Innate Lymphoid Cell Phenotypic and Functional Alterations in Patients With Systemic Juvenile Idiopathic Arthritis
Authors' conclusion
Patients with sJIA in CID exhibit significant innate immune abnormalities, including altered ILC subset distribution and impaired IFNγ production, strongly associated with IL-18 levels.
Study context
- Population
- Children with systemic juvenile idiopathic arthritis (sJIA) during clinically inactive disease receiving IL-1 inhibitors
- Participants
- 40 / 23 / 22
- Comparison
- sJIA in clinically inactive disease (CID) on IL-1 inhibitors vs healthy children and vs patients with unrelated autoinflammatory diseases on IL-1 inhibitors
- sJIAn = 40
- Healthy children (HC)n = 23reference
- Autoinflammatory diseasesn = 22
- Outcome
- Peripheral ILC phenotype and function in sJIA
Methodological detailsflow cytometry, plasma proteomic profiling
- Flow cytometry
- Plasma proteomic profiling
Main finding
As reported in the abstract
- Significant reduction in circulating natural killer (NK) cell frequencies vs HC, with increased proportion of CD56bright NK cells
- Overall hILC frequencies comparable to HC; ILC1s increased, ILC precursors reduced
- ILC1 frequency correlated positively, ILC2 frequency negatively, with IL-18 plasma levels
- NK cells had variable IFNγ production upon IL-18/IL-12 stimulation, inversely correlating with IL-18 levels
- Helper-ILCs (hILCs) showed specific impairment in IFNγ production despite normal IL-13 production, potentially linked to decreased IL-18 receptor α expression in ILC1s
- IL-18 the most up-regulated cytokine in sJIA plasma (proteomics)
Original publication
Innate Lymphoid Cell Phenotypic and Functional Alterations in Patients With Systemic Juvenile Idiopathic Arthritis. Arthritis Rheumatol. Published 27 June 2025. doi:10.
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