Is There a Role for Plasma Exchange in ANCA-Associated Vasculitis?

Abstract

Purpose of review This review summarises the evidence for the use of therapeutic plasma exchange (TPE) in anti-neutrophil cytoplasm antibody (ANCA)–associated vasculitis. TPE is an extra-corporeal treatment which efficiently removes IgG and other pathogenic small molecules from the blood. There are several mechanistic reasons why this should be of benefit in AAV including the well-described pathogenicity of ANCA. Recent findings The recently published PEXIVAS trial is the largest study of TPE in AAV to date. It did not show a benefit for adjunctive TPE on a primary end point of ESRD or death. There was no difference in serious adverse events between those treated with TPE and those treated with immunosuppressive drugs alone. Conclusions Based on the results of PEXIVAS, most patients with AAV should not be treated with adjunctive TPE. However, there are subgroups of patients with AAV for whom TPE may still be of benefit, including those with double positivity for anti-GBM antibodies and ANCA.

Unraveling the Immunopathogenesis and Genetic Variants in Vasculitis Toward Development of Personalized Medicine

“…Plasma exchange may benefit patients by removing pathogenic ANCAs in the plasma, as well as clotting factors involved in the coagulation cascade ( 85 , 86 ). However, the PEXIVAS trial, the largest study of plasma exchange in AAV, did not show that adjunctive plasma exchange benefits patients with severe AAV ( 87 , 88 ). Nevertheless, it was suggested that patients with both ANCA and anti-glomerular basement membrane (GBM) antibodies should still be treated with adjunctive plasma exchange.…”

“…Nevertheless, it was suggested that patients with both ANCA and anti-glomerular basement membrane (GBM) antibodies should still be treated with adjunctive plasma exchange. Treatments for patients with anti-GBM disease involve immunosuppression and adjunctive plasma exchange ( 87 ). Since both patients with single anti-GBM positivity and double positivity for anti-GBM antibodies and ANCA experience aggressive pulmonary and renal disease, plasma exchange seems appropriate for treating double positivity patients ( 87 ).…”

“…Treatments for patients with anti-GBM disease involve immunosuppression and adjunctive plasma exchange ( 87 ). Since both patients with single anti-GBM positivity and double positivity for anti-GBM antibodies and ANCA experience aggressive pulmonary and renal disease, plasma exchange seems appropriate for treating double positivity patients ( 87 ).…”

Plasma Exchange in ANCA-Associated Vasculitis: A Narrative Review

“…The results of the PEXIVAS trial created controversy across the medical community regarding the role of TPE in severe AAV [ 48 , 49 , 50 , 51 , 52 , 53 ]. The study included the largest patient population to date with a multinational enrollment, which allows for a broad generalizability of the results.…”