Abstract: PUB161
Dual Myeloperoxidase (MPO)/PR3-Positive ANCA-Associated Vasculitis with Hypocomplementemia and Epstein-Barr Virus Viremia Presenting as Fulminant Pulmonary-Renal Syndrome
Session Information
Category: Glomerular Diseases
- 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics
Authors
- Abro, Sheeraz, The University of Mississippi Medical Center, Jackson, Mississippi, United States
- Abro, Paras A., Liaquat University of Medical & Health Sciences, Jamshoro, Sindh, Pakistan
- Velagapudi, Ramya Krishna, The University of Mississippi Medical Center, Jackson, Mississippi, United States
Introduction
ANCA associated vasculitis (AAV) is a pauci-immune small-vessel vasculitis typically associated with either MPO-ANCA or PR3-ANCA seropositivity. Concurrent MPO and PR3 positivity is uncommon and has been associated with atypical or severe disease phenotypes. Hypocomplementemia may indicate additional immune dysregulation. Management becomes particularly challenging in the setting of active viral replication.
Case Description
A 31-year-old man with systemic lupus erythematosus and hypertension presented with massive hemoptysis and rapidly progressive kidney failure. He required mechanical ventilation for diffuse alveolar hemorrhage and emergent hemodialysis for severe hyperkalemia (K 8.0 mmol/L). Serum creatinine increased from a baseline of 1.0 mg/dL to 4.8 mg/dL with nephritic range proteinuria (UPCR 3.5 g/g) and active urinary sediment.
Serologic evaluation demonstrated dual MPO/PR3 ANCA positivity and positive ANA. Infectious workup revealed Epstein-Barr virus (EBV) viremia with 19,700 copies/mL. Kidney biopsy showed pauci-immune necrotizing crescentic glomerulonephritis with cellular crescents in 8 of 13 glomeruli and minimal immune complex deposition.
Induction therapy included pulse glucocorticoids, plasma exchange, and cyclophosphamide. Rituximab was deferred because of concern for worsening EBV viremia and risk of lymphoproliferative complications. Avacopan was added as a steroid-sparing adjunct. Subsequently, he was dialyssi independent with partial renal recovery.
Discussion
This case highlights fulminant pulmonary-renal syndrome associated with dual-positive AAV and hypocomplementemia, suggesting broader immune dysregulation beyond classic pauci-immune disease. Active EBV viremia significantly influenced immunosuppressive strategy by limiting use of B-cell depletion therapy. Cyclophosphamide provided effective induction while potentially reducing infectious risk compared with rituximab. Adjunctive avacopan facilitated glucocorticoid reduction in a critically ill patient.