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.
