ComPLEX controversy: management of alveolar hemorrhage in antineutrophil cytoplasmic antibody-associated vasculitis

Despite PEXIVAS, the largest global randomized trial in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), there is ongoing debate about plasma exchange (PLEX) in diffuse alveolar hemorrhage (DAH), an uncommon but clinically challenging scenario.1,2 The Plasma Exchange and Glucocorticoids in Severe AAV (PEXIVAS) trial assessed the effect of PLEX (and glucocorticoid dosing) on the composite of death or end-stage kidney disease at 1 year among 704 participants with glomerulonephritis and/or DAH. Although it was the first trial in AAV to enroll participants with DAH requiring mechanical ventilation, it was not designed to answer the specific question of the role of PLEX in life-threatening DAH.1,3 The prespecified DAH subgroup analysis found no effect of PLEX on 1-year mortality or key shorter-term outcomes, including ventilator liberation and early mortality, but was limited by the small number, only 61 participants with severe DAH (room air oxygen saturation