Current therapy for Systemic Juvenile Idiopathic Arthritis

What is the current therapy for Systemic Juvenile Idiopathic Arthritis? What is the prognosis?

Recommendations for the treatment of sJIA have been outlined by the American College of Rheumatology and the CARRA network. These recommendations provide guidance on the treatment of patients in three distinct clinical phenotypes (based upon presence/absence of systemic features, current number of swollen joints, and presence/absence of features concerning for MAS). A reasonable approach to therapy for sJIA is outlined in the below table

Approach to Therapy for Systemic Juvenile Idiopathic Arthritis

Clinical PhenotypeRecommended Treatment
No current active systemic features & varying levels of synovitisIf high active joint count (SJC > 4): first-line therapy is MTX or leflunomide for 3 months• If continued joint swelling after 3 months: bDMARDs (anakinra, tocilizumab, TNFα inhibitor, abatacept) should be initiatedNSAID monotherapy could be considered as well, but synovitis after 4 weeks should prompt initiation of DMARDs or anakinra
If low active joint count (SJC < 4): may initiate NSAID monotherapy and/or intraarticular steroids for 1 month• If continued joint swelling after 1 month: proceed to pathway for SJC > 4
Current active systemic features & varying levels of synovitisIn patients with moderate to severe systemic symptoms (physician global assessment of severity ≥ 5): first-line therapy is anakinra or GC monotherapy• If anakinra is used as first-line therapy, continued disease activity at 1 month should prompt a switch to tocilizumab or canakinumab• If GC monotherapy is used as first-line therapy, continued activity at 2 weeks should prompt addition of anakinra, tocilizumab, or canakinumab• MTX or leflunomide may be added to bDMARD if persistent arthritis• Adjunct intraarticular steroids & background GC can be used at any time
In patients with milder systemic symptoms (physician global < 5): first-line therapy is NSAIDs, GC monotherapy, or anakinra• If patient has no synovitis: first-line therapy with NSAIDs may be sufficient. If continued disease after 1 month, proceed with anakinra or GC• Adjunct intraarticular steroids & background GC can be used at any time
Features concerning for MAS are presentAnakinra (tocilizumab may be considered if anakinra failure/contraindication) Calcineurin inhibitor c
GC therapy c

bDMARD, biologic disease-modifying antirheumatic drug; GC, glucocorticoid; MAS, macrophage activation syndrome; MTX, methotrexate; NSAID, nonsteroidal antiinflammatory drug; SJC, swollen joint count.

a Active systemic features include any one of the following: fever, evanescent rash, lymphadenopathy, serositis, or hepatosplenomegaly

b Features concerning for MAS include any one of the following: persistent fever, cytopenias or falling cell line counts, falling erythrocyte sedimentation rate, hypertriglyceridemia, hypofibrinogenemia, hemophagocytosis, transaminitis, coagulopathy, organomegaly, low or absent natural killer cell activity, hyperferritinemia, and central nervous system dysfunction.

c Initial treatment options for patients with possible MAS are not necessarily mutually exclusive, and depending on the clinical situation combination therapy may be necessary.

sJIA course may proceed as a monophasic (42%), polycyclic (characterized by periods of remission interspersed with flares; 7%), or persistent disease (51%). During periods of active disease, linear growth is retarded, thus complicating physical growth and development of the child. Pulmonary disease may complicate sJIA patients and may be the cause of mortality in some patients.


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