Abstract: FR-PO1164
Unsuspected Recurrent FSGS and Complement Dysregulation in a Kidney Transplant Recipient with ADPKD
Session Information
- Transplantation: Clinical - Transplant Access, Recipient Evaluation, Living Donors, Pregnancy, and More
October 23, 2026 | Location: Exhibit Hall A, Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Transplantation
- 2002 Transplantation: Clinical
Authors
- Yang, Xiaokai, Center for Kidney Diseases, The Second Affiliated Hospital of Nanjing Medical University, Nanjing, Jiangsu, China
- Liu, Lilin, Center for Kidney Diseases, The Second Affiliated Hospital of Nanjing Medical University, Nanjing, Jiangsu, China
- Fang, Yi, Center for Kidney Diseases, The Second Affiliated Hospital of Nanjing Medical University, Nanjing, Jiangsu, China
- Gu, Min, Center for Kidney Diseases, The Second Affiliated Hospital of Nanjing Medical University, Nanjing, Jiangsu, China
- Dai, Chunsun, Center for Kidney Diseases, The Second Affiliated Hospital of Nanjing Medical University, Nanjing, Jiangsu, China
- Cao, Hongdi, Center for Kidney Diseases, The Second Affiliated Hospital of Nanjing Medical University, Nanjing, Jiangsu, China
Introduction
Focal segmental glomerulosclerosis (FSGS) recurrence is rarely suspected in autosomal dominant polycystic kidney disease (ADPKD) recipients. We report a case of post-transplant FSGS in an ADPKD patient, diagnosed through serial pathology and serology, with superimposed complement dysregulation.
Case Description
A 49-year-old woman with ADPKD underwent kidney transplantation (June 2023). Early graft function was stable; a 3-month protocol biopsy showed no FSGS. After unilateral native nephrectomy (September 2024), progressive proteinuria developed (August 2025). Indication biopsy revealed FSGS with podocyte injury. Genetic analysis identified a LAMA5 variant, increasing podocyte vulnerability. Native nephrectomy review showed segmental sclerosis without immune deposits, favoring primary FSGS. Serology revealed elevated pre- and post-transplant suPAR (3571→3897 pg/mL) and positive anti-nephrin antibody. Steroid pulse provided only transient improvement. Subsequent graft dysfunction with thrombotic microangiopathy-like features (anemia, thrombocytopenia, schistocytes) and elevated soluble C5b-9 indicated complement dysregulation. Eculizumab reduced C5b-9 but proteinuria persisted (peak 9.57 g/day; Cr 194 µmol/L). Rituximab plus double-filtration plasmapheresis partially reduced proteinuria. A repeat biopsy (February 2026) showed FSGS with progressive interstitial fibrosis/tubular atrophy. By April 2026, creatinine improved to 121 µmol/L, proteinuria decreased to 2.17 g/day.
Discussion
Post-transplant FSGS in ADPKD should not be routinely dismissed as de novo or secondary. The absence of FSGS on early biopsy followed by late podocyte injury supports recurrence. Elevated suPAR and anti-nephrin antibodies confirmed the diagnosis. Superimposed complement dysregulation likely accelerated allograft damage. Comprehensive serologic and pathologic workup is essential to identify occult primary FSGS in ADPKD recipients.