Theraperutic plasma exchange for systemic vasculitis

Abstract

Plasma exchange therapy has been found to be effective for anti-glomerular basement membrane (GBM) disease, antineutrophil cytoplasmic antibody-associated vasculitis (AAV), IgA vasculitis, and cryoglobulinemic vasculitis. The most common indications are life-threatening or organ function-threatening manifestations. For AAV, plasma exchange (PE) therapy is recommended for patients with either severe renal impairment or diffuse alveolar hemorrhage. The largest trial published to date is the MEPEX (Methylprednisolone versus Plasma Exchange) trial, which recruited individuals who had a serum creatinine level of ＞5.66mg/dL. PE therapy appeared to be of value in preventing end-stage renal disease at 12 months, but long-term follow-up revealed no statistically significant benefit for the PE group. The PEXIVAS (Plasma Exchange and Glucocorticoids for Treatment of ANCA-Associated Vasculitis) trial is a global factorial randomized controlled trial. Eligible patients had a severe manifestation defined as either renal involvement, with a documented estimated glomerular filtration rate of ＜50mL/min, or lung hemorrhage. In the Japanese insurance system, administration of plasma exchange therapy is allowed only for patients with rapidly progressive glomerulonephritis caused by anti-GBM disease. Two courses of PE, composed of seven times in 2 weeks, are permitted. Concerning IgA vasculitis and cryoglobulinemic vasculitis, no global guidelines have been established and no randomized controlled trial has been conducted to evaluate the efficacy of PE therapy. Case series and reports support the efficacy of PE therapy for refractory or severe cases.