Abstract: PO1717
A Case of Secondary Focal Segmental Glomerulosclerosis and Thrombotic Microangiopathy in a Heart Transplant Patient
Session Information
- Glomerular Diseases: Fibrosis and Extracellular Matrix
October 22, 2020 | Location: On-Demand
Abstract Time: 10:00 AM - 12:00 PM
Category: Trainee Case Report
- 1201 Glomerular Diseases: Fibrosis and Extracellular Matrix
Authors
- Deval, Neha, Westchester Medical Center, Valhalla, New York, United States
- Me, Hay Me, Westchester Medical Center, Valhalla, New York, United States
- Griffiths, Jennifer, Westchester Medical Center, Valhalla, New York, United States
- Kapoor, Aromma, Westchester Medical Center, Valhalla, New York, United States
- Chugh, Savneek S., Westchester Medical Center, Valhalla, New York, United States
Introduction
A few case reports have shown that focal segmental glomerulosclerosis (FSGS) can lead to thrombotic microangiopathy (TMA) in chronic kidney disease patients with severe hypertension. One case report presented the development of FSGS and TMA in liver transplant patient on Tyrosine kinase inhibitor. We present a case of FSGS without vascular injury despite clinically diagnosed TMA in heart transplant patient.
Case Description
A 42-year old female with history of postpartum cardiomyopathy with implantable cardioverter defibrillator since 2005 was admitted for heart transplant evaluation. Patient developed rapid progressive worsening of renal failure requiring hemodialysis after the heart transplant. Urinalysis showed proteinuria, hematuria; blood work showed hemolytic anemia, thrombocytopenia and schistocytes. TMA was diagnosed and eculizumab was started while continuing with hemodialysis. Heart biopsy showed no rejection, but kidney biopsy revealed the pathological diagnosis of secondary FSGS of not otherwise specified type without vasculitis under both light and electron microscopy. No significant glomerular staining seen on immunofluorescence microscopy as well. Patient was maintained on immunosuppressive regime with mycophenolate, tacrolimus and prednisone, receiving eculizumab weekly for 3 months, and subsequently recovered from hemodialysis.
Discussion
It is very rare to have FSGS without microangiopathy in hematologically confirmed TMA. Calcineurin induced inhibitors (CIN) are known to cause various forms of acute kidney injury including FSGS. In our case, presumed calcinuerin induced nephrotoxicity presented as secondary FSGS without angiopathy.
This case reflects the unpredictability of the etiology of kidney disease based solely on clinical features and blood tests. No improvement in kidney function necessitated the renal biopsy. It also raises the challenging points in treatment regime in transplant patient populations.