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This case explores the efficacy of pre-emptive plasma exchange in a patient with diffuse alveolar hemorrhage from ANCA-associated vasculitis.

May 20, 2026

C41-04 Flexing the PLEX: Pre-emptive Plasma Exchange for Diffuse Alveolar Hemorrhage From ANCA-associated Vasculitis and the Aftermath

Key Points

This case explores the efficacy of pre-emptive plasma exchange in a patient with diffuse alveolar hemorrhage from ANCA-associated vasculitis.
80-year-old female with chronic kidney disease treated with plasma exchange prior to ANCA diagnosis
5 sessions of plasmapheresis and pulse dose steroids administered due to hypotension
Follow-up included pulmonary function tests and imaging assessments.
Improvement in urine output, AKI, and PaO2/FiO2 ratio during treatment
Near complete resolution of ground-glass opacities on CT Chest after treatment
Decreased diffusion lung capacity normalized at 6-month follow-up.

Abstract

Abstract Introduction Diffuse alveolar hemorrhage (DAH) has high mortality rates. We report a case of a patient with acute kidney injury (AKI) and DAH requiring mechanical ventilation, who was treated with plasma exchange (PLEX) before a diagnosis of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) was confirmed. Description An 80-year-old female with chronic kidney disease presented with 2 weeks of dyspnea and hemoptysis. Hematuria, proteinuria, worsening AKI, and diffuse bilateral ground-glass opacities (GGOs) on imaging pointed towards a pulmonary-renal syndrome (PRS). Bronchoscopy with serial bronchoalveolar lavage (BAL) confirmed DAH. Pulse dose steroids were initiated with alternate day plasmapheresis for a total of 5 sessions in view of the hypotension. 50% fresh frozen plasma was used as replacement fluid due to decreased fibrinogen levels. The urine output, AKI, PaO2/FiO2 ratio, and chest X-ray improved over the next few days. She was discharged on 2L/min oxygen (O2). Workup demonstrated antibodies to neutrophil enzyme myeloperoxidase (anti-MPO). Kidney biopsy showed pauci-immune necrotizing glomerulonephritis with early crescent formations. After discharge, she was re-admitted multiple times due to immunosuppression-associated complications (reactivation of shingles, Pseudomonas pneumonia, Cryptococcus bacteremia, and Clostridium difficile infection). Therapy was switched to rituximab. Follow-up at 3 months demonstrated a decreased diffusion lung capacity of carbon monoxide (DLCO) on pulmonary function test (PFT), and improved GGOs with near complete resolution in CT Chest. DLCO normalized on the 6-month follow-up. Patient was weaned off O2 supplementation and reported improved exercise tolerance. Discussion This case highlights the importance of high clinical suspicion of PRS with early treatment and consideration of rescue therapies to improve clinical outcomes in severe cases. PLEX has a conditional recommendation as adjuvant in conjunction with glucocorticoids and either cyclophosphamide or rituximab in patients with DAH and hypoxemia. The major PEXIVAS trial did not show treatment benefits of PLEX for DAH, however, subgroup analysis suggested a possible trend towards benefit in severe cases. Observational and retrospective cohort studies report rapid improvement in respiratory function and reduced short-term mortality with PLEX in critically ill patients, but findings are not definitive. To date, there are no trials that specifically evaluate response to PLEX in terms of DAH resolution, ANCA level and PFTs trends. Given the beneficial effect of removing ANCA antibodies, the promising results of PLEX as a life-saving measure even in a small subset of patients should be considered. Risks of PLEX include hypotension, severe infections, anaphylactoid reactions, depletion coagulopathy, bleeding, hypocalcemia, and increased drug clearance. This abstract is funded by: None

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