Plasma exchange (PEX) can be an effective treatment in ANCA-associated vasculitis (AAV) with severe renal damage; however, it is still controversial. Among cases of newly diagnosed AAV with rapidly progressive glomerulonephritis (AAV-RPGN) at our department from 2008 onward, eleven patients who received PEX (7 cases for severe renal damage (R-PEX) and 4 cases for lung hemorrhage (L-PEX)) were retrospectively analyzed. All cases of R-PEX were dependent on hemodialysis at the beginning of PEX and all received 7 sessions of PEX (50 ml/kg or 1.3 plasma volume per exchange) within 2 weeks. All cases became dialysis-independent within 8 weeks, with 3- and 12-month cumulative renal survival rates of 100% and 80%, respectively. All cases of L-PEX retained their renal function. In rapidly developing, newly dialysis-dependent AAV-RPGN patients with normal renal function before disease onset, standard PEX can be expected to induce sufficient renal recovery to establish dialysis independence. This article is protected by copyright. All rights reserved.