Updated draft guidelines on juvenile idiopathic arthritis (JIA) from the American College of Rheumatology (ACR)’s JIA guideline team recommend decreased reliance on nonsteroidal anti-inflammatory drugs (NSAIDs) and glucocorticoids overall, a push toward earlier introduction of disease-modifying antirheumatic drugs (DMARDs), and a new preference for oral methotrexate (MTX) over the subcutaneous form.

And for the first time, the updated draft guidelines recommend routine screening for systemic JIA-associated lung disease in all patients with systemic JIA.

The expanded draft guidelines on JIA offer 33 new recommendations on nonsystemic JIA (including dactylitis for the first time) and 15 new recommendations covering systemic JIA (SJIA), which, all told, reflect therapeutic advances and rapid evolution in the management of JIA — a disease that often persists into adulthood, Susan Shenoi, MBBS, MS, a core member of the JIA guideline team, said at the 2026 Rheumatology Winter Clinical Symposium (RWCS).

The guidelines are expected to be published later this year and include a strong recommendation for ophthalmic screening every 3 months in JIA patients at high risk for JIA-associated uveitis.

The preference for oral over subcutaneous MTX comes as a conditional recommendation based largely on data from the German BIKER registry that suggests similar efficacy and fewer side effects with oral therapy. It represents a reversal of existing published guidelines, as does another conditional recommendation against oral glucocorticoids as part of initial therapy in JIA (except for life-threatening macrophage activation syndrome [MAS] in SJIA).

“Previously, we’d said bridging oral glucocorticoids for severe disease activity in those with high-risk features was OK to use,” said Shenoi, professor and clinical director of the Division of Pediatric Rheumatology at Seattle Children’s Hospital and Research Center, Seattle. The new draft guidelines favor earlier DMARD therapy with “much decreased reliance on glucocorticoids that have several side effects in growing children.”

The guidelines were developed using the GRADE methodology, which considers both the quality of evidence and other factors such as patient/family values, feasibility, and access.

Overall, the level of evidence for the draft guidance “is of low or very low certainty,” she said. Many of the recommendations are “conditional” recommendations, which reflect a lower level of confidence that desirable effects of a management strategy outweigh undesirable effects.

Nonsystemic JIA: Guidance Highlights

For oligoarthritis, step-up therapy is still preferred. But for other phenotypes of nonsystemic JIA — polyarthritis, enthesitis, dactylitis, and temporomandibular joint (TMJ) arthritis — biologic and/or conventional synthetic (cs) DMARDs are now strongly recommended as part of initial therapy.

“We’ve done away with the dogma of step-up therapy for polyarthritis. It’s such a huge change for us; it gives me goosebumps,” Shenoi said at the RWCS meeting. “It allows us flexibility to start combination therapy with biologic and conventional DMARDs earlier if desired.”

Driving the change is data from two large observational studies, both published in 2025. In the STOP-JIA comparative effectiveness trial using the North American Childhood Arthritis and Rheumatology Research Alliance (CARRA) Registry, children with polyarthritis who received combination therapy (biologics and MTX together) within 2 months of diagnosis were significantly more likely to “achieve inactive disease over time, clearly telling us that the sooner we get our patients on better drugs, the better,” Shenoi said.

The UCAN CAN-DU study, which enrolled patients with JIA across Canada and Netherlands, similarly showed that early start of biologics was associated with more patients reaching inactive arthritis. “Eighty-three percent achieved inactive disease if started on a biologic in the first 6 months. That’s huge,” she said.

The draft guidelines conditionally recommend MTX (oral) when conventional DMARDs are chosen and TNF inhibitors as first-line biologics. “And for immunogenic TNF inhibitor users, we conditionally recommend using a conventional synthetic DMARD concurrently, not just for synergistic effects, but also to help prevent antidrug antibodies,” Shenoi said.

For patients with inadequate response or intolerance to JAK inhibitors, newly recommended options include switching to another TNF inhibitor or trying a different biologic DMARD or a JAK inhibitor.

In the case of oligoarthritis that doesn’t respond to a trial of scheduled NSAIDs or first-line intra-articular glucocorticoids — or to subsequent therapy with a nonbiologic DMARD (preferably MTX) — the draft guidelines strongly recommend stepping up to a biologic DMARD, preferably a TNF inhibitor. (TNF inhibitors and other options to consider downstream if needed are conditionally recommended.)

As in prior guidance, consideration of risk factors for poor outcomes is strongly recommended to guide treatment decisions across categories of nonsystemic JIA. Risk factors include involvement of the ankle, wrist, sacroiliac joint, hip, or TMJ; erosive or symmetric disease; enthesitis; delay in diagnosis; elevated inflammatory markers; and rheumatoid factor or cyclic citrullinated peptide positivity.

SJIA: Guidance Highlights

In the absence of MAS, the draft guidelines continue to recommend interleukin (IL)-1 or IL-6 inhibitors, but this time, it’s a strong recommendation. Given the absence of head-to-head trials, no preferred agent is suggested. And in a reversal, NSAIDs are strongly recommended against as initial monotherapy.

Additionally, oral glucocorticoids as initial monotherapy are again conditionally recommended against, and csDMARDs as initial monotherapy continue to be strongly discouraged.

After initial therapy, the draft guidelines now base subsequent therapy recommendations on the presence or absence of systemic or residual arthritic symptoms. In the presence of ongoing systemic symptoms after initial therapy for SJIA without MAS, switching to a different IL-1 or IL-6 inhibitor or to targeted synthetic (ts) DMARD (JAK inhibitor) is conditionally recommended over adding a csDMARD or glucocorticoids. IL-1 and IL-6 inhibitors are preferred over JAK inhibitors, Shenoi said.

For a patient whose systemic symptoms are controlled but who has ongoing arthritis, on the other hand, there are several options: switching to a different bDMARD (IL-1 inhibitor, IL-6 inhibitor, abatacept, TNF inhibitor, etc.) or to a tsDMARD (JAK inhibitor) or adding a csDMARD or intra-articular glucocorticoid injections (IAGCIs). (The conditional recommendation also offers a preferred order: MTX and/or IAGCI over an alternative bDMARD over a JAK inhibitor.)

In the presence of MAS, IL-1 or IL-6 inhibitors and/or systemic glucocorticoids are now strongly recommended for initial therapy. For persistent MAS, “There’s now a strong recommendation to get disease under control with a different bDMARD [including the newly approved emapalumab] or a tsDMARD like a JAK inhibitor, and these are recommended over the addition of a csDMARD,” she said, noting that the panel “couldn’t reach consensus on a preferred order.”

Emapalumab is a monoclonal antibody against interferon gamma approved in July 2025 for MAS in Still’s disease (pediatric or adult-onset) that is refractory to systemic glucocorticoids.

Screening and Treatment of SJIA Lung Disease

The new conditional recommendation for routine screening for systemic JIA-associated lung disease is significant, Shenoi said. “It’s still an area of controversy in the pediatric community as we’ve started to see more lung disease over the last few decades,” she said. “The question has been, how do we screen for lung disease, and how do we treat it? Should we continue IL-1s and IL-6s, which have been revolutionary for this disease? Or should we stop these drugs?”

There is an ongoing debate about the etiology of systemic JIA lung disease. Some believe it is a Drug Reaction with Eosinophilia and Systemic Symptoms, while “others believe in the cytokine plasticity hypothesis,” she explained.

The draft guidelines weigh in on the ongoing debate, with a new recommendation that the presence of SJIA-lung disease should not be considered an absolute contraindication to the use of IL-1 and IL-6 inhibitors.

Regarding screening, the guidelines do not specify a recommended screening method. But “if there’s one thing you take away from this, I hope it’s universal screening of all your SJIA patients for lung disease,” Shenoi said at the RWSC.

Deprescribing Recommendations

The draft JIA guidelines also adopt the term “deprescribing” — a term increasingly used in medicine to describe the supervised reduction or discontinuation of drugs that might be causing harm or are no longer needed — and, based on recent data, offer deprescribing algorithms for JIA in clinical remission on DMARDs.

“Personally, I’m not sure deprescribing is a good idea, but it’s often what the families want…and I get it,” Shenoi said. If patients flare after deprescribing, “we recommend restarting the most recently effective systemic therapy…overusing intra-articular glucocorticoid injections.”

The draft guidelines also suggest performing imaging of difficult-to-examine joints before deprescribing, she said.

JIA-Associated Uveitis

Draft guidance on JIA-associated uveitis contains a new conditional recommendation against intraocular or periocular glucocorticoid injections as part of therapy, “and that’s because these [injections] carry a high risk of associated glaucoma and cataracts in our kids,” Shenoi said at the meeting.

For patients newly diagnosed with chronic anterior uveitis, it is conditionally recommended that a DMARD (csDMARD and/or bDMARD) be added to topical glucocorticoids with no recommended waiting period. “This is a big change” from prior recommendations, she said, and “gives you flexibility to do conventional step-up therapy or to start with early combination therapy at the get-go.”

Adalimumab is conditionally recommended as the first bDMARD over other DMARDs, based on findings from randomized controlled trials showing that the agent was superior to placebo in children with JIA-associated uveitis taking MTX. Infliximab and tocilizumab are next in line.

And in another major change, above-standard dosing of TNF inhibitors at treatment onset is conditionally recommended. “We know now…that much higher doses are required to cross the blood-ocular barrier,” Shenoi said. “Hopefully when these papers come out, you can use them to get higher doses approved [by payers].”

Given that JIA-associated uveitis is asymptomatic and has high morbidity, the draft guidelines strongly recommend ophthalmic screening every 3 months in patients at high risk. “Unlike adult uveitis, which presents as a red, painful eye, in children the eye [with chronic anterior uveitis] is white, quiet, and silent,” she said. “Oftentimes, we’ve picked up uveitis because the kids have gone for their routine eye screenings.”

To adult rheumatologists, Shenoi emphasized, “We recommend screening eyes in perpetuity, and our recommendations are now strong.”

Shenoi disclosed serving as a consultant for Pfizer and having consulted for Sobi and Cabaletta.