG, and etoposide. Conclusion The clinical spectrum of MAS is comparable across patients seen in different geographic settings or by diverse pediatric subspecialists. There was a disparity in the therapeutic choices among physicians that underscores the need to establish uniform therapeutic protocols. ER -