CHICAGO—The draft of the new ACR guideline for juvenile idiopathic arthritis (JIA) will consider at least 17 new medications and offer updated recommendations on how to prioritize therapies, on screening, and on how to address flares.
Dr. Karen Onel
Karen Onel, MD, chief of pediatric rheumatology at the Hospital for Special Surgery, New York, and the principal investigator on the guideline, said the need for updated guidelines is “never-ending,” with the continued approval of new treatments for the disease. “We’re going to need to update these more frequently,” she said.
The guideline is compiled by three teams: a core team, a voting team and a literature review team. Within each group, [less than] than 50% of the members can have a conflict of interest that is specific and relevant to the topics discussed, according to Dr. Onel.
The guideline is written first by defining the questions of interest, then asking for a public review. This is followed by a literature search, collating the evidence, querying patients and families, and then voting based on the evidence.
Several layers of review are needed before the guideline is finalized. Each party involved in creating the guideline can read it and make comments. The ACR guideline committee reviews it, as does the ACR Board of Directors. The editors of Arthritis & Rheumatology review the guideline as they would any other article, Dr. Onel said. Any guideline and the recommendations have “a long way to go before they’re official,” she said.
sJIA Guideline Changes
Dr. Susan Shenoi
Susan Shenoi, MBBS, MS, RhMSUS, clinical director of pediatric rheumatology at Seattle Children’s Hospital, discussed several important changes to the systemic JIA (sJIA) recommendations:
- For sJIA without macrophage activation syndrome (MAS), interleukin (IL) 1 and IL-6 inhibitors are now strongly recommended as first-line therapy, with no specific agent preferred.
- Non-steroidal anti-inflammatory drugs (NSAIDs) are now strongly recommended against as initial monotherapy.
- For sJIA without MAS in the presence of systemic symptoms, a different biologic disease-modifying anti-rheumatic drug (bDMARD) or a targeted synthetic DMARD (tsDMARD) is conditionally recommended as subsequent therapy over a conventional synthetic DMARD (csDMARD) or a glucocorticoid (GC). The preferred order is an IL-1 or IL-6 inhibitor over a Janus kinase (JAK) inhibitor.
- For sJIA without MAS in the presence of residual arthritic symptoms, a different bDMARD or tsDMARD or adding a csDMARD or intra-articular GC injection is conditionally recommended over systemic GC, with the preferred order of methotrexate or intra-articular GC injection, or both, preferred over an alternative bDMARD, followed with a JAK inhibitor.
- For sJIA with MAS, initial therapy with systemic GC is strongly recommended. IL-1 or IL-6 inhibitors are strongly recommended.
- For sJIA with persistent MAS, a different bDMARD or tsDMARD is conditionally recommended over the addition of a csDMARD.
- Routine screening of all sJIA patients for lung disease is now conditionally recommended. And the presence or absence of sJIA-related lung disease should not be considered an absolute contraindication for the use of IL-1 or IL-6 inhibitors. No method has been recommended for performing this screening, allowing clinicians to choose based on what they can access.
- No consensus has been reached on the length of time for having clinically inactive disease before de-prescribing a DMARD in sJIA, Dr. Shenoi said.
- Upon a flare after de-prescribing a DMARD, it is now conditionally recommended to restart the same medication regimen that was most recently effective, over starting a new regimen.
Consider Risk Factors for Poor Outcomes
Dr. Daniel Horton
Daniel Horton, MD, MSCE, associate professor of pediatrics and epidemiology at Rutgers University, New Brunswick, N.J., reviewed the recommendations for polyarthritis, oligoarthritis, enthesitis, dactylitis and temporomandibular joint (TMJ) arthritis. Although this recommendation is not a change, he underscored that it was important to remember to consider the risk factors for poor outcomes, such as the involvement of the ankle, wrist, sacroiliac joint, hip or TMJ, or erosive or symmetric disease, when making treatment decisions because this might warrant rapid escalation or alternative medications.
- DMARDs, biologic and/or conventional, are now strongly recommended as part of initial therapy for polyarthritis, enthesitis, dactylitis and TMJ arthritis.
- Oral methotrexate is now conditionally recommended over subcutaneous methotrexate. And methotrexate is conditionally recommended over alternative conventional agents, which vary depending on the phenotype.
- Tumor necrosis factor (TNF) inhibitors are conditionally recommended as the first bDMARD over other bDMARDs, which vary depending on the phenotype.
- For TNF inhibitors users, it is conditionally recommended to use a csDMARD concurrently to improve effectiveness and prevent anti-drug antibodies.
- Oral GCs are conditionally recommended against as part of initial therapy. If used, they should be used at the lowest effective dose and for the shortest duration possible.
- It is conditionally recommended against doing routine monitoring for anti-drug antibody levels because they might not be neutralizing and because of the added cost of this monitoring, Dr. Horton said.
- In the event of an inadequate initial response or intolerance to a TNF inhibitor, it is conditionally recommended to try a second TNFi or a medication with a different mechanism, such as an IL-6 inhibitor, a T cell co-stimulation-modulator, IL-17 inhibitor, IL-12/23 inhibitor or JAK inhibitor.
- For clinical remission on combination DMARDs, tapering or stopping csDMARDs first is conditionally recommended over tapering or stopping bDMARDs or tsDMARDS.
- For clinical remission on bDMARDs, it is conditionally recommended to taper DMARDs over immediately stopping DMARDs to prevent an exacerbation.
- For clinical remission on DMARDS, when considering a taper or a stop, it is conditionally recommended to do imaging of joints that are difficult to assess, such as the TMJ or the C-spine, Dr. Horton said.
- For a flare after a DMARD taper or stop, it is conditionally recommended to restart the most recently effective DMARD regimen over starting a new regimen.
JIA-Associated Uveitis
Dr. Sheila Angeles-Han
Sheila Angeles-Han, MD, MSc, professor of pediatrics and assistant professor of ophthalmology at Cincinnati Children’s Hospital, discussed changes to JIA-associated uveitis recommendations.
- Ophthalmic screening every three months is now strongly recommended for patients at high risk for uveitis.
- Intra-ocular or periocular GC injections are conditionally recommended against as part of therapy.
- Starting a DMARD is conditionally recommended in patients who are newly diagnosed with clinically active uveitis and require topical GC, with no time frame for waiting before systemic treatment can begin.
- Starting adalimumab is conditionally recommended as the first bDMARD over other DMARDs.
- Using an above-standard JIA dose of a TNFi is conditionally recommended when starting treatment.
- Starting methotrexate is now conditionally recommended as the first csDMARD over other csDMARDs.
Volunteers Welcome
Dr. Onel said more help for updating the guideline is always welcome. “It’s a lot of work. If people are interested, please volunteer,” she said. “It’s really necessary.”
Thomas R. Collins is a freelance medical writer based in Florida.