Twenty-Four Cases of Severe ANCA-Associated Vasculitis Treated With Plasma Exchange: A Clinical Analysis and...

Advances in the understanding of anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) pathophysiology have expanded the application of plasma exchange (PE) in severe cases; however, robust evidence on its efficacy, safety, and long-term outcomes remains scarce. This retrospective study evaluated the short-term efficacy, safety, and long-term prognosis of PE in patients with severe AAV. A total of 24 patients receiving PE alongside standard induction therapy (glucocorticoids plus cyclophosphamide or rituximab) were analyzed. Clinical and laboratory parameters-including ANCA titers, Birmingham Vasculitis Activity Score (BVAS), C-reactive protein (CRP), and hemoglobin (Hb)-were assessed before and after treatment, together with adverse events and survival. The cohort comprised 10 males and 14 females, mean age 62.5 ± 16.1 years (range 29-84). PE was associated with significant reductions in ANCA levels, BVAS, and CRP, alongside increased Hb. Over long-term follow-up, mortality rose progressively, mainly attributable to severe infections, respiratory failure, and heart failure. In conclusion, PE exhibits short-term effectiveness and a tolerable safety profile in severe AAV, yet long-term survival outcomes warrant further attention.