What is the role of plasma exchange in the treatment of granulomatosis with polyangiitis (GPA)?

Updated: Aug 31, 2021
  • Author: Christopher L Tracy, MD; Chief Editor: Herbert S Diamond, MD  more...
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Plasma exchange may be considered in patients with rapidly progressive renal disease (serum creatinine level >5.65mg/dL) in order to preserve renal function. [6] Additionally, plasma exchange, along with aggressive immunotherapy, may be helpful in DAH. [74] Plasma exchange is used with daily oral cyclophosphamide and glucocorticoids, usually pulse methylprednisolone. Plasma exchange has not been shown to improve overall survival rates or relapse rates but has been associated with improved long-term survival, free of hemodialysis. [75, 76]

Jayne et al reported improved renal outcomes in adults with GPA or microscopic polyangiitis with severe renal failure (creatinine >5.8 mg/dL) who were treated with plasma exchange, when compared with intravenous methylprednisolone. [76] The patients in this multicenter European trial were also treated with oral prednisone and oral cyclophosphamide at the time of enrollment.

The proposed mechanism of action of plasma exchange in AAV includes removal of pathologic circulating factors (eg, ANCA, activated lymphocytes), removal of excess physiologic factors (eg, complement, coagulation factors, cytokines/chemokines), replacement of deficient plasma factors, and other, less well-defined mechanisms. Potential adverse events associated with plasma exchange include electrolyte disturbances, anaphylaxis, hemorrhage, and transfusion-related lung injury. [77]

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