Abstract: PO1903
Concurrent Anti-Glomerular Basement Membrane Nephritis and Membranous Nephropathy
Session Information
- Glomerular Diseases: Clinical, Outcomes, and Trials - 2
October 22, 2020 | Location: On-Demand
Abstract Time: 10:00 AM - 12:00 PM
Category: Glomerular Diseases
- 1203 Glomerular Diseases: Clinical, Outcomes, and Trials
Authors
- Ahmad, Syeda B., Columbia University Irving Medical Center, New York, New York, United States
- Santoriello, Dominick, Columbia University Irving Medical Center, New York, New York, United States
- Canetta, Pietro A., Columbia University Irving Medical Center, New York, New York, United States
- Bomback, Andrew S., Columbia University Irving Medical Center, New York, New York, United States
- D'Agati, Vivette D., Columbia University Irving Medical Center, New York, New York, United States
- Markowitz, Glen S., Columbia University Irving Medical Center, New York, New York, United States
- Ahn, Wooin, Columbia University Irving Medical Center, New York, New York, United States
- Radhakrishnan, Jai, Columbia University Irving Medical Center, New York, New York, United States
- Appel, Gerald B., Columbia University Irving Medical Center, New York, New York, United States
Background
Anti-GBM nephritis is an uncommon entity with a rapidly progressive course. The concurrence of anti-GBM nephritis with membranous nephropathy (MN) is rare and poorly understood. We report a single center case series of this dual glomerulopathy with emphasis on presenting features, course, and outcome.
Methods
A total of 12 cases of combined anti-GBM nephritis and MN were identified from the archives of the Columbia Renal Pathology Laboratory over the past 18 years. Presenting clinical, histopathologic and laboratory data with follow up were obtained by chart review.
Results
The cohort of 12 cases included 7 men and 5 women with age range 18-81 years. The most common presenting feature was AKI (mean creatinine 9.3 mg/dL) , with one patient having pulmonary symptoms. Positive anti-GBM serology was available at presentation for 11 cases, 5 with titers > 100 au/mL, and all were ANCA negative. Of those tested the majority were PLA2r negative. Patients were predominantly Caucasian (N = 9). All patients required hemodialysis (HD) at presentation, and two patients, a 20-year-old woman and an 81-year-old woman had renal recovery with the later having a stable creatinine of 2.0 mg/dL 11 months later. Treatment regimens included the following: cyclophosphamide, plasmapheresis, and prednisone (N=9); cyclophosphamide and prednisone (N=1), prednisone and plasmapheresis (N=1) and rituximab alone (N=1). Two patients died, both on HD, one 16 years later from unknown cause and one 3 months after presentation from sepsis . Analysis of the 12 renal biopsies showed combined linear and granular staining of GBMs for IgG, with crescents involving 23-100% of glomeruli, and fibrinoid necrosis involving 15-100%. The two patients who recovered renal function had fewer total crescents (82% crescents, and 23%, respectively) and less fibrinoid necrosis (15% and 31%) compared to the subgroup without recovery respectively, on kidney biopsy.
Conclusion
Combined anti-GBM and MN is a rare entity presenting with severe AKI requiring dialysis. Renal recovery is uncommon. High percentage of crescents are consistent with poor outcomes. Treatment and course are dominated by anti-GBM nephritis. The MN component is predominantly PLA2R negative, and further studies into pathogenesis are needed.