JIA guidelines push for timely immunization, early biologic use, fewer corticosteroids
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Despite minimal evidence available, authors of the 2020 ACR juvenile idiopathic arthritis guidelines emerged with nearly 50 recommendations, emphasizing early use of biologics, regular immunizations and reduced reliance on corticosteroids.
“We wanted the guidelines to be comprehensive to JIA,” Susan Shenoi, MS, MBBS, RhMSUS, interim division chief of rheumatology at Seattle Children’s Hospital, said in her presentation, noting that the document covers oligoarthritis, temporomandibular joint (TMJ) arthritis and systemic JIA with or without macrophage activation syndrome (MAS).
Shenoi said the aims and objectives of the guideline development committee were fourfold.
“It was to develop recommendations for the use of glucocorticoids, non-biologic and biologic DMARDs for the treatment of children with oligoarticular JIA, taking into consideration both safety and efficacy,” she said.
The group also wanted to develop recommendations for the same therapeutic armamentarium in TMJ arthritis and develop similar screening guidelines in systemic JIA.
“Then, [we wanted to] develop guidelines around medication monitoring, imaging, infection screening, immunization, and non-pharmacologic treatments of children with JIA in general,” Shenoi said.
Guideline Development
The group also aimed to develop a document that was “timely, relevant and feasible in clinical practice,” according to Shenoi. The overarching goal was to provide clinicians guidance in situations when there “is less evidence than we typically like.”
While Shenoi described the guideline development process as “fairly complex and convoluted,” it largely adhered to the process seen in many rheumatology recommendation documents from recent years. Namely, the group developed Population- Intervention- Comparator- Outcomes (PICO) questions, conducted a rigorous literature review, employed the grading of recommendations assessment, development and evaluation (GRADE) methodology to make decisions and included a patient panel.
Recommendations were either strong or conditional and based not only on the strength of evidence backing them, but on the “balance desirable and undesirable consequences,” Shenoi said.
JIA Recommendations
Karen Brandt Onel, MD, a pediatric rheumatologist at the Hospital for Special Surgery in New York, reported that the result of this process was 47 recommendations. “The strength of those recommendations is largely conditional,” she said. “The quality of supporting evidence is largely very low.”
“Similar to the RA guidelines, although we know that corticosteroids are important, especially for children as regards growth stunting and weakened bones, we really try to avoid their use whenever possible so that was a critical part of what we presented,” Onel said during a press conference. “Secondly, strong recommendations for immunizations. Thirdly, early use of biologics, especially for children with systemic juvenile arthritis. And our first toe into the water of recommending stopping medications when children [exhibited inactive disease]. These were crucially important and I think really represent an advance forward.”
For oligoarthritis with fewer than five joints involved, intra-articular glucocorticoids are recommended as part of initial therapy, while triamcinolone acetonide is recommended as a preferred therapy, according to Onel. While NSAIDs are recommended initially, oral glucocorticoids are not.
“Non-biologic DMARDs are recommended [in cases of] no or incomplete response or intolerance to NSAIDs and/or intra-articular glucocorticoids,” Onel said of children with oligoarthritis with fewer than five joints involved. In the case of non-response or incomplete response to any of those drugs, a biologic is recommended for these patients.
For children with TMJ arthritis, intra-articular glucocorticoids are conditionally recommended initially. “There is no preferred agent,” Onel said. NSAIDs may conditionally be used in these patients, but oral glucocorticoids should not be added.
As with oligoarthritis, non-biologics may be used in the case of no response or poor response to other options, and biologics may be used with non-response to biologics or other options.
For children with systemic JIA without MAS, conditional recommendations exist for NSAIDs and interleukin)-1 and IL-6 inhibitors, while there is a strong recommendation for single over combination biologic therapy in the case of non-response or insufficient response to other options.
Turning to children with MAS — which Onel described as “a major cause of morbidity and mortality in children with sJIA” — glucocorticoids are conditionally recommended as initial monotherapy, while IL-1 and IL-6 inhibitors are conditionally preferable to calcineurin inhibitors for mitigating MAS.
In the setting of glucocorticoid failure or incomplete response to IL-1 or IL-6 inhibition, biologic or non-biologic DMARDs are recommended for patients in the MAS category.
Looking more broadly, for children with sJIA with or without MAS, tapering or discontinuation of medications is recommended for children with inactive disease.
Beyond pharmacotherapy, Onel addressed lab monitoring and infection screening. “Infection screening is quite controversial,” she said. This is largely due to the necessity — or lack thereof, as the case may be — of tuberculosis screening in certain regions.
The committee felt “extremely strongly about” immunizations in children with sJIA, according to Onel. “There is no increased risk of flare,” she said, but stressed that live vaccines are contraindicated in children receiving immunosuppressive medications.
In addition, physical and occupational therapy are recommended, as is a healthy “age-appropriate diet” that includes calcium and fats, according to Onel. “Special diets should not be used,” she said. “Supplements and herbs are not recommended.”
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Onel K, Shenoi S. JIA Guidelines. Presented at: ACR Convergence 2020; November 5-9, 2020 (virtual meeting).
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