Therapeutic plasma exchange in systemic vasculitis: an update on indications and results

Therapeutic plasma exchange has been long proposed as a potentially useful modality to treat several systemic vasculitis conditions. This review summarizes the available evidence for the effectiveness of plasma exchange in systemic vasculitis.

Therapy with plasma exchange, most often combined with immunosuppressive agents, has been found effective for antiglomerular basement membrane disease, antineutrophil cytoplasmic antibody-associated vasculitis (AAV), Henoch-Schönlein purpura, cryoglobulinemic vasculitis, hepatitis B virus-associated polyarteritis nodosa and Kawasaki disease. The most common indications are life-threatening or organ function-threatening manifestations, particularly advanced renal dysfunction and disease refractory to traditional therapy. Thus, most of the available evidence favoring plasma exchange in these circumstances is from small observational studies or expert consensus. Recent advances in findings include results strengthening the notion of a small beneficial effect on preserving renal function with adjunct plasma exchange therapy in AAV with renal failure and observational data suggesting plasma exchange as a promising effective salvage option for children with Kawasaki disease not responding to standard therapy with intravenous immunoglobulins.

Evidence from case reports and case series and a few randomized controlled trials continues to support plasma exchange as a major rescue-treatment modality for several systemic vasculitis diseases. These studies offer some guidance for use of plasma exchange in systemic vasculitis, but additional data from controlled trials are needed for more accurate assessment of the indications, practical modalities, benefits and shortcomings of this treatment approach.