In Selected Patients with Severe ANCA-Associated Vasculitis (AAV) and Diffuse Alveolar Hemorrhage (DAH), Plasma Exchange (PLEX) May Provide Benefit in Addition to Glucocorticoid: A Challenging Case

Introduction: AAV encompasses a group of diseases characterized by inflammation of small- to medium-sized blood vessels, including granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA).[1] The incidence and prevalence of AAV have been increasing over the past few decades. The global pooled incidence is approximately 17.2 per million person-years, with a prevalence of 198 per million persons.[2] Kidney involvement in AAV is common, with more than 75% of patients presenting with rapidly progressive glomerulonephritis, which can lead to end-stage kidney disease (ESKD).[3] The pathogenesis involves the formation of pauci-immune necrotizing glomerulonephritis, often associated with either proteinase 3 (PR3)-ANCA or myeloperoxidase (MPO)-ANCA.[3] DAH is a severe and potentially life-threatening manifestation of AAV.[4] Case Description: 74-year-old male with history of CKD stage III/IV,AAV,DAH, biopsy-proven ANCA associated GN, who was managed with rituximab and pulse dose steroids which was transitioned to prednisone. Shortly after starting the treatment, the patient presented to the ER and was admitted for acute hypoxic respiratory failure. Patient was managed with PLEX that showed significant improvement. Lab:ANA 1:1280,dsDNA 5,pANCA + >1:640 with positive MPO >800 and PR3 of 14.1, with negative SCL-70,Sm,SM/RNP,Ribosomal P Ab,SSA,SSB CT Chest: Upper predominant lung opacities compatible with DAH, confirmed with bronchoscopy Discussion: PLEX has been evaluated as an adjunctive therapy in AAV with DAH. The PEXIVAS trial found no significant benefit of PLEX in reducing mortality or progression to ESKD, even among patients with DAH[5,6]. However, in selected cases, particularly those with pulmonar involvement, PLEX may offer additional clinical benefit beyond standard high-dose corticosteroid therapy. In our patient, DAH was refractory to initial high-dose steroids but showed marked improvement following initiation of PLEX. This case supports consideration of early PLEX use in patients with AAV and DAH, as it may contribute to improved outcomes in appropriately selected individuals