Abstract: FR-PO767
When Rare Things Return: A Case of Late, Indolent, Post-Transplant Recurrent Collagenofibrotic Glomerulopathy
Session Information
- Post-Transplantation and Case Reports
November 03, 2023 | Location: Exhibit Hall, Pennsylvania Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Transplantation
- 2102 Transplantation: Clinical
Authors
- Sood, Divya, University of Nebraska Medical Center, Omaha, Nebraska, United States
- Foster, Kirk W., University of Nebraska Medical Center, Omaha, Nebraska, United States
- Westphal, Scott G., University of Nebraska Medical Center, Omaha, Nebraska, United States
Introduction
Collagenofibrotic glomerulopathy (CG), also known as Collagen Type III glomerulopathy, is an extremely rare disorder caused by abnormal glomerular deposition of type III collagen. Etiology and prognosis are poorly understood, and its implication in kidney transplantation is unclear.
Case Description
A 44-year-old female with a history of diabetes mellitus developed kidney dysfunction and nephrotic range proteinuria. A biopsy revealed mesangial expansion and nodularity suggestive of diabetic glomerulopathy, however, electron microscopy revealed extensive subendothelial deposition of type III collagen fibrils, consistent with CG. She progressed to ESKD and received a living donor kidney transplant. She experienced a straightforward transplant course with excellent graft function for more than a decade. 12 years post-transplant, she developed sub-nephrotic range proteinuria (UPCR 1.0) with otherwise stable graft function (serum creatinine 1.2 mg/dl). Biopsy revealed features of recurrent CG with notable curved, banded subendothelial aggregates (Figure 1). Diagnosis of recurrent collagenofibrotic glomerulopathy was made and supported by tandem mass spectrometry which confirmed composition of the aggregates as type III collagen.
Discussion
CG is a rare disorder characterized by abnormal glomerular deposition of type III collagen. Its cause and pathogenesis are unclear and lack definitive treatment. There is a paucity of cases describing recurrence post-transplant and its implication on long-term graft outcomes is unknown. Here, we describe a case of late recurrent CG, presenting with sub-nephrotic proteinuria and stable allograft function.