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Proteinase 3 (PR3) is known to be closely related to systemic lupus erythematosus (SLE) and a high level of PR3 is associated with a poor prognosis in SLE. However, although PR3 has been studied in adult patients, little is known about PR3 in juvenile-onset SLE (JOSLE). We measured the levels of PR3 in plasma and its clinical significance in 20 patients with JOSLE and age-matched controls. The level of PR3 was higher in the JOSLE group than in the control group. The PR3 level was highly correlated with disease activity in JOSLE. A strong correlation was found between PR3 and anti-double-stranded DNA (anti-dsDNA). The levels of PR3 in patients in remission were lower than those in active disease, but not significantly different from those of controls. The findings of this study suggest that PR3 may be a useful marker for the evaluation of disease activity
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