Type I Interferon Signature is Associated With Lung Disease, Drug‐Associated Immune Reactions, and Genetic Variation in Interferon‐Linked Pathways in Still Disease
Authors' conclusion
High IFN-I expression correlates with LD and DAIR in Still disease, linked to rare genetic variation in immune pathways.
Study context
- Population
- Consecutive patients with Still or Still-like disease (children and adults)32 children · 25 adults
- Participants
- 57 patients
- Exposure
- Elevated ISG-28 scores vs others
- High ISG-28n = 16
- Outcome
- Prevalence of LD, DAIR and IL-6 inhibitor use
Methodological detailsconsecutive patients, exome sequencing with family-based variant prioritization, functional enrichment analysis
- Consecutive patients
- Exome sequencing with family-based variant prioritization
- Functional enrichment analysis
Main finding
As reported in the abstract
Prevalence of lung disease (LD)
0.44 vs 0.1
P = 0.007
Elevated ISG-28
Prevalence of drug-associated immune reactions (DAIR)
0.63 vs 0.17
P = 0.003
Elevated ISG-28
IL-6 inhibitor use
0 vs 0.25
P = 0.048
Elevated ISG-28
Observational data: associations, not effects.
Further findings
HLA-DRB1*15 combined with high ISG-28 scores for LD and DAIR
- Combination associated with LD and DAIR with high specificity; absence of both biomarkers had high NPV
Candidate genes from high ISG-28 individuals
- Enriched in IFN-related pathways, incl. autophagy, IFN-I production, toll-like receptor signaling, macrophage activation, cytoskeletal organization, and responses to stress
Original publication
Type I Interferon Signature is Associated With Lung Disease, Drug‐Associated Immune Reactions, and Genetic Variation in Interferon‐Linked Pathways in Still Disease. Arthritis Rheumatol. Published 26 February 2026. doi:10.
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