EULAR: Systemic JIA, adult-onset Still’s disease ‘are the same disease’
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Systemic juvenile idiopathic arthritis and adult-onset Still’s disease have been combined into one entity called Still’s disease in new recommendations document presented at the EULAR 2023 Congress.
Bruno Fautrel, MD, professor of rheumatology and head of the department of rheumatology at the Pitié-Salpêtrière University Hospital, in Paris, and Fabrizio De Benedetti, MD, PhD, head of the division of rheumatology at Ospedale Bambino Gesù, in Rome, presented novel guidelines — which are shared between EULAR and the Pediatric Rheumatology Society — for the disease.
“The first [overarching principle] was that systemic JIA and adult-onset Still’s disease are the same disease and should be called by the same name, and that is Still’s disease,” Fautrel said.
The guideline development team was balanced between adult and pediatric rheumatologists. Two patient partners were also included.
The team identified four main issues for the document, according to Fautrel.
“The first one was to highlight why we want these two entities to be one single disease,” he said.
The second topic was about diagnosis, particularly early diagnosis. The third involved treatment paradigms, while the fourth covered complications like macrophage activation syndrome and other comorbidities.
As with other EULAR guidelines, the document contains three over-arching principles, the first of which identified the combined condition as Still’s disease. For the second principle, Fautrel stressed that “age matters” in terms of how care should be organized, and how treatment targets should be approached. The diseases were previously stratified via a patient cutoff age of 16 years, with those younger being managed through pediatric protocols and those older being treated as adults.
Moreover, a treatment target of drug-free remission should be used for all patients, regardless of age, according to Fautrel.
“Thus, the second principle is that treatment should be based on shared decision-making between the parent and treating team, or patient and treating team,” he said.
The third principle is that macrophage activation syndrome should be detected and treated as early as possible.
“Despite very powerful, innovative therapies, we continue to see macrophage activation syndrome,” Fautrel said. “We have to be really concerned about this potential complication.”
Regarding the recommendations, the first is that fever, rash and high levels of inflammation should be the main diagnostic criteria for pediatric and adult rheumatologists encountering these patients.
In addition, arthritis or arthralgias may or may not be present in Still’s disease, according to Fautrel. Clinicians should not wait for this development before initiating treatment. “Arthritis is likely to happen after a few weeks of evolution of the disease,” he said. “But it is not necessary to make the diagnosis.”
Regarding treatment targets, Fautrel noted that clinically active disease is defined as an absence of Still’s disease clinical activity and normal erythrocyte sedimentation rate (ESR) and C-reactive protein.
“Remission is defined as no clinical activity for more than 6 months,” he said.
For patients who may have Still’s disease but who have not been formally diagnosed, NSAIDs may be initiated to manage symptoms.
“It is a bridging therapy,” Fautrel said.
Although glucocorticoids can also be efficacious, Fautrel offered a warning about these drugs.
“We want to emphasize that long-term glucocorticoid use must be avoided due to safety issues,” he said.
Interleukin (IL)-1 or IL-6 inhibition should be initiated as soon as possible once a diagnosis of Still’s disease has been made.
Later, De Benedetti presented the group’s findings on macrophage activation syndrome. He noted that this complication should be considered in patients with fever, splenomegaly, elevated serum ferritin, low cell counts, abnormal liver function test, intravascular activation of coagulation or high triglycerides. The M- or H-score may be used for diagnostic purposes, as may the MAS 2016 criteria.
“Eventually, for treatment of macrophage activation syndrome, the mainstay remains high-dose glucocorticoids,” De Benedetti said. “Interferon-gamma targeted therapies are available and should be used promptly in severe patients.”
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Fautrel B. EULAR-PReS Recommendations on the management of sJIA and AOSD. Presented at: EULAR 2023 Congress; May 31 to June 3, 2023, Milan, Italy.
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