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Volume 51, Number 5

Classification of Juvenile Chronic Idiopathic Arthropathies:
Is It Time to Change Yet?

Hulya Bukulmez, MD
Murray H. Passo, MD
William S. Rowe Division of Rheumatology
Cincinnati Children’s Hospital Medical Center
Cincinnati, OH

 Summary Points

• The classification of the juvenile arthropathies is mostly based on the different presentations of arthritis in children.

• The classification includes diseases that can be associated with juvenile-onset arthritis, such as psoriasis.

• The two major clinical tools used today in the recognition and classification of juvenile arthritis  are the physical examination and laboratory testing, but in the future, pathophysiological mechanisms may play a role.

Introduction

Juvenile idiopathic arthritis is the most common rheumatic disease in childhood. This statement is somewhat provocative since it actually represents a heterogeneous group of different chronic arthropathies all starting by definition before 16 years of age. Estimates of prevalence vary from 30 to 150 per 100, 000 per year in Europe and the United States (1). These juvenile arthritides are characterized by idiopathic peripheral arthritis with an immunoinflammatory pathogenesis, possibly activated by contact with external antigens (2). 

There has been an ongoing dialogue among pediatric rheumatologists from different parts of the world about the classification of the juvenile arthritides for several decades (3,4,5,6). Childhood arthritis overlaps with many other conditions, and the clinical features lack definitive laboratory tests findings. The known genetic and serologic markers are not yet universally applicable or biologically meaningful. Despite these difficulties, an effort has been spent to include clinical and laboratory findings, autoantibodies, systemic organ involvement, and genetic risk factors (Table 1).

The international community of pediatric rheumatologists continues to be divided by the classification criteria controversy. Readers of the rheumatology literature need to be cognizant of the origin of the study and the classification system employed. There will likely be more refinement of the classification system. A final system is contingent on homogeneity of subgroups defined by immunogenetics and pathogenesis of the diseases.