Treatment recommendations for patients with systemic arthritis and active arthritis (and without active systemic features). These recommendations are intended for patients with juvenile idiopathic arthritis who have systemic arthritis with active arthritis and without active systemic features. Recommendations are based upon duration of current therapy, disease activity, and features of poor prognosis. If criteria for escalation of therapy are not met, then continue current therapy along with adjunct nonsteroidal antiinflammatory drugs (NSAIDs), as needed. Recommendations for reduction of therapy are not addressed. See Table 5 for definitions of disease activity and features of poor prognosis. MTX = methotrexate; TFNα = tumor necrosis factor α; * = initiation of anakinra for the treatment of arthritis may be less appropriate later in the disease course compared to nearer the onset of disease; † = switching from anakinra to a TNFα inhibitor may be appropriate for some patients with moderate or high disease activity, irrespective of features of poor prognosis, but there is a possible risk of unmasking latent systemic features when discontinuing anakinra.