For patients without active systemic features and varying degrees of synovitis, intraarticular GC injection is recommended as an initial treatment option for certain patients, and methotrexate or leflunomide for others. Indications are given for use of NSAID monotherapy in patients without prior treatment. Therapeutic options for patients with continued disease activity include, under certain situations, abatacept, anakinra, canakinumab, tocilizumab, methotrexate or leflunomide, and a TNF-alpha inhibitor.
Initial therapeutic options for patients with sJIA with features concerning for MAS include anakinra, a calcineurin inhibitor, and systemic GC monotherapy. The authors note that development of treatment recommendations for this phenotype was challenging, given that there are no diagnostic or even classification criteria for MAS complicating sJIA and the etiology is probably heterogeneous. These recommendations will most likely be modified and expanded as more data about treatments become available.
Use of combination therapy with a biologic agent was not considered in the development of the recommendations and is not recommended because of concerns about safety and the lack of data. Any use of a biologic, therefore, should be part of a sequential therapy with prior biologic agents discontinued before starting a new one.
Dr. Ringold noted that the level of evidence for many of the recommendations is quite low. “I think some people may be surprised by the overall low level of evidence for some of the accepted approaches to therapy.” A higher level of evidence about efficacy of the new drugs, particularly the IL-1 and IL-6 inhibitors, is available, with data drawn from randomized trials, but often with small patient populations.
TB Screening
Public comment spurred inclusion of new recommendations about repeat TB screening for patients with sJIA. These new recommendations specify that annual screening is inappropriate for children at low risk of TB who had an initial negative TB test.
However, if the child’s TB risk changes to moderate or high, as determined by regional infectious disease guidelines, the screening should be repeated before beginning treatment with a biologic agent.
2013 SSc Classification Criteria
The 2013 Classification Criteria for Systemic Sclerosis, published in Arthritis & Rheumatism, represent an improvement on the 1980 ACR criteria, which classified diffuse SSc but did not include as many patients within the limited subset, resulting in at least 20% of patients with limited cutaneous disease not being classified correctly, according to Janet Pope, MD, MPH, a coauthor of the 2013 document and ACR principal investigator.2 Dr. Pope is professor of medicine in the division of rheumatology at the University of Western Ontario, Schulich School of Medicine.
