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Published on: April 12, 2021
Management and long-term outcome of recurrent idiopathic FSGS in pediatric kidney transplant recipients
Moran Plonsky-Toder1,2, Shirley Pollack3,4, Rami Tibi3
1Pediatric Nephrology Institute, Ruth Children's Hospital, Rambam Health Care Campus, Haifa, Israel. m_plonsky@rmc.gov.il.
Insights
Early plasmapheresis and rituximab effectively treat recurrent focal segmental glomerulosclerosis (rFSGS) in pediatric kidney transplants, inducing remission and preventing graft loss. This approach offers a well-tolerated option for managing rFSGS post-transplantation.
Area of Science:
- Nephrology
- Pediatric Nephrology
- Transplantation Immunology
Background:
- Focal segmental glomerulosclerosis (FSGS) is a leading cause of pediatric kidney failure.
- Idiopathic FSGS in children carries a high risk of recurrence after kidney transplantation, potentially leading to graft loss.
- Current treatments for recurrent FSGS (rFSGS) post-transplantation include plasmapheresis and immunomodulatory therapies.
Purpose of the Study:
- To evaluate the efficacy and safety of an early plasmapheresis followed by rituximab treatment protocol for inducing and maintaining remission in pediatric kidney transplant recipients with rFSGS.
- To assess the rate of remission, time to remission, and the need for continued plasmapheresis or rituximab therapy.
Main Methods:
- Retrospective analysis of 8 pediatric kidney transplant recipients diagnosed with idiopathic FSGS between 2014 and 2023.
- Patients with rFSGS were initially managed with plasmapheresis.
- Rituximab was administered to patients not achieving complete remission with plasmapheresis or those dependent on it.
Main Results:
- Recurrent FSGS (rFSGS) occurred in 6 of 8 (75%) pediatric kidney transplant recipients.
- All patients achieved partial or complete remission with plasmapheresis, with a median response time of 13 days.
- Rituximab therapy led to sustained remission and cessation of plasmapheresis in 3 of 5 patients, and improved proteinuria/reduced frequency in the other 2. No grafts were lost due to rFSGS.
Conclusions:
- Early plasmapheresis combined with rituximab is an effective and well-tolerated strategy for inducing and maintaining remission in pediatric post-transplant rFSGS.
- This therapeutic approach appears to prevent graft loss associated with rFSGS.
- Larger prospective studies are warranted to confirm these findings and optimize treatment protocols.
Abstract:
Focal segmental glomerulosclerosis (FSGS) is a major cause of pediatric kidney failure. Most cases of FSGS in children are idiopathic and have a high risk of post-transplantation recurrence and graft loss. Common treatments for recurrent FSGS (rFSGS) post-transplantation include plasmapheresis, immunoadsorption, and/or immunomodulatory therapy. This study retrospectively evaluated the efficacy and safety of early plasmapheresis followed by rituximab for inducing and maintaining remission in rFSGS. Between 2014 and 2023, 8 of 65 pediatric kidney transplant recipients at our center were diagnosed with idiopathic FSGS. rFSGS was diagnosed based on nephrotic range proteinuria with no other cause and managed with plasmapheresis. Rituximab therapy was used for those who did not achieve complete remission with prolonged plasmapheresis or remained plasmapheresis dependent. 6 of 8 (75%) transplant recipients with idiopathic FSGS experienced rFSGS. All patients achieved partial or complete remission with plasmapheresis, with response times ranging from 8 to 379 days (median 13 days). Rituximab therapy was introduced for 5 plasmapheresis-dependent patients, leading to sustained remission and cessation of plasmapheresis in 3 patients, while 2 showed improved proteinuria and reduced plasmapheresis frequency. Adverse effects included rituximab-induced serum sickness in one patient and one mild allergic reaction. One patient experienced graft loss due to humoral rejection, but no grafts were lost to rFSGS, and all other grafts remained functional over an average follow-up of 50 months. Early plasmapheresis followed by rituximab therapy effectively induces remission in most post-transplantation rFSGS cases, is well tolerated, and prevents graft loss. Larger studies are needed to confirm these findings.
