Role of therapeutic plasma exchanges in systemic vasculitis.

Therapeutic management of systemic vasculitides is based on glucocorticoids (GCs), in combination or not with immunosuppressive and/or immunomodulatory agents. Plasma exchange (PLEX) has been used in some vasculitides, with various levels of evidence. Indeed, it is part of the first-line therapy in patients with anti-glomerular basement membrane (GBM) antibodies and in patients with severe hepatitis B related polyarteritis nodosa (PAN). PLEX might also be considered in some selected patients with ANCA-associated vasculitis and cryoglobulinemia vasculitis. Studies support the use of PLEX as second line therapy in refractory Kawasaki disease. Finally, there is not robust data to support the use of PLEX in unselected patients with non-HBV-related PAN or IgA vasculitis. Additional studies are required to address its role in these settings.