Juvenile rheumatoid arthritis

https://doi.org/10.1016/S0889-857X(02)00016-9Get rights and content

Section snippets

Classification of JRA

The classification of chronic arthritis in childhood continues to evolve. The optimal classification scheme has homogeneous disease categories with defined biological parameters, including etiological and pathogenetic factors. Homogeneity is not totally practical, however, because the phenotypic expression of a disease is influenced by numerous factors, including genetic determinants. The original descriptions of chronic arthritis in children by Meyer Diamant-Berger (1892) and George Frederic

Epidemiology of JRA

Early epidemiologic studies showed that the prevalence ranged from 0.16 to 1.10/1000, and incidence ranged from 0 to 9.2/100,000 [15]. Recent studies have also demonstrated a wide prevalence range (Table 1) [16], [17]. The data from the 1990 United States Bureau of Census cited by the National Arthritis Data Work Group estimate the number of cases of JRA in the United States to be between 30,000 and 50,000 [18]. Active cases probably constitute only one-half of this number. Manners and

Genetic update

Major histocompatibilty complex-associated risk factors within different subtypes of JRA have been demonstrated in numerous series. HLA-B27 was the first HLA association to be demonstrated in JRA. It has been consistently associated with pauciarticular disease, particularly in older men, which correlates with the enthesitis-related arthritis in the current ILAR classification scheme [1]. The Class II genes, HLA-DR1 and HLA-DR4, have been associated with polyarticular JRA [26]. HLA-DR4 (DRB1

Differential diagnosis

The diagnosis of JRA requires the meticulous exclusion of a broad range of conditions. The most important differential diagnoses for monoarticular, polyarticular and systemic onset disease are listed in the Box below.

Differential diagnosis of juvenile rheumatoid arthritis

Monoarticular onset disease

Rheumatic diseases

  • Pauciarticular JRA

  • Juvenile psoriatic arthritis

  • Inflammatory bowel disease related arthritis

  • Juvenile ankylosing spondylitis

Infection-related arthritis

  • Septic arthritis

  • Transient synovitis

  • Reactive arthritis

  • Tuberculous arthritis

  • Lyme arthritis

Trauma and overuse syndromes

Prognosis and outcome

Conflicting results of outcome studies may be attributed to differences in disease classification, patient selection methods, follow up duration, the assessment methods used and not least, the lack of a generally accepted definition of remission in JRA. Nevertheless, it is clear that active disease often persists into adult life in all subtypes of JRA. Several long term studies conducted from the 1960s to the late 1990s showed that 31% to 55% of patients with JRA have active disease when

Growth disturbances in JRA

Localized disturbances of growth in children with JRA are common and can have significant functional and cosmetic impacts. Localized overgrowth of bone may result from the accelerated development of ossification centers associated with inflammatory hyperemia, whereas premature physeal closure or destruction of growth centers can retard bone growth [81]. The most common growth abnormality in pauciarticular JRA is lower limb overgrowth associated with knee arthritis, which may result in a leg

Treatment

Despite the “dismantling” of the traditional therapeutic pyramid, a hierarchy of therapeutic strategies still exists in the pharmacologic management of patients with JRA, with shortened duration between additive treatments. Comprehensive reviews of treatment of patients with JRA have recently been reported [114], [115], [116].

Traditional NSAIDs still constitute the first line therapy for most children with JRA. The COX-2 inhibitors have not been fully studied in the pediatric population, and

Summary

Progress in achieving international consensus concerning the classification of juvenile idiopathic arthritis has been made, although further refinement and validation of these criteria is needed. It is hoped that this will facilitate more effective international collaboration in the study of these diseases, because much remains to be learned about genetic susceptibility, causation, pathogenesis, and treatment. Attention to the unique aspects of chronic arthritis in children such as impaired

First page preview

First page preview
Click to open first page preview

References (165)

  • R Schneider

    Prognostic indicators of joint destruction in systemic-onset juvenile rheumatoid arthritis

    J Pediatr

    (1992)
  • J.J Kanski

    Juvenile arthritis and uveitis

    Surv Ophthalmol

    (1990)
  • M.R Dana

    Visual outcomes prognosticators in juvenile rheumatoid arthritis-associated uveitis

    Ophthalmology

    (1997)
  • B.M Ansell et al.

    Prognosis in juvenile chronic polyarthritis

    Clin Rheum Dis

    (1976)
  • D.B Allen

    Growth suppression by glucocorticoid therapy

    Endocrinol Metab Clin North Am

    (1996)
  • J.T Cassidy

    Bone mineral metabolism in children with juvenile rheumatoid arthritis

    Pediatr Clin North Am

    (1995)
  • E.J Brewer et al.

    Criteria for the classification of juvenile rheumatoid arthritis

    Bull Rheum Dis

    (1973)
  • E.J Brewer et al.

    Current proposed revision of JRA Criteria. JRA Criteria Subcommittee of the Diagnostic and Therapeutic Criteria Committee of the American Rheumatism Section of The Arthritis Foundation

    Arthritis Rheum

    (1977)
  • P.H Wood

    Nomenclature and classification of arthritis in children

  • C.W Fink

    Proposal for the development of classification criteria for idiopathic arthritides of childhood

    J Rheumatol

    (1995)
  • R.E Petty

    Revision of the proposed classification criteria for juvenile idiopathic arthritis: Durban, 1997

    J Rheumatol

    (1998)
  • L Berntson

    Construct validity of ILAR and EULAR criteria in juvenile idiopathic arthritis: a population based incidence study from the Nordic countries. International League of Associations for Rheumatology. European League Against Rheumatism

    J Rheumatol

    (2001)
  • S.E Ramsey

    Comparison of criteria for the classification of childhood arthritis

    J Rheumatol

    (2000)
  • I Foeldvari et al.

    Validation of the proposed ILAR classification criteria for juvenile idiopathic arthritis. International League of Associations for Rheumatology

    J Rheumatol

    (2000)
  • M.F Hofer et al.

    Juvenile idiopathic arthritides evaluated prospectively in a single center according to the Durban criteria

    J Rheumatol

    (2001)
  • R Merino et al.

    Evaluation of ILAR classification criteria for juvenile idiopathic arthritis in Spanish children

    J Rheumatol

    (2001)
  • F Fantini

    Classification of chronic arthritides of childhood (juvenile idiopathic arthritis): criticisms and suggestions to improve the efficacy of the Santiago-Durban criteria

    J Rheumatol

    (2001)
  • R.E Petty

    Growing pains: the ILAR classification of juvenile idiopathic arthritis

    J Rheumatol

    (2001)
  • Thomas E., Barrett J.H., Donn R.P., Thomson W., Southwood T.R., and the British Paediatric Rheumatology Group,...
  • M.C Hochberg

    The epidemiology of juvenile rheumatoid arthritis: Review of current status and approaches for future research

  • B.A Gare

    Juvenile arthritis – who gets it, where and when? A review of current data on incidence and prevalence

    Clin Exp Rheumatol

    (1999)
  • R.C Lawrence

    Estimates of the prevalence of arthritis and selected musculoskeletal disorders in the United States

    Arthritis Rheum

    (1998)
  • P.J Manners et al.

    Prevalence of juvenile chronic arthritis in a population of 12-year-old children in urban Australia

    Pediatrics

    (1996)
  • K Oen

    Comparative epidemiology of the rheumatic diseases in children

    Curr Opin Rheumatol

    (2000)
  • M.M Schwartz

    Juvenile rheumatoid arthritis in African Americans

    J Rheumatol

    (1997)
  • M.T Pugh et al.

    The role of infection in juvenile chronic arthritis

    Br J Rheumatol

    (1993)
  • C.B Lindsley

    Seasonal variation in systemic onset juvenile rheumatoid arthritis

    Arthritis Rheum

    (1987)
  • Y Uziel

    Seasonal variation in systemic onset juvenile rheumatoid arthritis in Israel

    J Rheumatol

    (1999)
  • K Murray et al.

    Pathogenesis of juvenile chronic arthritis: genetic and environmental factors

    Arch Dis Child

    (1997)
  • M.A Fernandez-Vina et al.

    HLA antigens in juvenile arthritis. Pauciarticular and polyarticular juvenile arthritis are immunogenetically distinct

    Arthritis Rheum

    (1990)
  • D.N Glass et al.

    Juvenile rheumatoid arthritis as a complex genetic trait

    Arthritis Rheum

    (1999)
  • M.B Moroldo

    Juvenile rheumatoid arthritis in affected sibpairs

    Arthritis Rheum

    (1997)
  • S Prahalad

    Juvenile rheumatoid arthritis: linkage to HLA demonstrated by allele sharing in affected sibpairs

    Arthritis Rheum

    (2000)
  • K.J Murray

    Age-specific effects of juvenile rheumatoid arthritis-associated HLA alleles

    Arthritis Rheum

    (1999)
  • J.P Haas et al.

    Subtypes of HLA-DRB1*03, *08, *11, *12, *13, and *14 in early onset pauciarticular juvenile chronic arthritis (EOPA) with and without iridocyclitis

    Clin Exp Rheum

    (1994)
  • P Pratsidou-Gertsi

    Nationwide collaborative study of HLA class II associations with distinct types of juvenile chronic arthritis (JCA) in Greece

    Eur J Immunogenet

    (1999)
  • Y Date

    Identification of a genetic risk factor for systemic juvenile rheumatoid arthritis in the 5'-flanking region of the TNFalpha gene and HLA genes

    Arthritis Rheum

    (1999)
  • D Fishman

    The effect of novel polymorphisms in the interleukin-6 (IL-6) gene on IL-6 transcription and plasma IL-6 levels, and an association with systemic-onset juvenile chronic arthritis

    J Clin Invest

    (1998)
  • T.L McDowell

    A genetic association between juvenile rheumatoid arthritis and a novel interleukin-1 alpha polymorphism

    Arthritis Rheum

    (1995)
  • C.B Sanjeevi

    Polymorphism at NRAMP1 and D2S1471 loci associated with juvenile rheumatoid arthritis

    Arthritis Rheum

    (2000)
  • Cited by (77)

    • Skin and Rheumatic Diseases

      2016, Kelley and Firestein's Textbook of Rheumatology: Volumes 1-2, Tenth Edition
    View all citing articles on Scopus
    View full text