Long-Term Outcome of Kidney Transplantation in Recipients with Focal Segmental Glomerulosclerosis

Self Cite

Section: Discussionsupporting

confidence: 77%

The impact of donor/recipient age difference and HLA mismatch on graft outcome in pediatric kidney transplantation

Trnka

McTaggart

Francis

2018

Self Cite

“…The reported post‐transplant recurrence of FSGS in our survey was 30%. Previously reported recurrence rates vary between 30% and 60% . Almost one‐third of the respondents (54% response rate) reported recurrence within 1 week after transplantation in all of their FSGS patients.…”

Section: Discussionmentioning

confidence: 99%

“…However, it is generally accepted that the risk of recurrence is not high enough to contraindicate the transplantation procedure. Factors that are reported to be associated with a higher risk of recurrence are as follows: (a) progression to ESRD within 3 years; (b) mesangial hypercellularity on biopsy; (c) pediatric age at onset; (d) the presence of a circulating permeability factor; (e) non‐genetic forms of FSGS; (f) native kidney nephrectomy before or at the time of transplant; (g) histology of MCD on initial renal biopsy; (h) lower serum albumin at initial diagnosis; and (i) initial responsiveness to Cs treatment . Treatment of recurrent disease is still empirical with none of the multiple approaches providing consistent efficacy .…”

Section: Introductionmentioning

confidence: 99%

European Society of Pediatric Nephrology survey on current practice regarding recurrent focal segmental glomerulosclerosis after pediatric kidney transplantation

Bouts

Veltkamp

Tönshoff

et al. 2019

Introduction Primary FSGS is an important cause of ESRD in children. FSGS recurrence after kidney transplantation is associated with early graft loss. No guidelines for treatment of FSGS recurrence exist. We conducted a survey to gain insight into variation of treatment between centers. Methods A survey was sent to all members of the ESPN on behalf of the “Renal Transplantation” and “Idiopathic Nephrotic Syndrome” working groups. Results Fifty‐nine nephrologists from 31 countries responded, reporting 807 FSGS patients, with 241 (30%) FSGS recurrences after transplantation. Recurrence varied from 0% to 100% between respondents. Native nephrectomy before or during transplantation was performed, respectively, always (37%), never (39%), or on clinical indication (17%). Half of the respondents started preventive treatment before transplantation, using PF (n = 10); R (n = 4); PF or IA, plus R (n = 9); cyclosporine (n = 2); or unknown (n = 4). Immunosuppressive therapy for patients without known mutations consisted of a combination of steroids, tacrolimus/cyclosporine, and MMF, with or without IL‐2R‐blockade in, respectively, 61% and 86% of the respondents. Sixty‐three percent applied a similar regimen to patients with known mutations. FSGS recurrence was treated with PF or IA, plus R by 66% of respondents; 54% observed no response. Complete remission in >50% of patients was reported by 41% of the respondents. Discussion FSGS recurrence after transplantation is common, but varies greatly between centers. We found great variability in preventive and therapeutic treatment regimens. Future research should focus on predisposing factors, including biopsy findings and genetic mutations, and standardized treatment.

“…Recurrence of FSGS is an important cause of graft failure in children post–kidney transplantation. Prior reports from international transplant registries indicate recurrence rates of 15%‐36%, with higher rates of recurrence noted in children with idiopathic FSGS. The PodoNet Registry cohort reported a post‐transplant disease recurrence rate of 25.8% in patients without a confirmed genetic disorder compared to 4.5% in patients with a genetic diagnosis …”

Section: Introductionmentioning

confidence: 96%

Risk factors associated with allograft failure in pediatric kidney transplant recipients with focal segmental glomerulosclerosis

Koh

Martz

Blydt-Hansen

et al. 2019

Background With improved outcomes for children transplanted with FSGS since previous NAPRTCS registry reports, this study re‐evaluates the association of living donation, immunosuppression, and DGF on graft survival. Setting Patients transplanted between 2002 and 2016, comparing FSGS diagnosis vs other glomerular diseases. Methods Primary outcomes were allograft survival and FSGS recurrent‐free graft survival. Potential risk factors were obtained at the time of transplant and up to 30 days post‐transplantation. Analysis considered a priori that DGF may be a proxy for severe FSGS recurrence. Multivariable survival models for outcome were tested for sensitivity without/with DGF to determine features independent of recurrence. Results From the larger cohort of 3010 patients, 5‐year graft survival in children with FSGS (n = 455) was worse (74.3%) compared with other glomerular diseases (87.1%, n = 690) (HR 1.45, P = 0.033). Modeling all glomerular diseases, survival risk was associated with deceased donor (HR 1.83, P = 0.002), re‐transplantation (HR 1.58, P = 0.013), and recipient age (HR 1.06/y, P = 0.002). The living donor advantage was not confirmed in a FSGS model (HR 1.51 for deceased, P = 0.12). DGF was highly associated with graft failure (HR 4.39, P < 0.001) and independent of re‐transplant history but not FSGS diagnosis. Induction agents or primary immunosuppression choices were not associated with survival. Conclusion Graft survival rates have improved since the previous report. Living donor did not predict graft failure, but there remains no survival advantage. DGF was the primary independent predictor for graft loss secondary to FSGS recurrence, consistent with DGF being a proxy for severe recurrent disease.