Paeds · rheumatology-musculoskeletal-and-sports
Juvenile idiopathic arthritis
Also known as Juvenile rheumatoid arthritis · JIA · Juvenile chronic arthritis · Oligoarticular JIA · Polyarticular JIA · Enthesitis-related arthritis · Chronic anterior uveitis of childhood
Fellowship guide to juvenile idiopathic arthritis, the commonest rheumatic disease of childhood. Covers the ILAR classification with its seven categories, the dominance of the oligoarticular subtype, the chronic anterior uveitis that is silent and blinding and that drives the slit-lamp screening schedule, the methotrexate at ten to fifteen milligrams per square metre per week that is the anchor disease-modifying drug, the etanercept and adalimumab biologics that transformed refractory disease, the Wallace clinically inactive disease criteria and the treat-to-target TREAT trial, and the long-term remission and uveitis outcomes that frame the prognosis.
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- A child under sixteen with a joint swollen or stiff for more than six weeks, worst in the morning and improving with activity, has juvenile idiopathic arthritis until the slit lamp and the workup prove otherwise
- The young child with oligoarticular JIA who is antinuclear-antibody positive and under seven at onset is the highest-risk candidate for chronic anterior uveitis, which is silent until it blinds, and the slit-lamp screening every three months is non-negotiable
- A child on methotrexate with fever and neutropenia, or with vomiting and lethargy and a rising transaminase, may have the marrow suppression or the hepatotoxicity that demands urgent review, and the live vaccines are contraindicated
- A child on a biologic with fever must be assumed to have the serious infection that the tumour necrosis factor blockade masks, and the empiric antibiotics come before the cultures return
- A swollen joint in a febrile unwell child is septic arthritis until proven otherwise, and the Kocher criteria, the blood cultures and the joint aspirate separate the infection from the JIA
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Board mappings
- Defines juvenile idiopathic arthritis as the arthritis of one or more joints for at least six weeks in a child under sixteen years with other causes excluded, and recognises the oligoarticular subtype as the commonest form
- Identifies the morning stiffness and the gelling that improve with activity as the cardinal symptoms, and the swollen warm joint with restricted movement as the cardinal sign
- Names chronic anterior uveitis as the sight-threatening complication that is silent, commonest in the young antinuclear-antibody-positive child with oligoarticular disease, and detected only by the slit-lamp screening
- Applies the International League of Associations for Rheumatology classification with its seven categories of systemic, oligoarticular, polyarticular rheumatoid-factor negative, polyarticular rheumatoid-factor positive, psoriatic, enthesitis-related and undifferentiated arthritis
- Distinguishes persistent from extended oligoarticular arthritis at the six-month mark by whether further joints have become involved, and explains why this split changes the prognosis and the escalation
- Coordinates the stepwise pharmacotherapy from the non-steroidal anti-inflammatory drugs through the methotrexate at ten to fifteen milligrams per square metre per week to the etanercept and adalimumab biologics
- A young child with oligoarticular juvenile idiopathic arthritis who is antinuclear-antibody positive and under seven at onset needs slit-lamp screening every three months for the chronic anterior uveitis, because the uveitis is silent until it blinds
- Methotrexate at ten to fifteen milligrams per square metre once weekly is the anchor disease-modifying drug, given with folic acid, with the live vaccines contraindicated and the marrow and the liver monitored
- A child on a tumour-necrosis-factor inhibitor with fever has a serious infection until proven otherwise, because the blockade masks the signs and the empiric antibiotics come before the cultures return
- Examines the child with a suspected swollen joint systematically, distinguishing the JIA from the septic arthritis by the Kocher criteria and the morning stiffness, and examining all the joints and the eyes
- Counsels the family on the chronic relapsing course, the methotrexate and the biologics, the uveitis screening, the school and the physical activity, and the transition to the adult rheumatology
- Synthesises the treat-to-target plan that aims for the clinically inactive disease and the long-term remission while minimising the uveitis, the leg-length inequality and the growth and psychosocial burden
- Level 1: Recognition of the swollen joint for more than six weeks in the child under sixteen, the morning stiffness, and the need for the slit-lamp screening
- Level 2: Application of the ILAR classification, the methotrexate, the biologics and the uveitis screening schedule to the management
- Level 3: Coordination of the multidisciplinary treat-to-target care, the transition, and the long-term uveitis and remission surveillance
- The seven ILAR categories and the oligoarticular dominance, with the persistent-versus-extended split at six months
- The chronic anterior uveitis, its silent course, the antinuclear-antibody and the young-age risk factors, and the three-monthly slit-lamp screening
- The methotrexate, the etanercept and the adalimumab, and the Wallace clinically inactive disease criteria and the treat-to-target TREAT trial
- Structured musculoskeletal examination of the child using the look-feel-move-measure approach across all the joints
- Recognition of the pGALS screening and the chronic anterior uveitis signs of the band keratopathy, the synechiae and the cataract
- Communication of the chronic diagnosis, the uveitis risk and the treatment plan to the child and the family
- Juvenile idiopathic arthritis as the commonest rheumatic disease of childhood, presenting with the morning stiffness and the swollen joint, and dominated by the oligoarticular subtype
- The diagnostic pathway of the exclusion of other causes, the antinuclear antibody and the rheumatoid factor, and the urgent slit-lamp for the uveitis
- The stepwise treatment from the non-steroidal anti-inflammatory drugs through the methotrexate to the biologics, and the treat-to-target goal of the inactive disease
- Recognition of the swollen joint for more than six weeks in the child under sixteen as juvenile idiopathic arthritis requiring the paediatric rheumatology referral
- Application of the initial evaluation with the exclusion of infection and malignancy, the antinuclear antibody, and the slit-lamp
- Coordination of the multidisciplinary treatment and the uveitis surveillance across the chronic course
- Canadian approach to the child with the limp or the swollen joint, the exclusion of the septic arthritis and the osteomyelitis, and the recognition of the JIA
- The ILAR classification and the methotrexate and the biologics with the uveitis screening as the framework for the management
- The treat-to-target care, the transition to the adult rheumatology, and the long-term uveitis and remission surveillance
Overview & Definition
A four-year-old girl limps into the clinic with a knee that has been swollen for two months, stiffer in the morning and looser by recess. A six-year-old boy is found at the slit lamp to have quiet inflammation scarring his eye, three months into an oligoarticular course that never troubled him. Both have juvenile idiopathic arthritis, and both are the everyday face of the commonest rheumatic disease of childhood. Juvenile idiopathic arthritis is the arthritis of one or more joints that lasts for at least six weeks in a child under sixteen years of age, after every other cause is excluded. It is not one disease but a family of seven, unified by the chronic inflammatory arthritis and divided by the pattern, the serology and the extra-articular features, and the candidate who holds the unity and the diversity together holds the topic. [1][2]
The disease earns its gravity from two facts that the boards reward the candidate who carries. The first is the silent eye. The chronic anterior uveitis of JIA produces no pain, no redness and no visual complaint until the band keratopathy, the synechiae and the cataract have scarred the sight, and the only defence is the slit-lamp screening of the child at risk. The second is the long course. JIA is a chronic disease that stretches across the childhood and into the adult life, and the treatment has moved from the symptom control to the treat-to-target goal of the clinically inactive disease, driven by the methotrexate and transformed by the etanercept and adalimumab biologics. The candidate who can frame the disease as the treatable chronic illness, rather than the catalogue of subtypes, demonstrates reasoning boards reward. [2][3]
The clinical weight of the topic is concentrated in the classification, the uveitis and the therapeutics, because these are the three corners the examiner probes. The International League of Associations for Rheumatology classification sorts the children into the seven categories that guide the prognosis and the uveitis risk. The chronic anterior uveitis, commonest in the young antinuclear-antibody-positive child with oligoarticular disease, is the complication that changes the outcome and that drives the three-monthly slit-lamp. The methotrexate and the biologics are the drugs that turned JIA from a disease of deformity into a disease of remission, and the Wallace criteria and the TREAT trial are the evidence that anchors the treat-to-target care. [1][6]
References12ShowHide
- [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]Ravelli A, Martini A Juvenile idiopathic arthritis Lancet, 2007.PMID 17336654
- [3]Giancane G, Alongi A, Ravelli A Update on the pathogenesis and treatment of juvenile idiopathic arthritis Curr Opin Rheumatol, 2017.PMID 28538013
- [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
- [5]Lovell DJ, Giannini EH, Reiff A, et al Etanercept in children with polyarticular juvenile rheumatoid arthritis N Engl J Med, 2000.PMID 10717011
- [6]Wallace CA, Giannini EH, Spalding SJ, et al Trial of early aggressive therapy in polyarticular juvenile idiopathic arthritis Arthritis Rheum, 2012.PMID 22183975
- [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
- [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
- [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
- [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
- [11]Horneff G, Minden K, Rolland C, et al. Efficacy and safety of TNF inhibitors in the treatment of juvenile idiopathic arthritis: a systematic literature review Pediatr Rheumatol Online J, 2023.PMID 36829225
- [12]Shoop-Worrall SJW, Kearsley-Fleet L, Thomson W, et al. How common is remission in juvenile idiopathic arthritis: a systematic review Semin Arthritis Rheum, 2017.PMID 28625712