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• W3119062486 abstract "Vasculitis associated with the formation of ANCA manifests in several forms defined by the extent of organ involvement (renal limited or systemic), with or without granulomatous tissue involvement, and by the presence and nature of the ANCA (antimyeloperoxidase or antiproteinase 3 and associated autoantibodies, in particular antiglomerular basement membrane [anti-GBM] autoantibodies) (1). Severe kidney disease with extensive crescentic involvement and a clinical syndrome of rapidly progressive GN is common in all forms of ANCA-associated vasculitis (AAV) (1). Diffuse alveolar hemorrhage (DAH), of varying severity, can be seen in many kinds of GN (Goodpasture syndrome) but is particularly common in AAV, with or without anti-GBM antibody (2).The therapy of AAV has greatly improved in recent years, and the use of rituximab (RTX), cyclophosphamide (CYC), or combinations of RTX and CYC plus moderate doses of steroids can effectively and safely induce a complete or partial remission in the great majority of patients (3 ⇓–5). The role of adjunctive therapy with plasmapheresis (PLEX) has been controversial, except in cases where anti-GBM autoantibody disease is found alone or concomitantly with AAV. The publication of the large Plasma Exchange and glucocorticoid dosing in treatment of anti-neutrophil cytoplasmic antibody associated vasculitis (PEXIVAS) trial in 2020 has generated much attention to this controversy (6).This debate focuses on this controversy by laying out a precise question: should PLEX be used for severe AKI and/or pulmonary hemorrhage in patients with AAV? For this debate, I will interpret “severe” as meaning the need for artificial organ support (dialysis for AKI or mechanical ventilation for DAH). I will also interpret ANCA vasculitis as meaning a positive (antimyeloperoxidase or antiproteinase 3 autoantibody in the absence of anti-GBM antibody and clinical or morphologic evidence of a vasculitis involving the kidney without or with extrarenal involvement, including DAH. …" @default.
• W3119062486 created "2021-01-18" @default.
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• W3119062486 date "2021-05-01" @default.
• W3119062486 modified "2023-09-25" @default.
• W3119062486 title "Should PLEX Be Used for Severe AKI and/or Pulmonary Hemorrhage in ANCA-Associated Vasculitis (AAV)? CON" @default.
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• W3119062486 doi "https://doi.org/10.34067/kid.0004102020" @default.
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