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Oligoarticular juvenile idiopathic arthritis

Pamela F Weiss, MD, MSCE
Section Editor
Marisa Klein-Gitelman, MD, MPH
Deputy Editor
Elizabeth TePas, MD, MS


Oligoarticular juvenile idiopathic arthritis (formerly called pauciarthritis or pauciarticular-onset juvenile rheumatoid arthritis) is defined as juvenile idiopathic arthritis (JIA) involving fewer than five joints. It is the most common subgroup, constituting approximately 50 percent of cases of JIA (table 1). This subgroup of JIA is further divided into persistent oligoarthritis, in which there is no additional joint involvement after the first six months of illness, and extended oligoarthritis, in which there is involvement of additional joints after the first six months such that more than four joints are ultimately affected [1]. Approximately 50 percent of children with oligoarticular disease go on to have extended oligoarticular disease [2]. (See "Classification of juvenile arthritis".)

The clinical issues related to oligoarticular JIA are discussed in this topic review. Systemic JIA, polyarticular JIA, and psoriatic JIA are discussed separately. The epidemiology, classification, and immunopathogenesis of JIA are also discussed separately. (See "Systemic juvenile idiopathic arthritis: Clinical manifestations and diagnosis" and "Polyarticular juvenile idiopathic arthritis: Clinical manifestations, diagnosis, and complications" and "Psoriatic juvenile idiopathic arthritis: Pathogenesis, clinical manifestations, and diagnosis" and "Classification of juvenile arthritis" and "Juvenile idiopathic arthritis: Epidemiology and immunopathogenesis".)


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 of life. It is less common over five years of age and rarely begins after age 10 years.

The typical child with oligoarticular JIA is a toddler 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 (typically knees and ankles, sometimes also wrists and elbows, but rarely the hips). 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: Oct 2015. | This topic last updated: Oct 19, 2015.
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  1. Petty RE, Southwood TR, Manners P, et al. International League of Associations for Rheumatology classification of juvenile idiopathic arthritis: second revision, Edmonton, 2001. J Rheumatol 2004; 31:390.
  2. Macaubas C, Nguyen K, Milojevic D, et al. Oligoarticular and polyarticular JIA: epidemiology and pathogenesis. Nat Rev Rheumatol 2009; 5:616.
  3. 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.
  4. Baroni E, Russo BD, Masquijo JJ, et al. Pigmented villonodular synovitis of the knee in skeletally immature patients. J Child Orthop 2010; 4:123.
  5. 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.
  6. Guzman J, Oen K, Tucker LB, et al. The outcomes of juvenile idiopathic arthritis in children managed with contemporary treatments: results from the ReACCh-Out cohort. Ann Rheum Dis 2015; 74:1854.
  7. Nordal E, Zak M, Aalto K, et al. Ongoing disease activity and changing categories in a long-term nordic cohort study of juvenile idiopathic arthritis. Arthritis Rheum 2011; 63:2809.
  8. Oen K, Tucker L, Huber AM, et al. Predictors of early inactive disease in a juvenile idiopathic arthritis cohort: results of a Canadian multicenter, prospective inception cohort study. Arthritis Rheum 2009; 61:1077.
  9. Oen K, Duffy CM, Tse SM, et al. Early outcomes and improvement of patients with juvenile idiopathic arthritis enrolled in a Canadian multicenter inception cohort. Arthritis Care Res (Hoboken) 2010; 62:527.
  10. 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.
  11. Ruperto N, Nikishina I, Pachanov ED, et al. A randomized, double-blind clinical trial of two doses of meloxicam compared with naproxen in children with juvenile idiopathic arthritis: short- and long-term efficacy and safety results. Arthritis Rheum 2005; 52:563.
  12. Padeh S, Passwell JH. Intraarticular corticosteroid injection in the management of children with chronic arthritis. Arthritis Rheum 1998; 41:1210.
  13. 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.
  14. Silverman E, Mouy R, Spiegel L, et al. Leflunomide or methotrexate for juvenile rheumatoid arthritis. N Engl J Med 2005; 352:1655.
  15. van Rossum MA, van Soesbergen RM, Boers M, et al. Long-term outcome of juvenile idiopathic arthritis following a placebo-controlled trial: sustained benefits of early sulfasalazine treatment. Ann Rheum Dis 2007; 66:1518.
  16. 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.
  17. Horneff G, Schmeling H, Biedermann T, et al. The German etanercept registry for treatment of juvenile idiopathic arthritis. Ann Rheum Dis 2004; 63:1638.
  18. Simonini G, Druce K, Cimaz R, et al. Current evidence of anti-tumor necrosis factor α treatment efficacy in childhood chronic uveitis: a systematic review and meta-analysis approach of individual drugs. Arthritis Care Res (Hoboken) 2014; 66:1073.
  19. Lerman MA, Burnham JM, Chang PY, et al. Response of pediatric uveitis to tumor necrosis factor-α inhibitors. J Rheumatol 2013; 40:1394.
  20. Simonini G, Taddio A, Cattalini M, et al. Superior efficacy of Adalimumab in treating childhood refractory chronic uveitis when used as first biologic modifier drug: Adalimumab as starting anti-TNF-α therapy in childhood chronic uveitis. Pediatr Rheumatol Online J 2013; 11:16.
  21. Zannin ME, Birolo C, Gerloni VM, et al. Safety and efficacy of infliximab and adalimumab for refractory uveitis in juvenile idiopathic arthritis: 1-year followup data from the Italian Registry. J Rheumatol 2013; 40:74.
  22. Ogra PL, Chiba Y, Ogra SS, et al. Rubella-virus infection in juvenile rheumatoid arthritis. Lancet 1975; 1:1157.
  23. Chantler JK, Tingle AJ, Petty RE. Persistent rubella virus infection associated with chronic arthritis in children. N Engl J Med 1985; 313:1117.
  24. Linnemann CC Jr, Levinson JE, Buncher CR, Schiff GM. Rubella antibody levels in juvenile rheumatoid arthritis. Ann Rheum Dis 1975; 34:354.
  25. Al-Nakib W, Majeed HA. Serologic studies on the association of rubella virus infection and juvenile rheumatoid arthritis. Ann Trop Paediatr 1981; 1:93.
  26. 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.
  27. 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.
  28. 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.
  29. 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.
  30. 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.
  31. Weiss PF, Arabshahi B, Johnson A, et al. High prevalence of temporomandibular joint arthritis at disease onset in children with juvenile idiopathic arthritis, as detected by magnetic resonance imaging but not by ultrasound. Arthritis Rheum 2008; 58:1189.
  32. Billiau AD, Hu Y, Verdonck A, et al. Temporomandibular joint arthritis in juvenile idiopathic arthritis: prevalence, clinical and radiological signs, and relation to dentofacial morphology. J Rheumatol 2007; 34:1925.
  33. Twilt M, Schulten AJ, Verschure F, et al. Long-term followup of temporomandibular joint involvement in juvenile idiopathic arthritis. Arthritis Rheum 2008; 59:546.
  34. Twilt M, Mobers SM, Arends LR, et al. Temporomandibular involvement in juvenile idiopathic arthritis. J Rheumatol 2004; 31:1418.
  35. 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.
  36. 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.
  37. 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.
  38. Cassidy J, Kivlin J, Lindsley C, et al. Ophthalmologic examinations in children with juvenile rheumatoid arthritis. Pediatrics 2006; 117:1843.
  39. Bou R, Adán A, Borrás F, et al. Clinical management algorithm of uveitis associated with juvenile idiopathic arthritis: interdisciplinary panel consensus. Rheumatol Int 2015; 35:777.
  40. Vazquez-Cobian LB, Flynn T, Lehman TJ. Adalimumab therapy for childhood uveitis. J Pediatr 2006; 149:572.
  41. Tynjälä P, Kotaniemi K, Lindahl P, et al. Adalimumab in juvenile idiopathic arthritis-associated chronic anterior uveitis. Rheumatology (Oxford) 2008; 47:339.
  42. 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.
  43. Simonini G, Zannin ME, Caputo R, et al. Loss of efficacy during long-term infliximab therapy for sight-threatening childhood uveitis. Rheumatology (Oxford) 2008; 47:1510.
  44. Foeldvari I, Wierk A. Methotrexate is an effective treatment for chronic uveitis associated with juvenile idiopathic arthritis. J Rheumatol 2005; 32:362.
  45. Kilmartin DJ, Forrester JV, Dick AD. Cyclosporin A therapy in refractory non-infectious childhood uveitis. Br J Ophthalmol 1998; 82:737.
  46. 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.
  47. Zulian F, Balzarin M, Falcini F, et al. Abatacept for severe anti-tumor necrosis factor alpha refractory juvenile idiopathic arthritis-related uveitis. Arthritis Care Res (Hoboken) 2010; 62:821.
  48. Elhai M, Deslandre CJ, Kahan A. Abatacept for refractory juvenile idiopathic arthritis-associated uveitis: two new cases. Comment on the article by Zulian et al. Arthritis Care Res (Hoboken) 2011; 63:307.
  49. Foeldvari I, Nielsen S, Kümmerle-Deschner J, et al. Tumor necrosis factor-alpha blocker in treatment of juvenile idiopathic arthritis-associated uveitis refractory to second-line agents: results of a multinational survey. J Rheumatol 2007; 34:1146.
  50. 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.
  51. 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.
  52. 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.
  53. Gross RH. Leg length discrepancy: how much is too much? Orthopedics 1978; 1:307.
  54. RUSH WA, STEINER HA. A study of lower extremity length inequality. Am J Roentgenol Radium Ther 1946; 56:616.
  55. GREEN WT, ANDERSON M. The problem of unequal leg length. Pediatr Clin North Am 1955; :1137.
  56. Song KM, Halliday SE, Little DG. The effect of limb-length discrepancy on gait. J Bone Joint Surg Am 1997; 79:1690.
  57. Kaufman KR, Miller LS, Sutherland DH. Gait asymmetry in patients with limb-length inequality. J Pediatr Orthop 1996; 16:144.
  58. Friend L, Widmann RF. Advances in management of limb length discrepancy and lower limb deformity. Curr Opin Pediatr 2008; 20:46.
  59. 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.
  60. 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.
  61. Aggarwal B, Bhalla AK, Singh S. Longitudinal growth attainments of Indian boys with juvenile rheumatoid arthritis. Rheumatol Int 2011; 31:635.
  62. Minden K. Adult outcomes of patients with juvenile idiopathic arthritis. Horm Res 2009; 72 Suppl 1:20.
  63. Minden K, Niewerth M, Listing J, et al. Long-term outcome in patients with juvenile idiopathic arthritis. Arthritis Rheum 2002; 46:2392.