Abstract
Therapeutic plasma exchange (TPE) is an adjunctive intervention to immunosuppression for the treatment of severe renal involvement or lung hemorrhage in patients with ANCA-associated vasculitis (AAV). Patients with AAV have an increased risk for progression to end-stage kidney disease (ESKD) or death despite advances in immunosuppressive therapy. The potential pathogenicity of ANCA makes TPE a reasonable treatment approach for the life-threatening complications of AAV. The efficacy of intensive TPE in rapidly progressive glomerulonephritis was originally described in small studies almost four decades ago. Further randomized trials examined the addition of TPE to standard of care, exhibiting mixed results in both patient and renal survival. The largest clinical trial to date, PEXIVAS, failed to demonstrate a clear benefit for TPE in severe AAV. In light of new evidence, the role of TPE remains controversial across the vasculitis medical community. The purpose of this review is to summarize the clinical indications and the current available data for the use of TPE in patients with severe AAV.
Highlights
IntroductionTherapeutic plasma exchange (TPE) is an extracorporeal procedure in which plasma is separated from other blood constituents and subsequently removed from the patient in replacement of fresh frozen plasma or albumin solutions
2nd Department of Internal Medicine, National and Kapodistrian University of Athens Medical School, Abstract: Therapeutic plasma exchange (TPE) is an adjunctive intervention to immunosuppression for the treatment of severe renal involvement or lung hemorrhage in patients with Antineutrophilic cytoplasmic antibodies (ANCA)-associated vasculitis (AAV)
On account of the PEXIVAS trial results, American Society for Apheresis (ASFA) updated its guidelines on the use of TPE in AAV by changing the category recommendation for rapidly progressive glomerulonephritis from I to II, downgrading apheresis as a second-line therapy for this disorder, and by lowering the grade of evidence from 1A to 1B, i.e., a strong recommendation with moderate quality evidence [37]
Summary
Therapeutic plasma exchange (TPE) is an extracorporeal procedure in which plasma is separated from other blood constituents and subsequently removed from the patient in replacement of fresh frozen plasma or albumin solutions. The fundamental rationale for TPE lies in the removal of a circulating pathogenic factor, namely an antibody, an immune complex, or a monoclonal protein. This therapeutic potential has led to the use of TPE in a number of autoimmune neurologic, hematologic, and renal disorders [1,2,3,4,5]. Anaphylactic reactions, there is an increased need for concrete evidence regarding the use of TPE in AAV [19,20] This narrative review focuses on (i) the pathogenetic rationale, (ii) the current clinical indications, and (iii) the overview of clinical studies of TPE in AAV, as well as (iv) the PEXIVAS trial and (v) its impact on the future of TPE in severe AAV
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