Treatment Guidelines for JIA

Updated 3/30/22

The American College of Rheumatology (ACR)’s guidelines for treating juvenile idiopathic arthritis (JIA) focus on several facets and subsets of this pediatric disease. Several sets of guidelines complement each other and are periodically updated with consideration to the body of evidence and the most recent research and clinical knowledge.

The Arthritis Foundation partnered in 2019 with ACR, an international medical society, to produce two sets of JIA treatment guidelines with input from patients. One focuses on screening, monitoring and treating patients with the eye disease uveitis associated with JIA . The other guideline provides therapeutic approaches for three subsets of JIA: non-systemic polyarthritis, which involving five or more joints (often parallel, such as both knees or both wrists); sacroiliitis, or inflammation of the area where the spine meets the pelvis; and enthesitis, or inflammation of sites where tendons or ligaments attach to bone.

A panel of pediatric rheumatologists, parents and adult JIA patients reviewed the evidence and issued 39 recommendations, classified as either “strong” or “conditional,” based on the quality of evidence, benefits versus harms, and patient preference. The quality of evidence was generally poor, in some cases it is simply lacking, resulting in mostly “conditional” recommendations.

“These recommendations highlight the importance of prompt and effective treatment for children with JIA and polyarthritis, sacroiliitis and enthesitis,” says pediatric rheumatologist Sarah Ringold, lead author of the guidelines. “They also support relatively tight disease control, with inactive disease as the goal. While it is anticipated that these recommendations will lead to improved outcomes for children with these forms of JIA, they also emphasize the ongoing need to generate high-quality data about treatment effectiveness in JIA.”

The recommendations are intended to help guide treatment decisions, but each patient and/or parent should work closely with the child’s doctor to make shared decisions on the best course of treatment for that patient. Following are some of the key treatment recommendations.

Uveitis, or inflammation of the front part of the eye (uvea), is common with JIA, so patients should have regular screenings by an ophthalmologist, who should coordinate with the child’s rheumatologist. Uncontrolled, uveitis can lead to vision loss, glaucoma and other problems. Uveitis is sometimes asymptomatic, and it may occur as acute or chronic disease.

• Acute anterior uveitis (AAU) is a form of uveitis that typically occurs in children with spondyloarthritis (those with enthesitis-related or psoriatic arthritis). It is episodic and characterized by a sudden onset of pain, redness and light sensitivity. It usually can be treated topically and doesn’t require systemic treatment.
• Chronic anterior uveitis (CAU) develops in 10% to 20% of JIA patients and is usually asymptomatic, so there is rarely obvious evidence of inflammation. But CAU can lead to vision loss, so regular ophthalmic exams are necessary.

Strong Recommendations

• If uveitis is controlled and corticosteroids are being reduced, get ophthalmic screenings within a month of each dosage change.
• If uveitis and therapy are stable, get screened no less than every three months.
• If systemic therapy (like methotrexate ) is being reduced, get screened within two months of changing dosage.
• Patients and parents should be educated about the warning signs of AAU.
• If uveitis is well controlled with a biologic and/or DMARD, make sure it’s stable at least two years before reducing the medications.

Conditional Recommendations

• Children with JIA at high risk of developing uveitis should have ophthalmic screenings every three months. High risk includes those with oligoarthritis, polyarthritis (rheumatoid factor negative), psoriatic arthritis, or undifferentiated arthritis who are also antinuclear antibody positive ; who were younger than 7 years old at disease onset and have had it no more than four years.
• Initial treatment for uveitis is topical corticosteroids, and methotrexate is typically the first systemic treatment.
• Using prednisolone acetate 1% topical eye drops is preferred over difluprednate topical eye drops. If additional therapy is needed, a short-term course of topical corticosteroids is recommended over systemic corticosteroids.
• Add systemic therapy to reduce eye drops for those requiring one or two eye drops a day. After three months of eye drops and systemic therapy, changing or increasing systemic therapy is recommended.
• Those who develop uveitis despite systemic therapy are should try eye drops before changing or increasing systemic therapy.
• Start with injectible rather than oral methotrexate to treat active CAU.
• Those starting a biologic should first try a monoclonal antibody TNF inhibitor over etanercept ( Enbrel ).
• Those with severely active CAU and complications that threaten their sight should start methotrexate and a monoclonal antibody TNF inhibitor immediately rather than methotrexate alone. If there’s an inadequate response, then increase the dose or frequency of the biologic before switching to another.
• If methotrexate and these first two biologics don’t have the desired results, then try abatacept ( Orencia ) or tocilizumab ( Actemra ) as biologics and mycophenolate, leflunomide or cyclosporine as conventional DMARDs.
• If uveitis is well controlled with a DMARD and/or biologics but an isolated, short-lived episode of AAU occurs, treat it with corticosteroid eye drops before considering a different therapy.
• If uveitis is well controlled and medications are being tapered, start by reducing corticosteroid eye drops before the systemic therapy.

Strong Recommendations

• A long course of oral corticosteroids is strongly recommended against .

Conditional Recommendations

• Treatment with nonsteroidal anti-inflammatory drugs (NSAIDs) are conditionally recommended along with other medications but not as the sole medication.
• Treatment with methotrexate, a conventional, synthetic disease-modifying antirheumatic drug (DMARD) , is conditionally recommended over the conventional DMARDs leflunomide or sulfasalazine. It is also conditionally recommended over “triple therapy,” a combination of three DMARDS. Injectible is preferred over oral methotrexate.
• Corticosteroid injections into joints is conditionally recommended as an additional therapy, and triamcinolone hexacetonide is strongly recommended as the injection over triamcinolone acetonide.
• In patients with moderate or high disease activity, a short course of oral corticosteroids is conditionally recommended until other medications kick in, but not in those with low disease activity.
• Initial treatment with a biologic drug combined with a DMARD is conditionally recommended, and if the biologic is infliximab ( Remicade ) , the addition of a DMARD is strongly recommended.
• Physical and occupational therapy is conditionally recommended for those at risk of functional limitations.
• If the initial therapy isn’t having an adequate effect in a child with low disease activity, additional treatments might include joint corticosteroid injections, increasing the DMARD dose or adding a biologic.

• If the initial therapy isn’t having an adequate effect in a child with moderate to high disease activity, additional treatments might include adding a biologic to the initial DMARD (conditionally recommended over triple therapy). Or, if the child is on a biologic called a tumor necrosis factor (TNF) inhibitor, switching to either a different TNF inhibitor or a different kind of biologic is conditionally recommended.

JIA AND SACROILIITIS

Strong Recommendations

• Start treatment with an NSAID, and if it isn’t effective enough, add a TNF-inhibitor biologic. (Those who can’t take or don’t have an adequate response to the biologic are conditionally recommended to use sulfasalazine instead.)
• Do not treat with methotrexate alone.

Conditional Recommendations

• Treat with a short course of oral corticosteroids until other medications kick in. And try joint injections with corticosteroids if needed.
• Those at risk of functional limitations should get physical therapy.

JIA AND ENTHESITIS

Strong Recommendations

• Start with an NSAID, and if it isn’t effective enough, add a TNF-inhibitor biologic rather than methotrexate or sulfasalazine.
• Try a short course of oral corticosteroids until other medications kick in, and joint injections with corticosteroids if needed.
• Those at risk of functional limitations should try physical therapy.