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New Treatment Guidelines for Juvenile Idiopathic Arthritis

New Treatment Guidelines for Juvenile Idiopathic Arthritis

Major changes include a greater emphasis on early disease-modifying treatments, shared decision-making and immunizations.

Final publication of new clinical practie guidelines for the treatment of juvenile idiopathic arthritis (JIA) are expected soon from the American College of Rheumatology (ACR).

JIA is the most common rheumatic disease in children, causing significant short- and long-term disability

JIA is the most common rheumatic disease in children, causing significant short- and long-term disability for about 1 to 20 per 100,000 children. There are several types of JIA: oligoarticular, affecting four or fewer joints; polyarticular, affecting five or more joints; enthesitis-related arthritis; psoriatic arthritis and systemic onset. With all types, joint inflammation, pain and stiffness can be chronic and continue into adulthood.

The ACR set out to completely revamp the guidelines several years ago due to multiple advances in JIA treatment. Part one, published in 2019, covers treatments for polyarthritis, sacroiliitis, enthesitis and uveitis. Part two, final publication of which is expected in early 2022, covers treatments for oligoarthritis, TMJ arthritis and systemic arthritis as well as recommendations for imaging, medication monitoring, non-pharmacologic treatments and immunizations.

50%of children and teens with JIA are cared for by adult, not pediatric, rheumatologists

“The updated guideline was necessary to incorporate recently approved medications and shifting treatment paradigms,” says Karen Brandt Onel, MD, Chief of the HSS Division of Pediatric Rheumatology at HSS, who was selected as principal investigator for part two. “Given that about half of children and teens with JIA are cared for by adult, not pediatric, rheumatologists, we hope the new guideline will be helpful for them, too.”

Here are Dr. Onel’s answers to some key questions on the new JIA guideline.

Q: What were the major updates from the previous JIA guideline, published in 2011 and 2013?

A: First, these new guidelines recommend early use of disease-modifying antirheumatic drugs, both conventional synthetic and biologic drugs, and a decreased use of nonsteroidal anti-inflammatory drugs and oral corticosteroids. Second, they stress the importance of shared decision-making with parents, caregivers and patients. And third, as a reflection of the time, the guidelines make strong recommendations supporting regularly scheduled immunizations for children and teens with JIA.

Q: How was the methodology different from the previous guidelines and who contributed to their development?

A: This is the first set of JIA guidelines to use the Grading of Recommendations Assessment, Development and Evaluation [GRADE] approach to evaluate the strength of the available evidence in answering PICO [population, intervention, control and outcomes] questions. As a result, our recommendations reflect varying levels of evidence quality with caveats noted to assist clinicians in determining what’s right for their patients.

Given limited evidence, we sought to minimize potential conflict of interest. As chair of the ACR's Committee on Ethics and Conflicts of Interest, it is essential to me to ensure that guideline contributors be as free from potential bias as possible. I invited a mix of experts for the core team, the voting panel and the literature review team and put out a call for interested others to apply. Overall, only one-third of our contributors were conflicted, bettering the best practice for guideline development as suggested by the National Academy of Medicine, which sets a limit of less than 50% of the total.

Q: How did including patient and parent panels benefit guideline development?

A: Patient and parent panels shared valuable perspectives. Some of their opinions were the same and some were different from ours. All parents agreed that when a child or teen has JIA, it affects the whole family, including navigating finances and copays, infusion units only open during school and work hours and repeat appointments for monitoring. This insight kept us laser-focused on the fact that there are real people at the other end of our recommendations.

All parents agreed that when a child or teen has JIA, it affects the whole family... This insight kept us laser-focused on the fact that there are real people at the other end of [the guidelines]
Q: What was your biggest challenge?

A: The literature is not as voluminous for JIA as it is for other diseases, and most evidence was very low or low quality; none was moderate or high quality. JIA is also complex, with many phenotypes, and there are many medications to consider, including recently approved tofacitinib (Xeljanz®) and golimumab (Simponi Aria®). In some areas, we did not follow the manufacturers’ package inserts. For example, screening for hepatitis infection makes sense for adults, but we did not recommend it for children since an effective vaccine has been in use for the past 20 years and its incidence among children absent comorbidities is exceptionally rare in the United States. In other areas, we harmonized with adult recommendations, such as reducing the monitoring schedule for methotrexate where appropriate.

Q: Did you intentionally leave anything out of the new treatment recommendations?
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A: We removed timeframes, which have been in every previous version of the guidelines. We did not want insurers and other groups to insist patients wait three months before starting treatment, because that’s too long to wait for children who get sicker by the minute.

The evidence shows that aggressive, early treatment helps many children improve and do incredibly well. Rheumatologists should treat patients according to their symptoms and risk factors, in a shared decision-making process that takes their preferences into account. Insurers are not part of that decision.

Q: Why the shift to recommending immunizations and does that include the COVID-19 vaccine?

A: There is voluminous data showing that vaccines do not cause disease flares in children or teens with JIA. As trusted partners with our patients, we need to make sure they get their immunizations, including influenza and COVID-19 vaccines. The Centers for Disease Control and Prevention’s guidelines for immunizations for immunosuppressed patients are clear and the ACR also categorically says that all children should be immunized against infectious diseases.

Q: How often will the guidelines be updated?

A: It takes years to conduct a review process and formulate a guideline update, but it’s essential to keep pace as new medications are approved and treatment paradigms evolve. The 2022 JIA Guideline should be considered a work-in-process road map that will continue to be filled in over the years, as required.

Pediatric Rheumatology
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