ISN-TTS Webinar: Treatment of post-transplant recurrent FSGS in children
- 2 PM CEST
Up to 60% of pediatric renal transplant recipients with end‐stage renal disease due to primary focal and segmental glomerulosclerosis (FSGS) may develop recurrent disease. Such recurrence is associated with poor prognosis if no remission is achieved. There are different experiences with protocols based on plasmapheresis and increased immunosuppression that have resulted in a high long‐lived remission rate. Although a large number of therapies have been employed to treat FSGS recurrence, there is currently no consensus treatment and most of the treatments have not been evidence-based largely due to the lack of prospective randomized controlled trials. The decision to increase immunosuppression in FSGS recurrence in children is further complicated since most of these children have already had significant pretransplant exposure to immunosuppression as part of attempts to reverse FSGS in their native kidneys. Concerns for cumulative toxicity of certain medications, such as cyclophosphamide, may lead to decisions to forgo the use of what may be an efficacious therapy, especially when there is not clarity as to the best overall therapy or the best population for that drug’s efficacy. The paucity of prospective controlled studies not only exacerbates the lack of clarity as to best current management strategies but also complicates the assessment of novel therapies as they become available. For instance, rituximab and LDL-apheresis have both been used increasingly to treat FSGS recurrence, but their utility in children with recurrent disease in general is difficult to assess when there has been little formal assessment of current therapies to allow baseline levels of expectation.
The importance of identifying the best therapies is underscored by long-term outcomes in successfully transplanted patients as compared to those with failed allografts. FSGS recurrence that does not respond to treatment leads to early graft loss and return to dialysis, and most often pretends future episodes of recurrence with subsequent transplantation
Learning objectives:
- To describe biochemical and immunologic characteristics of Nephrotic syndrome different to SSNS: FSGS
- To point out options for Kidney transplantation in children with FSGS
- To show results from different approach to Kidney transplantation using: plasmapheresis, high dose of Inhibitors of calcineurin (Cyclosporine, Tacrolimus), rituximab or Cyclophosphamide.
Further reading:
- Raina R, Jothi S, Haffner D, et al. Post-transplant recurrence of focal segmental glomerular sclerosis: consensus statements. Kidney Int. 2024;105(3):450-463. doi:10.1016/j.kint.2023.10.017
- Kopp JB, Anders HJ, Susztak K, et al. Podocytopathies. Nat Rev Dis Primers. 2020;6:68.
- Watts AJB, Keller KH, Lerner G, et al. Discovery of autoantibodies targeting nephrin in minimal change disease supports a novel autoimmune etiology. J Am Soc Nephrol. 2022;33:238–252.
- VincentiF AA, Ghiggeri GM (2023) State of the art in childhood nephrotic syndrome: concrete discoveries and unmet needs. Front Immunol 14:1167741. https://doi.org/10.3389/fimmu.2023.11677
- Trautmann A, Vivarelli M, Samuel S et al (2020) IPNA clinical practice recommendations for the diagnosis and management of children with steroid-resistant nephrotic syndrome. Pediatr Nephrol 35:1529–1561. https://doi.org/10.1007/ s00467-020-04519-1
- Francis A, Didsbury M, McCarthy H, Kara T. Treatment of recurrent focal segmental glomerulosclerosis post‐kidney transplantation in Australian and New Zealand children: a retrospective cohort study. Pediatr Transplant. 2018;22. https://doi.org/10.1111/petr.13185.
- Baum MA, Ho M, Stablein D, Alexander SR. Outcome of renal transplantation in adolescents with focal segmental glomeruelosclerosis. Pediatr Transplant. 2
- Filler G, Restrepo JM. The urgent need for more research on how to treat recurrent focal and segmental glomerulosclerosis. Pediatr Transplant. 2018;22. https://doi.org/10.1111/petr.13215.
- Outcome of renal transplantation in adolescents with focal segmental glomerulosclerosis
Moderator
Nancy Rodig (USA)
Rupesh Raina (USA)
Speakers
Jaime M Restrepo R (Colombia)
Javier Andrés Tascón Hernández (Colombia)
David Andres Ballesteros Castro (Colombia)