Plasma exchange and glucocorticoids in anti-neutrophil cytoplasm antibody associated systemic vasculitis: a randomized controlled trial. PEXIVAS

ANCA associated systemic vasculitis is a multi-system autoimmune disease, of unknown cause, which causes vital organ failure and premature death. Therapy with high dose glucocorticoids and cyclophosphamide achieves disease control but contributes to the early mortality and chronic disease damage associated with the disease. Several studies have examined whether plasma exchange improves outcomes when applied in addition to standard therapy to patients with severe presentations. A meta-analysis has concluded that although benefits on renal function have been observed a convincing effect on the composite end-point of death and end stage renal failure remains to be confirmed. Two international vasculitis networks, the European Vasculitis study group (EUVAS) and the Vasculitis Clinical Research Consortium (VCRC, USA) have combined to develop the current project which aims to recruit 500 patients with severe ANCA vasculitis, and examine whether plasma exchange reduces the frequency of death and end stage renal disease. In view of the likely contribution of high dose glucocorticoids to severe adverse events, this study will also examine, in a factorial design, a reduced dose glucocorticoid regimen in comparison to a standard dose regimen. Patients will be recruited from Australia/New Zealand, Canada, the European Union and the USA over five years and will be followed for a minimum of two years.