Clinical Features, Treatment, and Outcome of Macrophage Activation Syndrome Complicating Systemic Juvenile Idiopathic Arthritis: A Multinational, Multicenter Study of 362 Patients. Issue 11 (November 2014)

• Record Type: Journal Article
• 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, Yi‐Jin; 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 and Rheumatology Research Alliance, the Pediatric Rheumatology Collaborative Study Group, and the Histiocyte Society;
• Is Part Of: Arthritis & rheumatology. Volume 66:Issue 11(2014)
• Page Start: 3160
• Page End: 3169
• Publication Date: 2014-11
• Subjects: Arthritis -- Periodicals; Rheumatism -- Periodicals; 616.72
• DOI: 10.1002/art.38802
• Languages: English
• ISSNs: 2326-5191

Abstract

<abstract abstract-type="main"> <title> <x xml:space="preserve">Abstract</x> </title> <sec id="art38802-sec-0001" sec-type="section"> <title>Objective</title> <p>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).</p> </sec> <sec id="art38802-sec-0002" sec-type="section"> <title>Methods</title> <p>In this multinational, multicenter study, pediatric rheumatologists and hemato‐oncologists entered patient data collected retrospectively into a web‐based database.</p> </sec> <sec id="art38802-sec-0003" sec-type="section"> <title>Results</title> <p>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 <sc>d</sc>‐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%.</p> </sec> <sec id="art38802-sec-0004" sec-type="section"> <title>Conclusion</title> <p>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.</p> </sec> </abstract>