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Paeds Casesrheumatology-musculoskeletal-and-sports

Paeds Cases · rheumatology-musculoskeletal-and-sports

Juvenile idiopathic arthritis: Case

Clinical long case of a three-year-old girl presenting with a swollen right knee for ten weeks, stiff in the morning and improving with activity, covering the ILAR oligoarticular classification, the persistent-versus-extended split, the chronic anterior uveitis and the three-monthly slit-lamp screening, the methotrexate at ten to fifteen milligrams per square metre per week, the etanercept and adalimumab biologics, the Wallace clinically inactive disease criteria, and the septic-arthritis mimic.

paediatric rheumatology long case4 min readVerification in progress

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RACP DCEMRCPCH ClinicalRCPSC Pediatrics
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RACP DCEMRCPCH ClinicalRCPSC Pediatrics
Prompt
A three-year-old girl is brought to the paediatric clinic by her parents, who have noticed a swollen right knee for ten weeks. She limps in the morning and after rest, and the limp improves markedly over the first hour and through the day at preschool. She is afebrile and well, with no rash, and she has grown and developed normally. On examination the right knee is swollen, warm, slightly tender and restricted in flexion and extension, with a small effusion and mild quadriceps wasting; all other joints are normal. The antinuclear antibody is positive at one in six hundred and forty, the rheumatoid factor is negative, the inflammatory markers are mildly raised, and the slit-lamp shows the cells and the flare of the chronic anterior uveitis. The examiner asks how you frame the problem, how you classify and risk-stratify the disease, how you manage the uveitis and the joint, and how your approach changes if she becomes febrile and unwell.

Framing the case

This three-year-old girl has the classic presentation of the oligoarticular juvenile idiopathic arthritis with the chronic anterior uveitis. The swollen knee for ten weeks, the morning stiffness that improves with activity, the single joint, the antinuclear-antibody positivity and the cells and the flare on the slit-lamp together make the diagnosis, and the framework that organises the case is the ILAR classification, the persistent-versus-extended split, the uveitis risk profile and the stepwise treat-to-target care. The first priority is the management of the uveitis and the joint, and the exclusion of the septic-arthritis mimic. [1][2]

Classifying and risk-stratifying the disease

The ILAR classification requires the arthritis of one or more joints for at least six weeks in a child under sixteen with the other causes excluded, and this child meets the criteria with the ten-week duration. She has one joint in the first six months, which assigns her to the oligoarticular category, the commonest subtype at about half to two-thirds of the cases. The oligoarticular disease splits at the six-month mark into the persistent, where the disease stays in four joints or fewer, and the extended, where further joints become involved after the first six months, and the split will be determined at the six-month review. The uveitis risk is the highest in the oligoarticular subtype, and she meets all four of the high-risk criteria: she is young, antinuclear-antibody positive, oligoarticular and within the first four years of the disease. [1][9]

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References9ShowHide
  1. [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.PMID 14760812
  2. [2]Ravelli A, Martini A Juvenile idiopathic arthritis Lancet, 2007.PMID 17336654
  3. [4]Giannini EH, Brewer EJ, Kuzmina N, et al Methotrexate in resistant juvenile rheumatoid arthritis. Results of the U.S.A.-U.S.S.R. double-blind, placebo-controlled trial N Engl J Med, 1992.PMID 1549149
  4. [5]Lovell DJ, Giannini EH, Reiff A, et al Etanercept in children with polyarticular juvenile rheumatoid arthritis N Engl J Med, 2000.PMID 10717011
  5. [6]Wallace CA, Giannini EH, Spalding SJ, et al Trial of early aggressive therapy in polyarticular juvenile idiopathic arthritis Arthritis Rheum, 2012.PMID 22183975
  6. [7]Wallace CA, Ravelli A, Huang B, et al. Preliminary criteria for clinical remission for select categories of juvenile idiopathic arthritis using the OMERACT filter J Rheumatol, 2006.PMID 16482643
  7. [8]Constantin T, Foeldvari I, Anton J, et al Consensus-based recommendations for the management of uveitis associated with juvenile idiopathic arthritis: the SHARE initiative Ann Rheum Dis, 2018.PMID 29592918
  8. [9]Nordal E, Rypdal V, Christoffersen T, et al. Incidence and predictors of uveitis in juvenile idiopathic arthritis in a Nordic long-term cohort study Pediatr Rheumatol Online J, 2017.PMID 28821293
  9. [10]Rypdal V, Glerup M, Songstad NT, et al. Uveitis in juvenile idiopathic arthritis: 18-year outcome in the population-based Nordic cohort study Ophthalmology, 2021.PMID 32866542
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