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Systemic juvenile idiopathic arthritis and macrophage activation syndrome

update on pathogenesis and treatment

Yasin, Shima; Schulert, Grant S.

doi: 10.1097/BOR.0000000000000526

Purpose of review The past decade has seen substantial progress in defining the cause and pathogenesis of the chronic childhood arthropathy systemic juvenile idiopathic arthritis (SJIA) and its related complication macrophage activation syndrome (MAS). The purpose of this review is to describe and synthesize advances in this field, particularly since 2016, with the potential to transform clinical practice.

Recent findings Newly developed MAS classification criteria have been further studied and validated in other diseases and populations, as well as a recently proposed score to distinguish MAS from familial hemophagocytic lymphohistiocytosis. There has also been substantial progress toward understanding the genetic underpinnings of SJIA and MAS, both through targeted study of specific genes and the results of a large genome-wide association study. The immunopathogenesis of SJIA has been further elucidated through several studies regarding the proinflammatory cytokines interleukin-18, interferon (IFN)γ, and how their interplay impacts emergence of MAS. Finally, big data studies integrating genomic information with immunophenotypes have potential to provide novel insights into disease mechanisms in SJIA.

Summary Collectively, these research advances have significant implications regarding the classification and diagnosis of SJIA and MAS, and support a next generation of biologic treatments including kinase inhibitors and targeted interleukin-18 or IFNγ blockade.

Division of Rheumatology, Cincinnati Children's Hospital Medical Center and Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio, USA

Correspondence to Grant S. Schulert, MD, PhD, Division of Rheumatology, MLC 4010, Children's Hospital Medical Center, 3333 Burnet Ave, Cincinnati, OH 45229, USA. Tel: +513 636 4676; fax: +513 636 3328; e-mail:

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