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Systemic juvenile idiopathic arthritis: Clinical manifestations and diagnosis

Author
Yukiko Kimura, MD
Section Editor
Marisa Klein-Gitelman, MD, MPH
Deputy Editor
Elizabeth TePas, MD, MS

INTRODUCTION

Systemic juvenile idiopathic arthritis (sJIA, formerly called Still's disease or systemic juvenile rheumatoid arthritis) is officially classified as a subset of JIA that includes patients characterized by daily quotidian fever, rash, and arthritis. It is termed "adult-onset Still's disease" when it begins in patients over the age of 16 years. sJIA accounts for approximately 10 to 20 percent of all cases of JIA. It typically affects both sexes equally and may present in children as young as one year of age or younger. (See "Juvenile idiopathic arthritis: Epidemiology and immunopathogenesis".)

The clinical manifestations and diagnosis of sJIA are reviewed here (table 1). The management, complications, and prognosis of sJIA, as well as oligoarticular, polyarticular, enthesitis-related, and psoriatic JIA, and general topics on JIA are discussed separately. (See "Systemic juvenile idiopathic arthritis: Treatment" and "Systemic juvenile idiopathic arthritis: Course, prognosis, and complications" and "Oligoarticular juvenile idiopathic arthritis" and "Polyarticular juvenile idiopathic arthritis: Clinical manifestations, diagnosis, and complications" and "Spondyloarthropathy in children" and "Psoriatic juvenile idiopathic arthritis: Pathogenesis, clinical manifestations, and diagnosis" and "Psoriatic juvenile idiopathic arthritis: Management and prognosis" and "Classification of juvenile arthritis" and "Juvenile idiopathic arthritis: Epidemiology and immunopathogenesis".)

OVERVIEW

Patients with sJIA fall into the category of systemic arthritis in the 2004 International League of Associations for Rheumatology (ILAR) proposed classification of the childhood arthritides [1]. Children with this illness comprise between 10 and 20 percent of all cases of JIA. However, data suggest that this illness is a unique condition closer to the autoinflammatory family of diseases, with distinct manifestations and treatment responses that distinguish it from the other diseases categorized as JIA [2,3]. (See "Classification of juvenile arthritis" and "Periodic fever syndromes and other autoinflammatory diseases: An overview".)

This form of JIA is very distinctive but may be the most difficult to diagnose and treat for the following reasons:

Arthritis, although necessary to establish a definitive diagnosis, may not be evident early in the course of the disease [1,4].

                 

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Literature review current through: Nov 2016. | This topic last updated: Wed Apr 27 00:00:00 GMT 2016.
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