Role of plasma exchange in the treatment of primary vasculitides

Abstract

Plasma exchange (PLEX) has been part of the therapeutic armamentarium for vasculitis for over 40 years and this article reviews the indications, strength of evidence and safety of PLEX in the vasculitides. The relative rarity of these autoimmune diseases and incomplete understanding of their pathogenesis coupled with a lack of the development of a robust scientific rationale for PLEX therapy has hampered the conduct of large controlled trials. The use of PLEX has changed in recent years to reflect the improved evidence from controlled trials and PLEX is currently recommended for primary vasculitides such as ANCA-associated vasculitis with severe renal failure and cryoglobulinemia, whilst there is less evidence to guide its use in Henoch–Schonlein purpura and polyarteritis nodosa (PAN). PLEX remains a nonselective, costly therapy with common adverse events and uncertainty remains over the optimal method of application, frequency, dose, replacement solution, monitoring and integration of PLEX with other the...