Tim Ulinski1. 1. Department of Pediatric Nephrology, Armand Trousseau Hospital (APHP) and University Paris 6 (UPMC), Paris, France. tim.ulinski@trs.aphp.fr
Abstract
PURPOSE OF REVIEW: Steroid-resistant nephrotic syndrome/focal segmental glomerulonephritis (FSGS) is the primary renal disease in approximately 10% of pediatric patients receiving a renal allograft. Risk factors for recurrence are a chronological age between 6 and 15 years at onset of the nephrotic syndrome and a rapid progression of the disease in the native kidneys leading to end-stage renal disease in less than 3 years. With rapid recurrence of FSGS and loss of the allograft, further renal transplants also carry a high likelihood of recurrence of nephrotic syndrome. RECENT FINDINGS: Different pathogenic factors have been discussed for the recurrence of proteinuria/FSGS in the transplanted kidney, especially the involvement of a proteinuric circulating factor. Treatment strategies are divided into two phases: induction of remission by plasma exchanges combined with high-dose intravenous or oral cyclosporine A; stabilization of remission by cyclophosphamide or rituximab, which showed promising results in several case reports. SUMMARY: No controlled studies have been performed yet to address the management of recurrent FSGS posttransplant. Complications related to the high-degree immunosuppression are not rare and should be regularly investigated. Therefore, the benefit: risk ratio for all immunosuppressive treatment strategies should be carefully evaluated for each individual patient.
PURPOSE OF REVIEW: Steroid-resistant nephrotic syndrome/focal segmental glomerulonephritis (FSGS) is the primary renal disease in approximately 10% of pediatric patients receiving a renal allograft. Risk factors for recurrence are a chronological age between 6 and 15 years at onset of the nephrotic syndrome and a rapid progression of the disease in the native kidneys leading to end-stage renal disease in less than 3 years. With rapid recurrence of FSGS and loss of the allograft, further renal transplants also carry a high likelihood of recurrence of nephrotic syndrome. RECENT FINDINGS: Different pathogenic factors have been discussed for the recurrence of proteinuria/FSGS in the transplanted kidney, especially the involvement of a proteinuric circulating factor. Treatment strategies are divided into two phases: induction of remission by plasma exchanges combined with high-dose intravenous or oral cyclosporine A; stabilization of remission by cyclophosphamide or rituximab, which showed promising results in several case reports. SUMMARY: No controlled studies have been performed yet to address the management of recurrent FSGS posttransplant. Complications related to the high-degree immunosuppression are not rare and should be regularly investigated. Therefore, the benefit: risk ratio for all immunosuppressive treatment strategies should be carefully evaluated for each individual patient.
Authors: Hee Gyung Kang; Heewon Seo; Jae Hyun Lim; Jong Il Kim; Kyoung Hee Han; Hye Won Park; Ja Wook Koo; Kee Hyuck Kim; Ju Han Kim; Hae Il Cheong; Il-Soo Ha Journal: J Int Med Res Date: 2017-01-01 Impact factor: 1.671
Authors: Wen Y Ding; Ania Koziell; Hugh J McCarthy; Agnieszka Bierzynska; Murali K Bhagavatula; Jan A Dudley; Carol D Inward; Richard J Coward; Jane Tizard; Christopher Reid; Corinne Antignac; Olivia Boyer; Moin A Saleem Journal: J Am Soc Nephrol Date: 2014-02-07 Impact factor: 10.121