Abstract: SA-PO533
Successful Remission of Recurrent FSGS Following Lipid Apheresis in Renal Transplant Recipients
Session Information
- Immunosuppression, Disease Recurrence, and Malignancy
November 04, 2017 | Location: Hall H, Morial Convention Center
Abstract Time: 10:00 AM - 10:00 AM
Category: Glomerular
- 1005 Clinical Glomerular Disorders
Authors
- Shah, Lokesh N., Thomas Jefferson University, Philadelphia, Pennsylvania, United States
- LaRosa, Christopher J., None, Wilmington, Delaware, United States
- Speckhals, Justine, Nemours/ Alfred I. DuPont Hospital for children, Avondale, Pennsylvania, United States
- Tait, Carolyn, Nemours A.I. duPont Hospital for Children, Wilmington, Delaware, United States
- Lasalvia, Kimberly A., Nemours/duPont Hospital for Children, Wilmington, Delaware, United States
- Clark-Freeman, Kyla, Nemours/Alfred I DuPont Hospital for Children , Wilmington, Delaware, United States
- Moodalbail, Divya G., Nemours/ Alfred I. duPont Hospital for Children, Wilmington, Delaware, United States
- Zaritsky, Joshua, Nemours/Alfred I. duPont Hospital for Children, Wilmington, Delaware, United States
Background
The Liposorber® LA-15, a lipoprotein apheresis device, has been used as a rescue therapy in patients with recurrent primary focal segmental glomerulosclerosis (FSGS). Using a protocol of weekly lipid apheresis in combination with IV methylprednisolone, we describe the remission of recurrent FSGS in two pediatric kidney transplant recipients.
Methods
Both patients were diagnosed with steroid-resistant biopsy-proven FSGS at age 2. They failed to respond to multiple interventions (high dose steroids, high dose calcineurin inhibition, mycophenolate, and rituximab). They eventually underwent renal transplantation due to ensuing ESRD. Both post-transplantation courses were complicated by immediate recurrence of FSGS with urine protein/creatinine (Up/cr) ratios > 50. Again, both failed to respond to high dose steroid and calcinuerin inhibition; nor did they respond to 3 months of thrice weekly standard plasmapheresis. As a rescue therapy both patients underwent weekly lipid apheresis using the Liposorber® LA-15 system with a treatment volume of 60 mL/kg along with a 10 mg/kg dose of IV methylprednisolone. The final cycle of lipid apheresis occurred approximately 80 days after its initiation in both patients. In both cases there was a dramatic decrease in the urine protein to creatinine ratios (FIGURE). After stopping lipid apheresis both patients have remained in clinical remission without further treatment.
Conclusion
Lipid apheresis has shown promise in the treatment of both primary and recurrent FSGS; however, experience with this modality is limited. Our successful use of lipid apheresis in combination with methylprednisolone to induce remission in treatment resistant recurrent FSGS provides a protocol for future studies, and provides a better understanding of the ill-defined pathophysiology of hyperlipidemia and nephrotic syndrome.