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We summarize evidence for the role of therapeutic plasma exchange (TPE) in the treatment of anti-neutrophil cytoplasm antibody (ANCA)-associated vasculitis (AAV). TPE rapidly removes ANCA IgG, complement and coagulation factors important in the pathogenesis of AAV. TPE has been used in patients with rapidly deteriorating renal function to achieve early disease control, allowing time for immunosuppressive agents to prevent resynthesis of ANCA. The PEXIVAS trial challenged the utility of TPE in AAV, as it did not show benefit of adjunctive TPE on a combined end point of end stage kidney disease (ESKD) and death. We analyze data from PEXIVAS and other trials of TPE in AAV, an up-to-date meta-analysis, and recently published large cohort studies. There remains a role for the use of TPE in AAV in certain groups of patients, in particular those with severe renal involvement (Cr &gt;500 μmol/L or dialysis-dependent). It should be considered in patients with Cr &gt;300 μmol/L and rapidly deteriorating function, or with life-threatening pulmonary hemorrhage. A separate indication is patients double positive for anti-GBM antibodies and ANCA. TPE may have the greatest benefit as part of steroid-sparing immunosuppressive treatment strategies.

Plasma exchange as an adjunctive therapy in anti-neutrophil cytoplasm antibody-associated vasculitis