Juvenile Rheumatoid Arthritis

Author: C Egla Rabinovich, MD, MPH, Associate Professor and Co-Division Chief, Department of Pediatrics, Division of Pediatric Rheumatology, Duke University Medical Center
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Updated: Apr 7, 2010

Introduction

Background

Juvenile rheumatoid arthritis (JRA) is the most common rheumatological disease in children and is one of the most common chronic diseases of childhood. It represents a group of disorders that all share the clinical manifestation of chronic joint inflammation. The etiology is largely unknown, and the genetic component is complex, making clear distinctions between the various subtypes difficult. As a result, various classification criteria are recognized, with different benefits and limitations. A new nomenclature, juvenile idiopathic arthritis (JIA), is increasingly used and is replacing the term juvenile rheumatoid arthritis.

The American College of Rheumatology classifies juvenile rheumatoid arthritis into 3 distinct subtypes: pauciarticular juvenile rheumatoid arthritis, polyarticular juvenile rheumatoid arthritis, and systemic JRA. Other childhood arthritis such as juvenile ankylosing spondylitis and psoriatic arthritis are classified under spondyloarthropathies.

In 1997, the International League of Associations for Rheumatology (ILAR) conducted a consensus conference during which they proposed the nomenclature juvenile idiopathic arthritis. The classification criteria include psoriatic arthritis and enthesitis-related arthritis, which encompasses juvenile ankylosing spondylitis, arthritis associated with inflammatory bowel disease, reactive arthritis, and spondyloarthropathies. This has resulted in some confusion in the literature; when reviewing existing literature, consider whether authors are referring to the juvenile rheumatoid arthritis or juvenile idiopathic arthritis nomenclature because this affects the population being discussed and thus the generalizability of the results.

This article focuses on oligoarticular juvenile idiopathic arthritis (pauciarticular juvenile rheumatoid arthritis), polyarticular juvenile idiopathic arthritis, both rheumatoid factor positive and negative (polyarticular JRA), and systemic juvenile idiopathic arthritis (systemic JRA).