What are the ACR treatment guidelines for systemic juvenile idiopathic arthritis (JIA)?

Updated: Jul 25, 2019
  • Author: David D Sherry, MD; Chief Editor: Lawrence K Jung, MD  more...
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In September 2013, the ACR released updated guidelines for the treatment of systemic JIA, which included the medications canakinumab, rilonacept, and tocilizumab. [31, 32] These guidelines include the following treatment recommendations:

  • For systemic JIA with active systemic features and varying degrees of synovitis, initial treatment for most patients should consist of anakinra with systemic glucocorticoids

  • For systemic JIA without active systemic features and with varying degrees of active synovitis, initial treatment should be methotrexate or leflunomide for an active joint count higher than 4, with a change to abatacept, anakinra, a tumor necrosis factor (TNF)-α inhibitor, or tocilizumab if disease activity continues after 3 months; for patients with 4 or fewer active joints, NSAID monotherapy or intra-articular glucocorticoid injections should be initial treatment

  • For systemic JIA with features suggesting macrophage activation syndrome (MAS), initial treatment should include anakinra, a calcineurin inhibitor, or systemic glucocorticoid monotherapy for up to 2 weeks

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