This protocol covers patients under 18 years of age with systemic juvenile idiopathic arthritis (systemic JIA) who do not have macrophage activation syndrome (MAS). Systemic JIA is distinct from all other categories of JIA due to fever, rash, and visceral involvement and is considered by some to be an autoinflammatory disorder.
The preceding treatment line involved an IL-1 or IL-6 inhibitor — anakinra, canakinumab, or tocilizumab — with switching among and between IL-1 and IL-6 inhibitors as needed. This protocol is triggered when that therapy did not achieve resolution of systemic signs or inactive disease, and residual joint synovitis remains.
For residual arthritis following incomplete response to IL-1 and/or IL-6 inhibitors, biologic or conventional synthetic DMARD therapy is strongly recommended over long-term glucocorticoids. The full protocol specifies the recommended options and sequencing — see the complete regimen below.
The clinical targets for this line are resolution of residual joint synovitis and achievement of inactive disease.
DOI: 10.1002/art.42037