Title: Clinical features, treatment and outcome of macrophage activation syndrome complicating systemic juvenile idiopathic arthritis A multinational, multicenter study of 362 patients
Authors: Minoia, Francesca ×
Davì, Sergio
Horne, AnnaCarin
Demirkaya, Erkan
Bovis, Francesca
Li, Caifeng
Lehmberg, Kai
Weitzman, Sheila
Insalaco, Antonella
Wouters, Carine
Shenoi, Susan
Espada, Graciela
Ozen, Seza
Anton, Jordi
Khubchandani, Raju
Russo, Ricardo
Pal, Priyankar
Kasapcopur, Ozgur
Miettunen, Paivi
Maritsi, Despoina
Merino, Rosa
Shakoory, Bita
Alessio, Maria
Chasnyk, Vyacheslav
Sanner, Helga
Gao, Yijin
Huasong, Zeng
Kitoh, Toshiyuki
Avcin, Tadej
Fischbach, Michel
Frosch, Michael
Grom, Alexei
Huber, Adam
Jelusic, Marija
Sawhney, Sujata
Uziel, Yosef
Ruperto, Nicolino
Martini, Alberto
Cron, Randy Q
Ravelli, Angelo
on behalf of the Pediatric Rheumatology International Trials Organization
the Childhood Arthritis & Rheumatology Research Alliance
the Pediatric Rheumatology Collaborative Study Group, and the Histiocyte Society #
Issue Date: Nov-2014
Publisher: Wiley
Series Title: Arthritis & Rheumatology vol:66 issue:11 pages:3160-3169
Article number: 10.1002/art.38802
Abstract: Objective. To describe the clinical, laboratory and histopathologic features, current treatment and outcome of patients with macrophage activation syndrome (MAS) complicating systemic juvenile idiopathic arthritis (sJIA). Methods. In this multinational, multicenter study, international pediatric rheumatologists and hemato-oncologists entered their patient data collected retrospectively in a web-based database. Results. A total of 362 patients, 22% of whom had MAS at sJIA onset, were included in the study by 95 investigators from 33 countries. The most frequent clinical manifestations were fever (96%), hepatomegaly (70%) and splenomegaly (58%); CNS dysfunction and hemorrhages were recorded in 35% and 20% of patients, respectively. Platelet count, liver transaminases, ferritin, lactate dehydrogenase, triglycerides and D-dimer levels were the sole laboratory biomarkers showing a percentage change > 50% between pre-MAS visit and MAS onset. Evidence of macrophage hemophagocytosis was found in 60% of patients who underwent bone marrow aspirate. MAS occurred most frequently in the setting of active underlying disease, in the absence of a specific trigger. Nearly all patients were given corticosteroids and 61% received cyclosporine; biologic medications and etoposide were used in 15% and 12% of cases, respectively. Around one-third of patients required admission to the intensive care unit (ICU), and the mortality rate was 8%. Conclusion. This study provides information on the clinical spectrum and current management of sJIA-associated MAS through the analysis of a very large patient sample. MAS remains a serious condition as a sizeable proportion of patients required ICU admission or died. © 2014 American College of Rheumatology.
ISSN: 2326-5191
Publication status: published
KU Leuven publication type: IT
Appears in Collections:Laboratory of Pediatric Immunology
× corresponding author
# (joint) last author

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