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5 Articles in this Series
Introduction
ACR Releases Updated Draft Guidelines for Juvenile Idiopathic Arthritis (JIA)
Debate: When Methotrexate Fails – The Use of JAK or TNF Inhibitors
Pharmacotherapy & Rheumatic Disease in Older Adults
Rheumatoid Disease Therapy and Immunological Complications
When Rheumatoid Arthritis Treatment Gets Difficult: 3 Cases Offer Potential Solutions

ACR Releases Updated Draft Guidelines for Juvenile Idiopathic Arthritis (JIA)

An ACR Convergence 2020 Meeting Highlight with Eyal Muscal, MD, MS, Karen Onel, MD, and Daniel Horton, MD, MS

New, updated draft guidelines for juvenile idiopathic arthritis (JIA) were presented at ACR Convergence 2020, taking place the first week of November. The guidelines, which still need to be finalized and approved by the American College of Rheumatology (ACR), emphasize disease-modifying treatments and urge continued inactivated or non-live immunizations.

JIA is the most common type of arthritis in children and teens. About 300,000 children in the US have some form of arthritis or rheumatic disease. JIA was formerly called juvenile rheumatoid arthritis (JRA); the name changed because it is not simply a child's version of the adult disease. The term juvenile arthritis is now used to describe all joint conditions that affect kids and teens, including JIA.

While the ACR and Arthritis Foundation issued guidelines just last year for JIA, those guidelines targeted non-systemic polyarthritis, sacroiliitis, and enthesitis. The new proposed recommendations make up the second half of a set of new guidelines, covering recommendations for systemic JIA, oligoarticular JIA, and temporomandibular joint (TMJ) pain. The drafting process included input from experts, patients, and parents as well as a peer review of electronic databases for studies to provide evidence for the recommendations, which are graded by strength of the evidence using the Grading of Recommendations Assessment, Development and Evaluation model.

At the ACR virtual session on November 8, expert panelists involved in the developing of the guidelines discussed how they might play out in clinical practice. At a press conference on November 9,   Karen Onel, MD, chief of pediatric rheumatology at the Hospital for Special Surgery in New York City and lead investigator for the guidelines, discussed the draft recommendations further.

She noted that about half of children and adolescents with JIA are cared for by adult rheumatologists – not pediatric rheumatologists – so she is hopeful the updated draft recommendations will be helpful to them as well.

Overview of the 2020 Draft JIA Treatment Guidelines

In total, there are 47 recommendations contained in the draft guidelines. While the strength behind the recommendations is largely conditional, said Dr. Onel, the update is still a high priority for ACR because new medications have been approved and treatment paradigms for JIA have changed. Among the major shifts is a recommendation to reduce the use of steroids to treat acute inflammation and a larger emphasis on using biologic drugs.

Other noteworthy recommendations include:

  • Most children with JIA should undergo regular immunizations for infectious diseases, including annual influenza shots, following CDC and the American Academy of Pediatrics guidelines, Dr. Onel said. This is a departure from the last published guideline. Evidence suggests many vaccines will not cause a flare, she explained,  however, live virus immunizations should be avoided in children receiving immunosuppressive medications, as the CDC also recommends.
  • NSAIDs should be used for a shorter period. Experts say NSAIDs do not treat underlying inflammation and can cause unpleasant side effects, such as gastritis.
  • Shared decision-making on treatment plans should take place between patients, their families, and the physicians. The panelists noted that having a child with arthritis affects all aspects of life in a household, such as missed school and work, finances, the need to get medical or hospital care, and the time and sometimes travel needed to do so.
  • The guidelines address the need for medication monitoring, lab testing, infection screening, and blood tests (to check liver function and other parameters) before treatment begins. The infection screening guideline was controversial, Dr. Onel noted, and the panel of experts did not reach consensus on all points. “When dealing with a diverse population in demographics and risk profiles, it is challenging to make a blanket recommendation for screening infection,” she said. “What works for one is unlikely to work for all, so screening needs to reflect the community standards and needs.” What they did agree on was the need for immunization to prevent infection.
  • Physical therapy and occupational therapy are among the nonpharmacological approaches that may be tried (with or without medications).
  • Special diets and supplements are not advised. This is the same recommendation as in the last round of guidelines, Dr. Onel said, as there is no evidence to support that a specific diet will treat childhood arthritis. The best advice? “A healthy-age-appropriate diet,” she said.

Applying the New Draft Guidelines

Daniel Horton, MD, MS, assistant professor of pediatrics at the Robert Wood Johnson Medical School of Rutgers University, provided some case histories to illustrate how the new guidelines may be put into practice once approved, including the following sample.

A 3-year-old girl is diagnosed with oligoarticular JIA. She has involvement in both knees and one ankle, with a JADAS (Juvenile Arthritis Disease Activity Score) of 11. After talking over treatment options, the family agrees to a trial on NSAIDs and to returns in 1 month. At the 1-month follow-up, the patient’s JADAS score is 7 and there is new joint involvement, the left wrist, ''which is worrisome.” 

The new treatment plan would be to prescribe this patient a biologic DMARD and methotrexate. The patient would be advised to take folic acid supplements and the clinician should order blood work (CBC, renal and liver function, TB test) before DMARD treatment begins. The clinician may share with the family evidence advising against live vaccinations, such as MMR and the varicella booster, that this patient will soon be due to receive.      

The folate advised here, Dr. Onel explained, is specifically to help with methotrexate side effects, such as GI distress and mouth sores. The other recommendations against supplement use refer to their use specifically to treat JIA. There is no evidence as yet, she said, that a particular herb or supplement will treat childhood arthritis and ''we don't know enough about safety in childhood.”)

Overall, Dr. Horton told the ACR audience to remember that the “levels of evidence supporting these [draft] recommendations are generally quite low,” and that there is ''plenty of room for clinical judgment.”

On the other hand, Dr. Onel pointed out that, while the scarcity of evidence was sometimes ''disheartening,” there is ''quite a lot of evidence” for the recommendations that were made.

Expert Perspective

“Shared decision-making seems to be front and center” in the updated guidelines, said Eyal Muscal, MD, MS, associate professor at Baylor College of Medicine who moderated the ACR panel. He said that since many of the recommendations were not graded strongly due to insufficient evidence, providers are largely going to be left to make their own clinical decisions.

The takeaway, however, is the added emphasis on using targeted therapies earlier in the treatment process than previously recommended and to make less use of oral corticosteroids, said Dr. Muscal. “This approach is often impaired by American insurance companies,” due to lack of approval. “We all hope once guidelines are approved that advocacy efforts on state and national levels may allow clinicians to get biologics approved earlier.”

The ACR expects publication of the final guidelines in early 2021.  

 

Sources

Ringold S, Angeles-Han T, Beukelman T et al. 2019 American College of Rheumatology/Arthritis Foundation Guideline for the Treatment of Juvenile Idiopathic Arthritis: Therapeutic Approaches for Non-Systemic Polyarthritis, Sacroiliitis, and Enthesitis. Arthritis Care & Res. June 2019. Available at: https://www.rheumatology.org/Portals/0/Files/JIA-Guideline-2019.pdf

ACR Press Release: New Juvenile Idiopathic Arthritis Guideline Emphasizes Disease-Modifying Treatments, Urges Immunizations. November 6, 2020.  Available at: https://www.rheumatology.org/About-Us/Newsroom/Press-Releases/ID/1126

 

Next summary: Debate: When Methotrexate Fails – The Use of JAK or TNF Inhibitors
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