Lack of HLH in Familial Mediterranean Fever (FMF)
Authors' conclusion
Enhanced responsiveness to IFN-γ distinguishes sJIA/MAS from FMF patients, which may explain the lower occurrence of MAS in FMF.
Study context
- Population
- Patients with systemic juvenile idiopathic arthritis (sJIA)/macrophage activation syndrome (MAS) and patients with FMF
- Participants
- 5 vs 5
- Comparison
- In vitro PBMC responsiveness to IFN-γ: sJIA/MAS vs FMF
- sJIA/MASn = 5
- FMFn = 5reference
- Outcome
- IFN-γ-induced CXCL9, CXCL10 and IL-18 levels in PBMC supernatants before and after in vitro IFN-γ stimulation (45 min)
Methodological detailsin vitro PBMC stimulation with IFN-γ
- In vitro PBMC stimulation with IFN-γ
Main finding
As reported in the abstract
PBMC responsiveness to IFN-γ
- Baseline CXCL9 higher in MAS than FMF in a flare, with differences increasing after IFN-γ stimulation
- IFN-γ stimulation upregulated IL-18 production in MAS but not in FMF
Original publication
Lack of HLH in FMF. Pediatr Rheumatol Online J. Published 8 February 2025. doi:10.
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