“We tried hard to find phenotypes that represent the most common presentations and cover the vast majority of patients that we see. But there clearly will be some sJIA patients that have complex phenotypes that are not covered with our groupings,” Dr. Weiss says.
Efficacy of IL-1, IL-6 Inhibitors
As with other categories of JIA, the goal of therapy for sJIA is “prompt control of active inflammation and symptoms and the prevention of a number of disease- and/or treatment-related morbidities such as growth disturbances, joint damage, and function limitations,” according to the document. Children with sJIA can have a particularly refractory course and persistent disease that leads to high risk of joint damage and severe growth impairment.
Because of the role that IL-1 and IL-6 play in the inflammatory process of sJIA, new drugs targeting those cytokines have been the focus of an increasing number of clinical trials. The new IL-1 and IL-6 inhibitors included in these recommendations are canakinumab, rilonacept, and tocilizumab.
When the first recommendations about JIA treatment were issued two years ago, IL-1 and IL-6 inhibitors had not been approved for sJIA. “Their approval and clinical data published since the last set of guidelines have had a profound impact on how we approach treatment of sJIA,” Dr. Weiss says.
Dr. Ringold agrees that recommendations about use of IL-6 and IL-1 inhibitors included in these new guidelines represent the most substantial change and should be particularly helpful for physicians who may not be aware of recent advances in treatment.
“A significant number of children are still cared for by adult rheumatologists who may be less familiar with the pediatric data. These recommendations will assist them,” Dr. Ringold says. Cost implications of these new therapies were not considered in development of the recommendations, per RAND/UCLA methodology.
Initial and Continuing Therapy
Treatment recommendations are listed for each of the phenotypes, and information is given about inappropriate use of certain therapies in certain situations. Among recommendations for patients with active systemic features and varying degrees of synovitis are ones about initiating anakinra, as well as systemic glucocorticoid (GC) monotherapy, either oral or intravenous, and nonsteroidal antiinflammatory drug (NSAID) monotherapy. Therapeutic options and indications are outlined for the treatment of patients within this category who have continued disease activity, including recommendations about use of abatacept, anakinra, a calcineurin inhibitor, and canakinumab, as well as when to initiate methotrexate, leflunomide, tocilizumab, or a tumor necrosis factor (TNF)–alpha inhibitor.
