Panelists Explain ACR’s Expanding JIA Guidelines

Experts from the ACR’s juvenile idiopathic arthritis (JIA) guideline team reviewed key recommendations and changes. Owing to numerous therapeutic advances — and emerging challenges — the recommendations have expanded.

In the session Charting the Path Forward: 2024 DRAFT Updates to ACR JIA Guidelines, Karen Onel, MD, Chief of Pediatric Rheumatology, Hospital for Special Surgery at Weill Cornell Medical School, said the development emphasizes unconflicted voting panels to ensure unbiased recommendations.

Recorded sessions at ACR Convergence 2025 are available on demand to all registered meeting participants through October 31, 2026, by logging in to the meeting website.

The guideline format has been restructured to include shifting immunization guidance to vaccine-specific guidelines and introduces the term “deprescribing,” which refers to the supervised reduction or cessation of medications that may no longer benefit the patient, she explained.

A multidisciplinary panel collaborated to define clinical questions using the PICO framework, which takes into account patient/population/problem, intervention, comparison, and outcome considerations.

“Literature reviews and evidence grading are central, with the grade system factoring in patient preferences and practical considerations,” Dr. Onel noted, emphasizing that even if most evidence is rated as low or very low, strong recommendations can still be made when the risk of missing a diagnosis is high.

Systemic JIA: Guideline Updates and Evolving Practice

The updates reflect the rapid evolution in the field since 2021; and with a more complex landscape of systemic JIA, they have been expanded accordingly — from nine recommendations in 2021 to 15 in 2025.

“The approach to systemic JIA has changed,” according to Susan Shenoi, MBBS, MS, RhMSUS, Professor at the University of Washington, Seattle, and Clinical Director of the Pediatric Rheumatology Division at Seattle Children’s Hospital and Research Center. “The new algorithm reflects disease heterogeneity and individualized care.”

The now 15 recommendations reflect greater stratification based on clinical presentation. For patients without macrophage activation syndrome (MAS), initial therapy recommends interleukin-1 (IL-1) or interleukin-6 (IL-6) inhibitors, elevating these agents to a strong recommendation. Neither agent is preferred, with head-to-head trials lacking. In a shift, nonsteroidal anti-inflammatory drugs (NSAIDs) are strongly recommended against as initial monotherapy. NSAIDs may be used during diagnosis, but more effective treatments should follow confirmation.

“For those without MAS, management is stratified by the presence of systemic versus arthritic symptoms,” Dr. Shenoi explained. “For patients with systemic features, the guidelines recommend switching to a different biologic disease-modifying antirheumatic drug (DMARD) or targeted synthetic DMARD, with IL-1 or IL-6 inhibitors preferred over Janus kinase (JAK) inhibitors.”

For patients with predominant arthritic symptoms, options include tumor necrosis factor (TNF) inhibitors, targeted synthetic DMARDs, or conventional synthetic DMARDs, such as methotrexate, as well as intra-articular glucocorticoid injections. The preferred order is the addition of methotrexate or intra-articular glucocorticoid injection over alternative JAK inhibitors, according to the draft guideline, she said.

As MAS remains a life-threatening complication, the guidelines strongly recommend IL-1 or IL-6 inhibitors for initial therapy. For persistent MAS, options are switching to a different IL-1 or IL-6 inhibitor, targeted synthetic DMARDs (JAK inhibitors), or conventional synthetic DMARDs, such as cyclosporine or tacrolimus.

“Emapalumab, approved for Still’s disease MAS, is another therapeutic avenue,” Dr. Shenoi said.

Systemic JIA-associated lung disease is increasingly recognized, and the guideline now conditionally recommends routine screening.

“While specific screening modalities are not prescribed, this empowers clinicians to tailor screening based on available resources and patient needs,” Dr. Shenoi emphasized.

For patients who achieve clinical inactive disease, the guideline strongly recommends tapering and discontinuing glucocorticoids and DMARDs rather than abrupt cessation.

“However, consensus was not reached regarding the optimal duration of inactive disease before deprescribing,” Dr. Shenoi said. “If patients flare after deprescribing, the recommendation is to restart the most recently effective regimen rather than initiating a new one.”

Data from the CARRA JIA study indicate that approximately 70% of systemic JIA patients recaptured disease control after flaring from medication discontinuation, she noted.

“As we move forward, I encourage all clinicians to adopt these recommendations and remain engaged in the evolving debate around optimal management strategies for systemic JIA,” Dr. Shenoi said.

Non-Systemic JIA Guidelines

“The guidelines in non-systemic JIA bring over 50 recommendations, with more than half representing new directions. These are designed to help clinicians make evidence-based decisions,” said Daniel Horton, MD, MS, Associate Professor of Pediatrics and Epidemiology and Chancellor’s Scholar at Rutgers University. He is a member of the Core Oversight Team of the ACR JIA Management Guidelines Committee.

A key general recommendation remains: Clinicians should always consider risk factors for core outcomes when making treatment decisions. The presence of one or more risk factors should prompt consideration of more aggressive therapy.

“DMARDs are strongly recommended as initial therapy for most phenotypes, with step-up therapy preferred for oligoarthritis,” he noted.

For polyarthritis, a conditional recommendation favors oral methotrexate over subcutaneous administration, due to similar efficacy and fewer side effects. TNF inhibitors are conditionally recommended as first-line biologics for polyarthritis, oligoarthritis, and temporomandibular joint (TMJ) arthritis. JAK inhibitors are not recommended as first-line advanced therapy due to limited safety data.

A conditional recommendation advises against routine use of oral glucocorticoids, except for short-term symptom relief at the lowest effective dose. For patients with inadequate response or intolerance to TNF inhibitors, options include switching to another TNF inhibitor, trying a different biologic, or considering a JAK inhibitor.

“For patients in clinical remission on combination DMARD therapy, tapering or stopping the conventional agent first is conditionally recommended to reduce flare risk,” Dr. Horton said. “When deprescribing biologics, tapering is preferred over abrupt cessation. Imaging of difficult-to-examine joints (e.g., TMJ, C-spine) is advised before deprescribing to detect subclinical synovitis.”

JIA-Associated Uveitis

“Screening for uveitis is strongly recommended in high-risk patients, despite limited evidence. The panel agreed that early detection is critical, given the severe consequences of missed cases,” said Sheila T. Angeles-Han, MD, MSc, a Professor of Pediatrics and Assistant Professor of Ophthalmology at Cincinnati Children’s Hospital Medical Center and the University of Cincinnati.

Intraocular/periocular glucocorticoids are now conditionally recommended against in children. With ophthalmologists on board, panelists recognized the importance of explicitly discouraging the use of ocular steroids in pediatric patients, Dr. Angeles-Han explained.

“For children and adolescents with active chronic anterior uveitis, initial DMARD therapy is conditionally recommended, with topical glucocorticoids continued,” she said. “No waiting period before starting systemic treatment; and combination therapy (methotrexate plus TNF agents) can be initiated early.”

Adalimumab is conditionally recommended as the first biologic DMARD, followed by infliximab and tocilizumab, she said. Above-standard dosing of TNF inhibitors is also conditionally recommended at treatment onset, allowing for aggressive management.