Moreover, circulating mtDNA has been implicated in the TLR9-dependent inflammatory pathology of diverse diseases such as RA, atherosclerosis, and acute liver injury [29], and we hypothesized that AOSD neutrophils spontaneously release NETs enriched in mtDNA, leading to enhanced proinflammatory potential mainly via TLR9, while many mechanistic questions will need to be determined in our future studies. This evidence concerns the gene TLR9 and adult-onset Still disease.