Dissecting the heterogeneity of macrophage activation syndrome complicating systemic juvenile idiopathic arthritis

Francesca Minoia, Sergio Davì, Annacarin Horne, Francesca Bovis, Erkan Demirkaya, Jonathan Akikusa, Nuray A. Ayaz, Sulaiman M. Al-Mayouf, Patrizia Barone, Bianca Bica, Isabel Bolt, Luciana Breda, Carmen De Cunto, Sandra Enciso, Romina Gallizzi, Thomas Griffin, Teresa Hennon, Gerd Horneff, Michael Jeng, Ageza M. KapovicJeffrey M. Lipton, Silvia Magni Manzoni, Rumba Ingrida Rozenfelde, Claudia Saad Magalhaes, Wafaa M. Sewairi, Kimo C. Stine, Olga Vougiouka, Lehn K. Weaver, Zane Dāvidsone, Jaime De Inocencio, Maka Ioseliani, Bianca Lattanzi, Hasan Tezer, Antonella Buoncompagni, Paolo Picco, Nicolino Ruperto, Alberto Martini, Randy Q. Cron, Angelo Ravelli

Research output: Contribution to journalArticlepeer-review

40 Citations (Scopus)


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.

Original languageEnglish
Pages (from-to)994-1001
Number of pages8
JournalJournal of Rheumatology
Issue number6
Publication statusPublished - 1 Jun 2015
Externally publishedYes



Field of Science*

  • 3.2 Clinical medicine

Publication Type*

  • 1.1. Scientific article indexed in Web of Science and/or Scopus database


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