Systemic juvenile idiopathic arthritis (SJIA) confers high risk for macrophage activation syndrome (MAS), a life-threatening episode of hyperinflammation driven by IFN-. Monocytes in SJIA display IFN-hyperresponsiveness, but the molecular basis of this remains unclear. The objective of this study is to identify circulating monocyte polarization phenotypes including features of interferon response. Bulk RNA-seq of purified monocytes was performed on healthy controls as well as patients with inactive SJIA (satisfy Wallace criteria for clinically inactive disease), active SJIA, new-onset SJIA (NOS), and macrophage activation syndrome (2016 MAS Classification Criteria). We observed marked transcriptional changes in patients with elevated ferritin levels. We also identified substantial overlap with multiple polarization states but little evidence of IFN-induced signature. SOURCE: Grant Schulert (grant.schulert@cchmc.org) -  Cincinnati Children's Hospital

Pluto Bioinformatics

GSE147608: Transcription profiling of monocytes from patients with active and inactive systemic juvenile idiopathic arthritis.