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. Author manuscript; available in PMC: 2023 May 5.
Published in final edited form as: Arthritis Rheumatol. 2022 Mar 1;74(4):553–569. doi: 10.1002/art.42037

Table 6.

Systemic JIA with MAS*

Recommendation Certainty of evidence PICO evidence report(s) basis Page no(s). of evidence tables†
Formal recommendation deferred Very low PICO 24. In patients with Systemic JIA, does the presence of subclinical MAS alter the treatment paradigm? 130
IL-1 and IL-6 inhibitors are conditionally recommended over calcineurin inhibitors alone to achieve inactive disease and resolution of MAS.
Glucocorticoids are conditionally recommended as part of initial treatment of systemic JIA with MAS.

There is no preferred agent.		 Very low PICO 25. In patients with Systemic JIA and overt MAS, is biologic therapy superior to calcineurin inhibitors for achievement of inactive disease and resolution of MAS? 131–136
Formal recommendation deferred Very low PICO 26. For nonresponse or partial response to biologic therapy, is addition of a calcineurin inhibitor superior to etoposide or IVIG or plasmapheresis for achievement of inactive disease and resolution of MAS? 137–138
Biologic DMARDs or conventional synthetic DMARDs are strongly recommended over long-term glucocorticoids for residual arthritis and incomplete response to IL-1 and/or IL-6 inhibitors.

There is no preferred agent.		 Very low PICO 27. In patients with Systemic JIA in whom inactive disease is not achieved despite treatment with both IL-1 and IL-6 agents and/or who are chronically steroid dependent, is long-term stable steroid treatment superior to nonsteroid treatments (cyclophosphamide or abatacept or rituximab or IVIG or mesenchymal stem cell transplant or bone marrow transplant) for achievement of inactive disease, achievement of partial response, growth, ability to taper/discontinue steroids, and minimization of side effects/medication toxicity? 138
*

JIA = juvenile idiopathic arthritis; MAS = macrophage activation syndrome; PICO = Patient/Population, Intervention, Comparison, and Outcomes; IL-1 = interleukin-1; IVIG = intravenous immunoglobulin; DMARDs = disease-modifying antirheumatic drugs.