RT Journal Article
SR Electronic
T1 Dissecting the Heterogeneity of Macrophage Activation Syndrome Complicating Systemic Juvenile Idiopathic Arthritis
JF The Journal of Rheumatology
JO J Rheumatol
FD The Journal of Rheumatology
SP 994
OP 1001
DO 10.3899/jrheum.141261
VO 42
IS 6
A1 Francesca Minoia
A1 Sergio Davì
A1 AnnaCarin Horne
A1 Francesca Bovis
A1 Erkan Demirkaya
A1 Jonathan Akikusa
A1 Nuray A. Ayaz
A1 Sulaiman M. Al-Mayouf
A1 Patrizia Barone
A1 Bianca Bica
A1 Isabel Bolt
A1 Luciana Breda
A1 Carmen De Cunto
A1 Sandra Enciso
A1 Romina Gallizzi
A1 Thomas Griffin
A1 Teresa Hennon
A1 Gerd Horneff
A1 Michael Jeng
A1 Ageza M. Kapovic
A1 Jeffrey M. Lipton
A1 Silvia Magni Manzoni
A1 Ingrida Rumba-Rozenfelde
A1 Claudia Saad Magalhaes
A1 Wafaa M. Sewairi
A1 Kimo C. Stine
A1 Olga Vougiouka
A1 Lehn K. Weaver
A1 Zane Davidsone
A1 Jaime De Inocencio
A1 Maka Ioseliani
A1 Bianca Lattanzi
A1 Hasan Tezer
A1 Antonella Buoncompagni
A1 Paolo Picco
A1 Nicolino Ruperto
A1 Alberto Martini
A1 Randy Q. Cron
A1 Angelo Ravelli
YR 2015
UL http://www.jrheum.org/content/42/6/994.abstract
AB 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.