NextGen 2024: Refractory SJIA & MAS Session Part 1

NextGen 2024: Refractory SJIA & MAS Session Part 1

@SJIA_Foundation
@SJIA_Foundation
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2 weeks ago 65

NextGen 2024: Refractory SJIA & MAS Session Part 1

@SJIA_Foundation2 weeks ago

The changing face of SJIA
Grant Schulert, MD PhD
Associate Professor of Pediatrics
Division of R…
1/10
Complicated or refractory SJIA
• Outcomes for children with SJIA have markedly improved with intro…
2/10
Defining refractory SJIA
• Refractory SJIA (broadly)
– Failure to respond to IL-1 AND 
IL-6 bloc…
3/10
Defining refractory SJIA
• “Persistent partial MAS”
– A patient with active SJIA and 
persistent…
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What is refractory SJIA biologically?
• Most severe patients (correct treatments but strong not en…
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New refractory courses - IBD
• Anecdotally – also increasing
• Case series lead by Betsy Mellins …
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New refractory courses – lung disease
• “SJIA-LD” likely encompasses several disease phenotypes (s…
7/10
Might “SJIA-LD” be a distinct phenotype of the 
disorders classified as “SJIA”??
• Much younger a…
8/10
Atypical rashes in systemic JIA - definitely bad 
but maybe not always DRESS?
• 30 patients with …
9/10
What about HLA-DRB1*15? Could this be 
marker for refractory disease phenotypes?
• How common is …
10/10


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