Abstract: FR-PO0742
Rescuing Kidney Function in Anti-GBM Disease Despite Advanced Chronicity: A Treatment Paradox
Session Information
- Glomerular Diseases: ANCA Vasculitis, Anti-GBM Disease, and Crescentic GN
October 23, 2026 | Location: Exhibit Hall A, Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Glomerular Diseases
- 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics
Authors
- Pham, Steven, Olive View – UCLA Medical Center, Sylmar, California, United States
- Kamarzarian, Anita, Olive View – UCLA Medical Center, Sylmar, California, United States
- Gunasekaran, Vidhya, Olive View – UCLA Medical Center, Sylmar, California, United States
- Jafari, Golriz, Olive View – UCLA Medical Center, Sylmar, California, United States
- Pham, Phuong-Chi T., Olive View – UCLA Medical Center, Sylmar, California, United States
- Hou, Jean, Cedars-Sinai, Los Angeles, California, United States
Group or Team Name
- Olive View – UCLA Medical Center
Introduction
Anti-GBM disease is a rare autoimmune glomerulonephritis caused by antibodies against type IV collagen. It typically is rapidly progressive and causes severe renal injury, when biopsy demonstrates advanced chronicity. Conventional teaching discourages aggressive immunosuppression (IST) when extensive global sclerosis is present. We report a case that challenges this paradigm, highlighting the importance of clinical acuity over histologic chronicity alone.
Case Description
A 41-year-old previously healthy woman presented with acute kidney injury (creatinine 3.5 mg/dL), proteinuria (UPC 1.9), hematuria (26–50 RBC/hpf), and positive anti-GBM IgG (3.8 AI). Kidney biopsy revealed severe chronicity with global glomerulosclerosis in all sampled glomeruli and predominantly fibrous crescents, suggesting inactive disease. However, her acute presentation and elevated antibody titers raised concern for ongoing immunologic activity and potential sampling limitations.
Given this discordance, aggressive IST was pursued. She underwent daily plasmapheresis (PLEX) for five sessions, then cyclophosphamide (CYC) and prednisone taper. This resulted in rapid clinical and serologic improvement, with anti-GBM titers decreasing to 1.7 AI after PLEX and undetectable (<0.1 AI) by two months. She received only two doses of CYC. Creatinine stabilized (1.8 mg/dL) with improved proteinuria (UPC 0.6) and resolved hematuria. She does not require dialysis with stable kidney function at 2-year follow-up.
Discussion
Histologic chronicity may not fully reflect active injury. Despite biopsy features suggesting irreversible damage, early IST and PLEX led to renal recovery. These findings support an individualized treatment approach integrating clinical trajectory, serologic activity, and timing of presentation rather than relying solely on histopathologic severity. In selected patients with discordant findings, a trial of intensive therapy may preserve kidney function and avoid dialysis.