Rheumatology (Oxford). 2025 Oct 30:keaf576. doi: 10.1093/rheumatology/keaf576. Online ahead of print.
ABSTRACT
OBJECTIVE: This study aimed to clarify the activation profile of inflammasome-associated genes in systemic juvenile idiopathic arthritis (s-JIA).
METHODS: Sixteen patients with s-JIA, including four patients with macrophage activation syndrome (MAS) associated with s-JIA, and 10 healthy controls (HCs) were enrolled. Whole blood samples were collected upon diagnosis with s-JIA or MAS. mRNA was purified, and the mRNA expressions of inflammasome-associated genes was analyzed using the Human Inflammasomes RT2 Profilerâ„¢ polymerase chain reaction (PCR) Array (Qiagen Sciences).
RESULTS: The mRNA expression levels of six genes (NLRC4, AIM2, CASP5, NAIP, TNFSF4, MAPK11) were significantly elevated in patients with s-JIA compared with HCs. The expression of IRF1 and NFKB1A was significantly elevated in patients with MAS compared with those with s-JIA in the acute phase. The mRNA expression levels of patients with s-JIA were clustered into two distinct groups: the MYD88-dominant and NLRC5-dominant groups. The MYD88-dominant group had a significantly higher number of joints with active disease.
CONCLUSION: NLRC4, AIM2 inflammasome, and CASP5 activation may play an important role in s-JIA. IRF1 may be associated with the development of MAS. The distinct expression profile of inflammasome-associated genes in s-JIA may indicate the heterogeneity of s-JIA.
PMID:41165588 | DOI:10.1093/rheumatology/keaf576
