Abstract: FR-PO711
Anti-Glomerular Basement Membrane Disease Overlap with Pauci-Immune Glomerulonephritis: Temporal Concurrence at a Single Center
Session Information
- Glomerular Diseases: From Inflammation to Fibrosis - II
November 03, 2023 | Location: Exhibit Hall, Pennsylvania Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Glomerular Diseases
- 1401 Glomerular Diseases: From Inflammation to Fibrosis
Authors
- Fechter, Buckley, Medical College of Wisconsin, Milwaukee, Wisconsin, United States
- Batool, Aisha, Medical College of Wisconsin, Milwaukee, Wisconsin, United States
- Gallan, Alexander James, Medical College of Wisconsin, Milwaukee, Wisconsin, United States
- Chaudhry, Shahzad, Medical College of Wisconsin, Milwaukee, Wisconsin, United States
- Mariyam Joy, Christina, Medical College of Wisconsin, Milwaukee, Wisconsin, United States
Introduction
Anti-GBM and pauci-immune vasculitis are small vessel vasculitis and cause RPGN with diffuse alveolar hemorrhage (DAH). Overlap between these entities is rare. Upto 38% of the individuals with anti-GBM disease demonstrate double-positivity and approximately 10% of patients with ANCA vasculitis have positive titers of anti-GBM antibodies. We present two double-positive cases diagnosed within 6 weeks at our institution.
Case Description
Case 1:A 74-years-old white male with h.o SARS-COV19 two months ago presented with cough and metallic taste, BUN 60 with S/Cr 5.05 mg/dl, p-ANCA 1:640 and anti-GBM+. Kidney biopsy showing Diffuse crescentic GN with cellular crescents involve 50% of the glomeruli,IF linear IgG staining. Patient was started on PLEX, IV Methylprednisolone and Cyclophosphamide. Patient remains HD dependent.
Case 2:A 71-years-old white male with recently diagnosed lung biopsy proven GPA 4 months ago. Patient was admitted with SARS-COV-19 infection, serum creatinine 6.42mg/dl (baseline 0.9mg/dl few months ago). Kidney biopsy showing Crescentic glomerulonephritis with overlapping anti-glomerular basement membrane disease and pauci-immune glomerulonephritis. Patient was started on iv methylprednisolone, PLEX and cyclophosphamide. Patient needed hemodialysis for 2.5 months and has come off with most recent s/cr 3.54 mg/dl.
Discussion
In anti-GBM disease DAH can be the predominant presenting feature and will prompt early diagnosis necessitating early intervention. Hallmark for diagnosis is linear IgG staining of GBM on immunofluorescence. ANCA-associated vasculitis, in contrast, is pauci-immune with few or no immune deposits on IF. Given rarity of the overlap disease and paucity of literature standard treatment strategy is not established. Approach remains similar to treatment of anti GBM disease with PLEX and induction immunosuppression with cyclophosphamide. However longer and closer follow-up is required for double-positive disease due to more frequent relapses.