Systemic onset Juvenile Idiopathic Arthritis (SoJIA): a monocentric study of 114 patients

Results During the study period, 114 children were included. Mean age at diagnosis was 4 years and 4 months (range, 4 months to 15.5 years). Familial history of autoimmunity was found in 27% of the patients. Glycosylated ferritin was low (≤20%) for 79% of patients. Twelve percent of patients had macrophage activation syndrome, 2 patients anti-neutrophil cytoplasmic antibodies (ANCA) associated glomerulonephritis, 3 patients pulmonary restrictive syndrome without vertebral arthritis, and one patient Crohn’s disease; 11 % had positive antinuclear antibody (ANA) (≥1/160) at diagnosis. None developed neoplasia. Patients in whom diagnosis was made after 2000, and had been treated by biotherapy, especially anakinra, had less osteoarticular sequelae and lower inflammatory syndrome (p = 0,004) than others (Figure 1). Early polyarticular and wrists involvements were associated to a poor outcome (p = 0,009, p = 0,027).