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Oligoarticular juvenile idiopathic arthritis

Thomas JA Lehman, MD
Section Editor
Marisa Klein-Gitelman, MD, MPH
Deputy Editor
Elizabeth TePas, MD, MS


Oligoarticular juvenile idiopathic arthritis (JIA) (formerly called pauciarthritis or pauciarticular onset juvenile rheumatoid arthritis) is the subset of JIA that includes patients with involvement of fewer than five joints. It is the most common subgroup, constituting about 50 percent of cases of JIA (table 1). A proposed nomenclature divides this group into persistent oligoarticular JIA (no additional joint involvement after the first six months of illness) and extended oligoarticular JIA (involvement of four or less joints during the first six months of illness; additional joints are involved over time, resulting in more than four joints ultimately being affected). (See "Classification of juvenile arthritis".)

The clinical issues related to oligoarticular JIA are discussed in this review. Systemic JIA and polyarticular JIA are discussed separately. (See "Systemic juvenile idiopathic arthritis: Clinical manifestations and diagnosis" and "Polyarticular juvenile idiopathic arthritis: Clinical manifestations and diagnosis".)


Oligoarticular juvenile idiopathic arthritis (JIA) affects females more often than males, as does polyarticular disease. The peak incidence of oligoarticular JIA is in the second and third years. It is less common over 5 years of age and rarely begins after age 10. A child with large joint involvement beginning in the early teenage years most commonly has a spondyloarthropathy. The spondyloarthropathies are called "enthesitis-related arthritis", which is a distinct subset of juvenile idiopathic arthritis (JIA) in the new nomenclature. (See "Spondyloarthropathy in children".)

The typical child with oligoarticular JIA is a girl who is noticed to be limping without complaint. Often the family notices that the child "walks funny" in the morning, but after a little while seems fine. In many cases, the child has never complained of pain; the family seeks medical advice only because the knee is swollen. It is unusual for the family to be able to specify exactly when the illness started.

Oligoarticular JIA affects the large joints (knees, ankles, wrists, elbows), but virtually never begins in the hips. There are several other types of arthritis that may have oligoarticular onset in this age group, but are not juvenile rheumatoid arthritis (JRA). However, under the new nomenclature these conditions are covered under the classification of oligoarticular JIA. Systemic manifestations (other than uveitis) are characteristically absent. Thus, fever, rash, or other constitutional symptoms suggest a different diagnosis. (See 'Differential diagnosis' below.)


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Literature review current through: Jun 2015. | This topic last updated: Jul 15, 2013.
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  1. Criteria for the classification of juvenile rheumatoid arthritis. Bull Rheum Dis 1972; 23:712.
  2. Trapani S, Grisolia F, Simonini G, et al. Incidence of occult cancer in children presenting with musculoskeletal symptoms: a 10-year survey in a pediatric rheumatology unit. Semin Arthritis Rheum 2000; 29:348.
  3. Minden K, Niewerth M, Listing J, et al. Long-term outcome in patients with juvenile idiopathic arthritis. Arthritis Rheum 2002; 46:2392.
  4. Zak M, Pedersen FK. Juvenile chronic arthritis into adulthood: a long-term follow-up study. Rheumatology (Oxford) 2000; 39:198.
  5. Al-Matar MJ, Petty RE, Tucker LB, et al. The early pattern of joint involvement predicts disease progression in children with oligoarticular (pauciarticular) juvenile rheumatoid arthritis. Arthritis Rheum 2002; 46:2708.
  6. Saurenmann RK, Levin AV, Feldman BM, et al. Prevalence, risk factors, and outcome of uveitis in juvenile idiopathic arthritis: a long-term followup study. Arthritis Rheum 2007; 56:647.
  7. Heiligenhaus A, Niewerth M, Ganser G, et al. Prevalence and complications of uveitis in juvenile idiopathic arthritis in a population-based nation-wide study in Germany: suggested modification of the current screening guidelines. Rheumatology (Oxford) 2007; 46:1015.
  8. Bolt IB, Cannizzaro E, Seger R, Saurenmann RK. Risk factors and longterm outcome of juvenile idiopathic arthritis-associated uveitis in Switzerland. J Rheumatol 2008; 35:703.
  9. Cassidy JT, Petty RE. Juvenile rheumatoid arthritis. In: Textbook of pediatric rheumatology, Cassidy JT, Petty RE (Eds), WB Saunders Company, Philadelphia 2001. p.218.
  10. Cassidy J, Kivlin J, Lindsley C, et al. Ophthalmologic examinations in children with juvenile rheumatoid arthritis. Pediatrics 2006; 117:1843.
  11. Kilmartin DJ, Forrester JV, Dick AD. Cyclosporin A therapy in refractory non-infectious childhood uveitis. Br J Ophthalmol 1998; 82:737.
  12. Vazquez-Cobian LB, Flynn T, Lehman TJ. Adalimumab therapy for childhood uveitis. J Pediatr 2006; 149:572.
  13. Richards JC, Tay-Kearney ML, Murray K, Manners P. Infliximab for juvenile idiopathic arthritis-associated uveitis. Clin Experiment Ophthalmol 2005; 33:461.
  14. Tynjälä P, Lindahl P, Honkanen V, et al. Infliximab and etanercept in the treatment of chronic uveitis associated with refractory juvenile idiopathic arthritis. Ann Rheum Dis 2007; 66:548.
  15. Heiligenhaus A, Miserocchi E, Heinz C, et al. Treatment of severe uveitis associated with juvenile idiopathic arthritis with anti-CD20 monoclonal antibody (rituximab). Rheumatology (Oxford) 2011; 50:1390.
  16. Kalinina Ayuso V, Ten Cate HA, van der Does P, et al. Male gender and poor visual outcome in uveitis associated with juvenile idiopathic arthritis. Am J Ophthalmol 2010; 149:987.
  17. Kalinina Ayuso V, Ten Cate HA, van der Does P, et al. Male gender as a risk factor for complications in uveitis associated with juvenile idiopathic arthritis. Am J Ophthalmol 2010; 149:994.
  18. Sherry DD, Stein LD, Reed AM, et al. Prevention of leg length discrepancy in young children with pauciarticular juvenile rheumatoid arthritis by treatment with intraarticular steroids. Arthritis Rheum 1999; 42:2330.
  19. Padeh S, Pinhas-Hamiel O, Zimmermann-Sloutskis D, Berkun Y. Children with oligoarticular juvenile idiopathic arthritis are at considerable risk for growth retardation. J Pediatr 2011; 159:832.
  20. Simon D, Fernando C, Czernichow P, Prieur AM. Linear growth and final height in patients with systemic juvenile idiopathic arthritis treated with longterm glucocorticoids. J Rheumatol 2002; 29:1296.
  21. Aggarwal B, Bhalla AK, Singh S. Longitudinal growth attainments of Indian boys with juvenile rheumatoid arthritis. Rheumatol Int 2011; 31:635.
  22. Minden K. Adult outcomes of patients with juvenile idiopathic arthritis. Horm Res 2009; 72 Suppl 1:20.
  23. Beukelman T, Patkar NM, Saag KG, et al. 2011 American College of Rheumatology recommendations for the treatment of juvenile idiopathic arthritis: initiation and safety monitoring of therapeutic agents for the treatment of arthritis and systemic features. Arthritis Care Res (Hoboken) 2011; 63:465.
  24. Padeh S, Passwell JH. Intraarticular corticosteroid injection in the management of children with chronic arthritis. Arthritis Rheum 1998; 41:1210.
  25. Lanni S, Bertamino M, Consolaro A, et al. Outcome and predicting factors of single and multiple intra-articular corticosteroid injections in children with juvenile idiopathic arthritis. Rheumatology (Oxford) 2011; 50:1627.
  26. Huppertz HI, Tschammler A, Horwitz AE, Schwab KO. Intraarticular corticosteroids for chronic arthritis in children: efficacy and effects on cartilage and growth. J Pediatr 1995; 127:317.
  27. Woo P, Southwood TR, Prieur AM, et al. Randomized, placebo-controlled, crossover trial of low-dose oral methotrexate in children with extended oligoarticular or systemic arthritis. Arthritis Rheum 2000; 43:1849.
  28. Horneff G, Schmeling H, Biedermann T, et al. The German etanercept registry for treatment of juvenile idiopathic arthritis. Ann Rheum Dis 2004; 63:1638.
  29. Ogra PL, Chiba Y, Ogra SS, et al. Rubella-virus infection in juvenile rheumatoid arthritis. Lancet 1975; 1:1157.
  30. Chantler JK, Tingle AJ, Petty RE. Persistent rubella virus infection associated with chronic arthritis in children. N Engl J Med 1985; 313:1117.
  31. Linnemann CC Jr, Levinson JE, Buncher CR, Schiff GM. Rubella antibody levels in juvenile rheumatoid arthritis. Ann Rheum Dis 1975; 34:354.
  32. Al-Nakib W, Majeed HA. Serologic studies on the association of rubella virus infection and juvenile rheumatoid arthritis. Ann Trop Paediatr 1981; 1:93.
  33. Morin MP, Quach C, Fortin E, Chédeville G. Vaccination coverage in children with juvenile idiopathic arthritis followed at a paediatric tertiary care centre. Rheumatology (Oxford) 2012; 51:2046.
  34. Heijstek MW, Pileggi GC, Zonneveld-Huijssoon E, et al. Safety of measles, mumps and rubella vaccination in juvenile idiopathic arthritis. Ann Rheum Dis 2007; 66:1384.
  35. Borte S, Liebert UG, Borte M, Sack U. Efficacy of measles, mumps and rubella revaccination in children with juvenile idiopathic arthritis treated with methotrexate and etanercept. Rheumatology (Oxford) 2009; 48:144.
  36. Heijstek MW, Kamphuis S, Armbrust W, et al. Effects of the live attenuated measles-mumps-rubella booster vaccination on disease activity in patients with juvenile idiopathic arthritis: a randomized trial. JAMA 2013; 309:2449.
  37. Heijstek MW, Scherpenisse M, Groot N, et al. Immunogenicity and safety of the bivalent HPV vaccine in female patients with juvenile idiopathic arthritis: a prospective controlled observational cohort study. Ann Rheum Dis 2014; 73:1500.