Management for systemic JIA

Management for systemic juvenile idiopathic arthritis (JIA), under the direction of a paediatric rheumatology service, is based on a multidimensional approach that incorporates pharmacological and nonpharmacological interventions.

Management for systemic JIA by a specialist paediatric rheumatology service involves:

  • comprehensive assessment (including blood investigations)
  • managing systemic features based on their severity—the therapies recommended for systemic features may also have benefits for arthritic features
    • patients with mild initial systemic symptoms can be managed in primary care—nonsteroidal anti-inflammatory drugs (NSAIDs) may be adequate and can also provide relief of arthritic symptoms; they are rarely appropriate for more severe systemic symptoms
    • patients with more severe initial systemic symptoms require admission to hospital—intravenous corticosteroids are used for initial symptom management; oral corticosteroids are subsequently used to maintain control of systemic features and may induce remission of arthritis
    • patients with persistent or recurrent severe systemic features not controlled by oral corticosteroids require disease-modifying antirheumatic drugs (DMARDs) for long term management—methotrexate, despite not being effective for systemic features, is used because it is a prerequisite for accessing biological DMARDs via the Pharmaceutical Benefits Scheme (PBS). It also has the benefit of being effective for arthritic symptoms. Biological DMARDs are effective for treating both systemic and arthritic features
  • managing arthritic features in the majority of patients whose arthritis persists despite treatment for systemic features—management is as for rheumatoid factor-negative polyarticular JIA
  • implementing nonpharmacological interventions such as physiotherapy to manage symptoms, and maintain overall health and wellbeing
  • screening for JIA-associated uveitis
  • helping adolescents achieve supported self-management before their transition into adult care
  • providing the patient and their caregivers with information about the diagnosis and its management, and developing a shared understanding
  • tailoring multidisciplinary care to the patient’s needs
  • regularly assessing the patient’s pain intensity, function and quality of life, and the adequacy of therapy.