Autoinflammatory & rare systemic diseases
Free IL-18 in NLRC4-associated autoinflammatory disease without macrophage activation syndrome
Authors' conclusion
Germline and somatic NLRC4-AID without MAS are associated with elevated levels of both total and free IL-18.
Study context
- Population
- Patients with NLRC4-associated autoinflammatory disease (NLRC4-AID) without macrophage activation syndrome (MAS)
- Participants
- 5 vs 1
- Comparison
- FCAS4 vs Somatic NLRC4-AID
- FCAS4n = 5
- Somatic NLRC4-AIDn = 1
- Outcome
- Serum free IL-18, total IL-18 and related cytokines
Main finding
As reported in the abstract
- Total and free IL-18 elevated in both FCAS4 patients and the somatic NLRC4-AID patient
- No relevant correlation between total and free IL-18 and laboratory markers of inflammation or MAS
Further findings
MAS-associated cytokines IFN-γ, CXCL9 and CXCL10
- Not elevated in NLRC4-AID patients without MAS
Original publication
Free IL-18 in NLRC4-associated autoinflammatory disease without macrophage activation syndrome. Rheumatology (Oxford). Published 13 July 2026. doi:10. 1093/ rheumatology/ keag363
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