management

Last edited 04/2021 and last reviewed 10/2021

At present there is no cure for JIA and main aim of management is clinical remission. 

  • treatment of JIA should control the inflammatory process by decreasing the number of actively affected joints and to improve the quality of life
  • patients are said to be in remission when they have had
    • no active arthritis, fever, rash, serositis, or generalised lymphadenopathy attributable to juvenile idiopathic arthritis
    • no active uveitis
    • a normal erythrocyte sedimentation rate or C reactive protein
    • no disease activity as assessed by a doctor for the past six month (1)

Immediately refer all patients with suspected or confirmed JIA to a paediatric rheumatologist and prescribe a NSAID to relive symptoms (1)

Management of JIA should be carried out by a multidisciplinary team (MDT) which should include specialist doctors, nurses, physiotherapists, podiatrists and psychologists (2).

Two major trends have emerged recently in management of inflammatory condition including JIA.

  • the first concept is “window of opportunity”
    • idea is to ‘switch off’ the immune process by early and aggressive treatment of the condition which results in better long term outcomes
    • evidence to support this concept have been reported  e.g. - children who achieve a state of inactive disease with early and aggressive treatment within the first five years following diagnosis experience less disease-related damage.
  • the second concept is “treat-to-target”
    • main aim is to achieve tighter disease control by formally measuring and tracking patient outcomes, with low tolerance for persistent disease activity, so that incremental gains continue to be made and patient care optimised

Specific therapeutic agents used in the treatment of JIA include:

  • NSAIDs
    • used widely as first-line therapy in the primary care setting
    • provides reasonable symptom relief and will not mask other diagnoses such as infection or malignancy
    • naproxen (7.5mg/kg orally twice daily) is often given initially
    • newer cyclo-oxygenase-2 inhibitors (rofecoxib, celecoxib, and meloxicam) have not shown to be more effective or safer than naproxen
  • intra articular steroid injections
    • is used increasingly and earlier in the disease course
      • in a retrospective study of 121 injected joints
        • a response was seen within one week in all joints while 52% were still in remission after one year
    • carried out under general anaesthetic for younger children, or sedation (such as nitrous oxide) for older children
    • triamcinolone hexacetonide is the preferred agent due to its longer action than alternative preparations
    • may be used in
      • treatment of oligoarticular disease - as monotherapy
      • children whose disease has a polyarticular course - to ‘mop up’ residual or flaring joints in children who have a good response to systemic therapies
    • adverse effects include
      • cutaneous atrophy at the injection site
      • septic arthritis - risk is minimal if performed under sterile conditions
      • Cushing’s syndrome and transient increases in blood glucose in patients with diabetes - have  been reported due to systemic absorption of triamcinolone
  • disease-modifying antirheumatic drug (DMARD)
    • used in patients with a definite diagnosis of polyarticular disease or oligoarticular disease refractory to intra-articular steroids
    • DMARDs used in the treatment of JIA include
      • methotrexate
        • used for decades and remains a safe and effective mainstay of treatment (if dose is controlled appropriately)
        • 10-15 mg/m2once weekly orally or subcutaneously (maximum 25 mg/m2)
        • usually co-administered with folic acid since the drug disrupt folate metabolism
          • folate can be given daily, or weekly, however not given on the same day as methotrexate due to a theoretical risk of reducing methotrexate efficacy
        • contraindicated in - liver and kidney dysfunction, immunodeficiency, bone marrow dysfunction, active infection, breast feeding, pregnancy
        • side effects - gastrointestinal affects (nausea, vomiting, anorexia), less commonly transient rises in liver transaminases and haematological disturbances
      • sulfasalazine - especially for enthesitis-related arthritis, the juvenile spondyloarthropathy)
      • leflunomide
      • ciclosporin
      • hydroxychloroquine
    • all DMARDs require routine blood tests, monitoring for cytopenias, hepatic and renal dysfunction
  • biologic agents (also known as biologicals or biologics)
    • represents a new class of drugs which targets the inflammatory cytokines resulting in “switching off”  of the autoimmune system
    • drugs currently available or under investigation for JIA include:
      • TNF-α blocking agents - Etanercept, Adalimumab, Infliximab 
      • T cell co-stimulation modulator - Abatacept 
      • interleukin 1 blocking agents - Anakinra, Rilonacept, Canakinumab
        • NICE have suggested that (4):
          • Anakinra is recommended as an option for treating Still's disease with moderate to high disease activity, or continued disease activity after non-steroidal anti-inflammatory drugs (NSAIDs) or glucocorticoids. It is only recommended for:
            • adult-onset Still's disease that has responded inadequately to 2or more conventional disease-modifying antirheumatic drugs (DMARDs)
            • systemic juvenile idiopathic arthritis in people 8months and older with a body weight of 10kg or more that has not responded to at least 1conventional DMARD.
      • interleukin 6 blocking agent - Tocilizumab 
      • B cell depletion agent - Rituximab 
  • systemic corticosteroids
    • often used in JIA induction therapy or in the management of disease flares
      • e.g. -while waiting for DMARDs to take effect and in patients with severe systemic or polyarticular juvenile idiopathic arthritis unresponsive to treatment with synthetic and biological DMARDs
    • used mainly in the treatment of children with polyarticular or systemic disease  - given as a high-dose ‘pulse‘ of 30mg/kg daily intravenous methylprednisolone for three days followed by a weaning course of oral prednisolone starting between 1–2mg/kg daily (1,2)

Other treatments for JIA include:

  • physiotherapy - to maintain normal muscle and joint function
  • rehabilitation
    • e.g. - heat or cold treatment, massage, therapeutic exercise, and splints
    • aids in returning to normal daily activity
  • recommended by British Society for Paediatric and Adolescent Rheumatology standards of care guideline
    • psychological therapy and education
    • regular checks by a paediatric rheumatologist, ophthalmologist, dermatologist, orthopaedic surgeon, orthodontist, general practitioner, psychologist, and physiotherapist or occupational therapist according to the subtype and severity of JIA (1)

Reference:

Related pages:

aspirin

ibuprofen

indomethacin

methotrexate

NICE guidance - Tofacitinib for treating juvenile idiopathic arthritis