Role of plasma exchange therapy in the treatment of ANCA vasculitis
What are the indications for the use of plasma exchange therapy in the treatment of ANCA vasculitis?
Plasma exchange or plasmapheresis is thought to rapidly clear ANCAs, coagulation factors, and inflammatory cytokines that may be pathogenic in vasculitis.
At present, plasma exchange is recommended in addition to corticosteroids and cyclophosphamide for patients presenting with severe kidney failure in the setting of rapidly progressive glomerulonephritis and/or those presenting with severe pulmonary hemorrhage.
The role of plasmapheresis as adjunctive therapy is not established for patients with less severe disease. The use of plasmapheresis in ANCA vasculitis is the focus of a large ongoing clinical trial (Clinicaltrial.gov PEXIVAS).
Those patients also presenting with concomitant anti-GBM disease (identified with kidney biopsy demonstrating linear IgM staining of glomerular basement membrane) should receive early initiation of plasma exchange.