New recommendations for juvenile idiopathic arthritis (JIA) were published in 2013, updating the previous set of recommendations from 2011 (Ringold S, et al. Arthritis Care Res [Hoboken]. 2013;65:1551-1563). The recommendations encompass 3 common phenotypes of JIA and incorporate the following agents not included in the previous guidelines: canakinumab (Ilaris) and rilonacept (Arcalyst), both anti–interleukin (IL)-1 agents, and the anti–IL-6 agent tocilizumab (Actemra).
“Systemic JIA is one of the most difficult categories of JIA to treat, and the updated recommendations now include first-line and second-line treatment recommendations for children with this disease,” said Sarah Ringold, MD, MS, Seattle Children’s Hospital and the University of Washington School of Medicine, Seattle. Dr Ringold and Pamela Weiss, MD, The Children’s Hospital of Philadelphia and University of Pennsylvania’s Perelman School of Medicine, Philadelphia, were coprincipal investigators for the 2013 treatment recommendations.
Drs Ringold and Weiss and colleagues reviewed the literature to develop the new JIA recommendations using the RAND/UCLA Appropriateness Method to develop evidence-based recommendations by determining interventions that provide a health benefit exceeding that of health risk by a wide margin. The recommendations took about a year to develop and were voted on by an American College of Rheumatology task force panel of pediatric rheumatologists and researchers. The updated recommendations differ in several ways from the 2011 version:
- The phenotypes are different
- Previous risk stratification was not used
- Anakinra (Kineret), nonsteroidal anti-inflammatory drugs (NSAIDs), and systemic glucocorticoids are still recommended as initial therapeutic options for patients with active systemic features, based on slightly different disease activity parameters
- Methotrexate/leflunomide are now an initial therapeutic option for systemic JIA without systemic features but active arthritis. Previously, all children had to have up to 1 month of NSAIDs plus or minus intra-arterial injection
- Canakinumab and tocilizumab are now considered additional therapeutic options for continued disease activity.
The new recommendations also incorporate repeat tuberculosis testing for all children with JIA and for children on biologics with an initial negative scan. Repeat testing can be undertaken at any point if the risk changes from moderate to high.
Anakinra is now an option for first-line treatment of patients with active systemic features and synovitis, and patients with JIA and features of macrophage activation syndrome—a potentially fatal complication occurring in about 10% of JIA patients. Canakinumab and tocilizumab are recommended as second-line options for patients with active systemic features and synovitis. Anakinra and tocilizumab are also recommended as options for second-line treatment for patients without active systemic features but with active synovitis.
These recommendations provide guidance for healthcare professionals who treat JIA for appropriate initiation of therapeutic agents, and they are not intended to supplant individualized patient care, the investigators emphasized, because phenotypes and patient scenarios cannot encompass all possible presentations of JIA.
The authors note that the updated guidelines have several limitations, including no validated disease activity score; low levels of evidence for many recommendations; and no specific dose, route, or tapering for glucocorticoid administration.