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Clinical Features, Treatment, and Outcome of Macrophage Activation Syndrome Complicating Systemic Juvenile Idiopathic Arthritis: A Multinational, Multicenter Study of 362 Patients
Author(s) -
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
Publication year - 2014
Publication title -
arthritis and rheumatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 4.106
H-Index - 314
eISSN - 2326-5205
pISSN - 2326-5191
DOI - 10.1002/art.38802
Subject(s) - medicine , hemophagocytosis , macrophage activation syndrome , arthritis , hemophagocytic lymphohistiocytosis , gastroenterology , bone marrow , pediatrics , disease , pancytopenia
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 (JIA). Methods In this multinational, multicenter study, pediatric rheumatologists and hemato‐oncologists entered patient data collected retrospectively into a web‐based database. Results A total of 362 patients, 22% of whom had MAS at the onset of systemic JIA, were included in the study by 95 investigators from 33 countries. The most frequent clinical manifestations were fever (96%), hepatomegaly (70%), and splenomegaly (58%). Central nervous system dysfunction and hemorrhages were recorded in 35% and 20% of the patients, respectively. Platelet count and liver transaminase, ferritin, lactate dehydrogenase, triglyceride, and d ‐dimer levels were the sole laboratory biomarkers showing a percentage change of >50% between the pre‐MAS visit and MAS onset. Evidence of macrophage hemophagocytosis was found in 60% of the patients who underwent bone marrow aspiration. 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 given to 15% and 12% of the patients, respectively. Approximately one‐third of the 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 systemic JIA–associated MAS through the analysis of a very large patient sample. MAS remains a serious condition, as a sizeable proportion of patients required admission to the ICU or died.