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Rituximab (RTX) was added to the ongoing immunosuppressive treatment in 29 patients with refractory ANCA\u2010associated vasculitis. The disease activity was measured using Birmingham Vasculitis Activity Score\/Wegener\u2019s granulomatosis (BVAS\/WG score), and clinical laboratory variables were recorded. The median BVAS\/WG score before treatment was 6 (IQR 3\u20138), and 28 patients (97%) had disease flare classified either severe (62%) or limited (34%). Six of 29 patients (21%) achieved a complete remission, and 12 (41%) had a treatment response with \u226550% decrease in BVAS\/WG score at 6\u2003months. Fourteen patients (64%) with kidney involvement achieved remission, and in seven patients (50%), no flare was seen during the follow\u2010up period. Three patients had renal flare and were successfully re\u2010treated with RTX. Seventeen patients had disease symptoms from airways and eyes at RTX initiation, whereas only 29% displayed \u226550% treatment response. Limited clinical improvement was seen in patients with endobronchial lesions and trachea\u2010subglottic granulomatous disease. RTX is a potent therapeutic option for ANCA\u2010associated vasculitis refractory to conventional treatment. Best response may be expected in patients with vasculitic manifestations.<\/jats:p>","DOI":"10.1111\/j.1365-3083.2012.02747.x","type":"journal-article","created":{"date-parts":[[2012,7,23]],"date-time":"2012-07-23T16:37:27Z","timestamp":1343061447000},"page":"411-420","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":25,"title":["Off\u2010Trial Evaluation of the B cell\u2013Targeting Treatment in the Refractory Cases of Antineutrophil Cytoplasmic Antibodies (ANCA)\u2010Associated Vasculitis: Long\u2010Term Follow\u2010Up from a Single 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