Updated guidelines for juvenile arthritis

The American College of Rheumatology (ACR) just updated its recommendations for the treatment of systemic juvenile idiopathic arthritis (JIA) and secondary tuberculosis screening among children receiving biologic medications.

The ACR believes the update constitutes an important contribution to the care of children with this challenging and disabling disease.

The updated treatment guidelines reflect data from randomized trials of new interleukin (IL)-1 inhibitors and IL-6 inhibitors in children that have been published since ACR last addressed the topic in 2011.

Another impetus for the update, according to the Task Force panel, is that experts now know that about 1 in every 10 children with systemic JIA develops overt clinical features of macrophage activation syndrome (MAS), a life-threatening condition characterized by fever, organomegaly, cytopenias, hyperferritinemia, hypertriglyceridemia, hypofibrinogenemia, and coagulopathy. Children hospitalized with systemic JIA and MAS have a mortality rate as high-or higher, according to some reports-as 6%.

The new recommendations, which used the RAND/University of California, Los Angeles (UCLA) Appropriateness Method, contain algorithms depicting treatment pathways for children with and without active systemic features of the disease and varying degrees of synovitis. Based on more than 1,200 clinical scenarios, the expert panel includes recommendations for use of nonsteroidal anti-inflammatory drugs, glucocorticoids, methotrexate, leflunomide, intravenous immunoglobulin, calcineurin inhibitors, abatacept, rituximab, anakinra, canakinumab, rilonacept, and tocilizumab.

The panel did not consider cost in its therapy equations, nor did it address using combination therapy with a biologic agent due to safety concerns and lack of data.

According to the Arthritis Foundation, about 294,000 children under the age of 18 years are affected by pediatric arthritis and other rheumatologic conditions. According to the update, systemic JIA accounts for anywhere between 4% and 15% of that total. 

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