Objective: Immunosuppressive therapy in anti-neutrophil cytoplasmic antibody-associated vasculitis is indispensable for patient and kidney survival. There is a controversy about whether the risks of plasma exchange treatment override the probability of kidney-related outcomes. Hence, the question arises in which conditions the plasma exchange will be required? In this study, we aimed to evaluate the effect of plasma exchange adding to immunosuppressive therapy in antineutrophil cytoplasmic antibody-associated vasculitis patients. Methods: We retrospectively analyzed 57 patients with biopsy-proven anti-neutrophil cytoplasmic antibody-associated vasculitis. We grouped patients according to treatment options with or without plasma exchange. We investigated the 1-year and 5-year patients and kidney outcomes. Results: Thirty-six (63.2%) of 57 patients were treated with plasma exchange besides the routine immunosuppressive treatment. Sixteen (44.5%) of 36 patients were with active pulmonary hemorrhage and the remaining 20 (55.5%) were with vasculitic pulmonary involvement. The survival rate was 80.7% and 68.8% in the first and fifth year, respectively. In the multivariate Cox regression analysis model, risk factors affecting patient survival were age >50 years (hazard ratio = 17.11 P = .034), pulmonary involvement (hazard ratio = 13.25, P =.02), positive perinuclear anti-neutrophil cytoplasmic antibody-associated vasculitis (hazard ratio = 5.93, P =.036), and lower albumin level (hazard ratio = 0.18, P =.014). It is found that C-reactive protein level and plasma exchange did not relate to better patient and kidney outcomes (P > .05). Conclusions: In anti-neutrophil cytoplasmic antibody-associated vasculitis, although pulmonary hemorrhage and pulmonary involvement are serious complications, plasma exchange did not provide additional benefit to standard treatment.