The American College of Rheumatology (ACR) has published guidance regarding the treatment and management of juvenile idiopathic arthritis (JIA), with emphasis placed on the treatment of oligoarthritis, systemic JIA (both with and without macrophage activation syndrome (MAS), monitoring drug toxicities, immunizations, and radiographic imaging. The original guidelines were published in 2011 and 2013.
“As rheumatologists, our patients and caregivers expect us to review the literature and weigh the evidence so that we can suggest the best treatments, while also considering their preferences,” stated Karen Onel, MD, lead investigator of the guidelines. “The field has changed tremendously since the 2011 and 2013 efforts, so we needed to adapt our guidance to the times in order to offer our patients the most nimble and state-of-the-art care possible.”
Non-pharmacologic Therapies Guideline:
- Rheumatologists should discuss maintaining a healthy, age-appropriate diet.
- Specific diets to treat JIA is strongly recommended against due to lack of evidence supporting diet alone to treat JIA, the risk of nutritional deficits, and other harms (eg cost, delay in treatment).
- Folic or folinic acid, in conjunction with methotrexate (MTX) is strongly recommended and may mitigate adverse events while improving tolerability.
- Complete blood counts (CBCs), liver function tests (LFTs), and renal function tests should be used to monitor patients within the first 1-2 months of MTX and every subsequent 3-4 months. While rates of liver toxicity are low, there is a rare potential for serious harm.
- Tofacitinib should be altered if laboratory results are abnormal and discontinued if hemoglobin level is 2 gm/dl or for severe neutropenia. This recommendation was added considering the 2020 FDA approval of tofacitinib.
- Inactivated influenza immunization is strongly recommended for all children with JIA.
- Both live and inactivated immunizations are strongly recommended for children with JIA who are not being treated with immunosuppressive drugs.
- Vaccines are strongly recommended for household contacts of children with JIA who are receiving immunosuppressive treatment to minimize risk of exposure in the home.
- Radiography as a screening test prior to advanced imaging is strongly recommended against as it is not sensitive enough to properly assess and may delay appropriate imaging and treatment. It may also harm the development of the child.
Pharmacologic Therapies Guideline:
- Intraarticular glucocorticoids (IAGCs) are strongly recommended as part of initial therapy for active oligoarthritis.
- Triamcinolone hexacetonide is strongly recommended as the preferred IAGC for the treatment of oligoarthritis due to low rates of adverse events and high likelihood of sustained response.
- Conventional synthetic disease modifying antirheumatic drugs (csDMARDs) are strongly recommended if there is inadequate response to nonsteroidal anti-inflammatory drugs (NSAIDs) and/or IAGCs for the treatment of oligoarthritis.
- Biologic DMARDs (bDMARDs) should be used if there is an inadequate response or intolerance to NSAIDs and/or IAGCs and at least 1 csDMARD.
- Temporomandibular (TMJ) arthritis should be treated with csDMARDs if patient exhibits inadequate response to or intolerance of NSAIDs and/or IAGCs.
- Conventional synthetic DMARDs are strongly recommended against as initial monotherapy in sJIA without MAS.
- IL-1 and IL-6 are strongly preferred over a single or combination of csDMARDs for patients with sJIA without MAS who have an inadequate response or intolerance to NSAIDs and/or glucocorticoids.
- Biologic DMARDs or csDMARDs are strongly recommended over long-term glucocorticoids for patients with sJIA (both with and without MAS) who have an incomplete response to IL-1 and/or IL-6 inhibitors.
- Tapering and discontinuing glucocorticoids is strongly recommended for patients with sJIA who have attained inactive disease. Risk of flares outweigh potential harms from long-term glucocorticoid use.