The American College of Rheumatology releases two updated guidelines for treatment of juvenile idiopathic arthritis

The American College of Rheumatology (ACR) released two updated guideline papers for the treatment and management of Juvenile Idiopathic Arthritis (JIA). These two guidelines are companions to previously updated JIA guidelines released by the ACR and Arthritis Foundation in 2019 covering the treatment of polyarthritis, sacroiliitis, uveitis and enthesitis. One paper provides updates on the pharmacologic management of JIA, focusing on treatment of oligoarthritis, temporomandibular (TMJ) arthritis and systemic JIA (sJIA), with and without macrophage activation syndrome (MAS). The other focuses on non-pharmacologic therapies, medication monitoring, immunizations and imaging, irrespective of JIA phenotype.

The original JIA guidelines were published in 2011 and 2013, and this update reflects the ever-changing rheumatology field with new criteria on how to define disease and new medications to treat those diseases.

"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," said Karen Onel, MD, Chief of the Pediatric Rheumatology Division at the Hospital for Special Surgery in New York and the 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."

One example of where the guideline team has adapted is in their recommendations for using disease-modifying antirheumatic drugs (DMARDs). The guideline on the pharmacologic management of JIA emphasizes early use of conventional synthetic and biologic disease-modifying antirheumatic drugs. This is a much different treatment approach than what was previously recommended.

"For many years, treatment of JIA consisted of corticosteroids, non-steroidal anti-inflammatory drugs (NSAIDs), physical therapy, bracing and surgery. There were no DMARDs and even if there were, they were not tested or used in children," said Dr. Onel. "These guidelines stress the early use of conventional synthetic and biologic DMARDs and the avoidance of glucocorticoids and NSAIDs. In fact, for systemic JIA the guidelines suggest using biologic DMARDs as a first line. We have turned the pyramid upside down."

Two areas of importance for the non-pharmacologic paper were guidance on how to monitor drug toxicities for children with JIA and the importance of immunization. Laboratory test monitoring recommendations for medications such as NSAIDs, methotrexate, and hydroxychloroquine can be found on pages 6-9. There was strong support for the use of immunizations in children with JIA and specific guidance for children with JIA receiving immunosuppression, not on immunosuppression, and children who are under-immunized or unimmunized can be found on pages 10-11. Additionally, the guideline recommends the use of physical therapy and occupational therapy interventions and a healthy, well-balanced, age-appropriate diet.

Though the scope of the two guidelines differs, one thing they have in common: the importance of shared decision-making with the patient/caregiver.

"Not every decision will be appropriate for every patient, which is why it was so instrumental to receive input from both patients and caregivers when creating these recommendations," said Dr. Onel.

While the guideline was being developed, the COVID-19 pandemic began, and COVID-19 immunization became possible. As none of the currently available vaccines against COVID-19 are live vaccines, recommendations for use in JIA should be similar to those stated for inactivated vaccines. While specific guidance on immunizing children with rheumatic diseases against COVID-19 is still lacking, the ACR has published guidance on COVID-19 vaccines for adults with rheumatic and musculoskeletal diseases.

At the time the manuscript was approved for publication, the Pfizer-BioNTech COVID-19 vaccine was approved for emergency use in children 5-15 years of age in the U.S and FDA approved for adolescents 16-18. In addition, two new medications were also approved while the guidelines were developed. These will be considered for future updates.

"There were areas that we didn't consider at the start that now belong. The guidelines will have to be updated again. But that is a sign of a growing and changing field," said Dr. Onel.

Like many other ACR guidelines, the updated guidelines for JIA were developed using Grading of Recommendations Assessment, Development and Evaluation (GRADE) methodology, which creates rigorous standards for judging the quality of the literature available and assigns strengths to the recommendations.