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J Rheumatol. 2015 Jun;42(6):994-1001. doi: 10.3899/jrheum.141261. Epub 2015 Apr 15.

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

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From the Istituto Giannina Gaslini, Genoa, Italy; Karolinska University Hospital Solna, Stockholm, Sweden; Gulhane Military Medical Faculty, Ankara, Turkey; Royal Children's Hospital, Melbourne, Australia; Bakırkoy Maternity and Children Education and Research Hospital, Istanbul, Turkey; King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia; Policlinico Università di Catania, Catania, Italy; Hospital Universitário Clementino Fraga Filho, Rio de Janeiro, Brazil; Universitaetsklinik fuer Kinderheilkunde, Inselspital, Berne, Switzerland; Ospedale Policlinico, Chieti, Italy; Hospital Italiano de Buenos Aires, Buenos Aires, Argentina; Hospital Infantil de Mexico Federico Gomez, Mexico City, Mexico; University of Messina, Messina, Italy; Carolinas HealthCare System, Charlotte, North Carolina; Women and Children's Hospital, Buffalo, New York, USA; Zentrum fuer Allgemeine Paediatrie und Neonatologie, Sankt Augustin, Germany; Stanford School of Medicine, Palo Alto, California, USA; Department for Immunology and Rheumatology, Children's Hospital of Zagreb, Zagreb, Croatia; Steven and Alexandra Cohen Children's Hospital of New York, New York, New York, USA; IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy; University of Latvia, Riga, Latvia; Hospital das Clínicas, Botucatu, Brazil; King Fahad National Guard Hospital, Riyadh, Saudi Arabia; Arkansas Children's Hospital, Little Rock, Arkansas, USA; Kyriakou Children's Hospital of Athens, Athens, Greece; The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA; Children's University Hospital, Riga, Latvia; Hospital Universitario 12 de Octubre, Madrid, Spain; M. Iashvili Children's Central Clinic, Tbilisi, Georgia; Azienda Ospedaliera Universitaria Ospedali Riuniti, Ancona, Italy; Gazi University, Ankara, Turkey; Università degli Studi di Genova, Genoa, Italy; University of Alabama at Birmingham, Birmingham, Alabama, USA.F. Minoia, MD; S. Davì, MD, Istituto Giannina Gaslini; A. Horne, M



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.


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.


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.


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.



[Indexed for MEDLINE]

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