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Abstract: SA-PO862

A Rare Presentation of Recurrent c-ANCA Glomerulonephritis 10 Years Post-Kidney Transplant

Session Information

Category: Transplantation

  • 2002 Transplantation: Clinical

Authors

  • Masry, Ahmad Al, University of Iowa Hospitals and Clinics, Department of Internal Medicine, Division of Nephrology, Iowa, Iowa, United States
  • Rastogi, Prerna, University of Iowa Hospitals and Clinics, Department of Pathology, Iowa, Iowa, United States
  • Sanders, M. Lee, University of Iowa Hospitals and Clinics, Department of Internal Medicine, Division of Nephrology, Iowa, Iowa, United States
Introduction

Pauci-immune glomerulonephritis (GN) is the most common type of crescentic rapidly progressive GN. While kidney transplantation remains the optimal treatment for those patients who progress to end stage kidney disease (ESKD), patients remain at risk for disease recurrence even in the modern era of immunosuppression. Most anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis cases reoccur with a median time of 30 months post-transplant. We report a rare case presentation of a first recurrence of crescentic pauci-immune GN ten years following kidney transplantation.

Case Description

A 74-year-old female with a history of living related kidney transplant ten years ago was found to have blood and protein on her yearly urinalysis specimen. Her post-transplant course up to that point had been unremarkable with no rejection episodes and a baseline serum creatinine (sCr) range maintained between 0.9-1.1 mg/dL. Her chronic immunosuppression regimen consisted of tacrolimus and mycophenolate. While her sCr was within her baseline range, her urine protein to creatine ratio was 3.0 compared to 0.2 the year prior. No history of diabetes, no rashes were present, and she had no complaints. Additional work-up showed no monoclonal spike on serum protein electrophoresis, no donor specific antibodies, and a donor derived cell free DNA value of 0.16%. Of note, the original cause of her kidney failure was officially listed as lupus nephritis; therefore, an immunologic work-up was performed which revealed a positive ANCA with MPO titer of 1:320. A subsequent renal biopsy revealed pauci-immune necrotizing and crescentic GN. Treatment with rituximab 1000 mg for two doses 14 days apart and high dose oral prednisone was initiated. We were able to obtain the native kidney biopsy for review which showed a crescentic GN with negative immunofluorescence making pauci-immune GN the more likely cause of her original ESKD.

Discussion

New onset hematuria and proteinuria in a kidney transplant recipient warrants additional work-up. In this case, we surprisingly discovered a crescentic GN in the setting of stable renal function. This likely recurrence of primary disease ten years post-transplant is also well beyond the typical median time for recurrence. Our next clinical step will be to perform a repeat biopsy after treatment completion to ensure disease resolution.