Treatment outcomes of recurrent primary focal segmental glomerulosclerosis (FSGS) after kidney transplantation

2018 ◽  
Author(s):  
Rute Aguiar
Keyword(s):  
Kidney Transplantation ◽  
Treatment Outcomes ◽  
Focal Segmental Glomerulosclerosis ◽  
Segmental Glomerulosclerosis

scholarly journals Successful long-term management of recurrent focal segmental glomerulosclerosis after kidney transplantation with costimulation blockade

2020 ◽  
Author(s):  
Thomas Mühlbacher ◽  
Kerstin Amann ◽  
Moritz Mahling ◽  
Silvio Nadalin ◽  
Nils Heyne ◽  
...  
Keyword(s):  
Kidney Transplantation ◽  
Focal Segmental Glomerulosclerosis ◽  
Costimulation Blockade ◽  
Segmental Glomerulosclerosis ◽  
Remission Maintenance ◽  
Maintenance Immunosuppression ◽  
Therapeutic Concepts ◽  
Allograft Outcome

Abstract Recurrence of primary focal segmental glomerulosclerosis (FSGS) occurs in up to 50% of patients after kidney transplantation and is associated with poor allograft outcome. Novel therapeutic concepts directly target podocyte function via B7-1 with inconsistent response. We present the case of a 19 yr. old patient with recurrent primary FSGS early after living donor kidney transplantation. Plasmapheresis and rituximab did not induce remission. Repetitive abatacept administration was able to achieve partial remission. Maintenance immunosuppression was subsequently switched to a belatacept-based CNI-free immunosuppression, resulting in sustained complete remission with excellent allograft function throughout a follow-up of more than 56 months.

scholarly journals Novel diagnostic and therapeutic techniques reveal changed metabolic profiles in recurrent focal segmental glomerulosclerosis

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Janina Müller-Deile ◽  
George Sarau ◽  
Ahmed M. Kotb ◽  
Christian Jaremenko ◽  
Ulrike E. Rolle-Kampczyk ◽  
...  
Keyword(s):  
Kidney Transplantation ◽  
Raman Spectra ◽  
Focal Segmental Glomerulosclerosis ◽  
Disease Recurrence ◽  
Early Recurrence ◽  
Metabolome Analysis ◽  
Novel Technologies ◽  
Filtration Barrier ◽  
Segmental Glomerulosclerosis ◽  
Living Related

AbstractIdiopathic forms of Focal Segmental Glomerulosclerosis (FSGS) are caused by circulating permeability factors, which can lead to early recurrence of FSGS and kidney failure after kidney transplantation. In the past three decades, many research endeavors were undertaken to identify these unknown factors. Even though some potential candidates have been recently discussed in the literature, “the” actual factor remains elusive. Therefore, there is an increased demand in FSGS research for the use of novel technologies that allow us to study FSGS from a yet unexplored angle. Here, we report the successful treatment of recurrent FSGS in a patient after living-related kidney transplantation by removal of circulating factors with CytoSorb apheresis. Interestingly, the classical published circulating factors were all in normal range in this patient but early disease recurrence in the transplant kidney and immediate response to CytoSorb apheresis were still suggestive for pathogenic circulating factors. To proof the functional effects of the patient’s serum on podocytes and the glomerular filtration barrier we used a podocyte cell culture model and a proteinuria model in zebrafish to detect pathogenic effects on the podocytes actin cytoskeleton inducing a functional phenotype and podocyte effacement. We then performed Raman spectroscopy in the < 50 kDa serum fraction, on cultured podocytes treated with the FSGS serum and in kidney biopsies of the same patient at the time of transplantation and at the time of disease recurrence. The analysis revealed changes in podocyte metabolome induced by the FSGS serum as well as in focal glomerular and parietal epithelial cell regions in the FSGS biopsy. Several altered Raman spectra were identified in the fractionated serum and metabolome analysis by mass spectrometry detected lipid profiles in the FSGS serum, which were supported by disturbances in the Raman spectra. Our novel innovative analysis reveals changed lipid metabolome profiles associated with idiopathic FSGS that might reflect a new subtype of the disease.

scholarly journals Preoperative Low-Density Lipoprotein Apheresis for Preventing Recurrence of Focal Segmental Glomerulosclerosis after Kidney Transplantation

2018 ◽  
Vol 2018 ◽  
pp. 1-5 ◽  
Author(s):  
Akihito Sannomiya ◽  
Toru Murakami ◽  
Ichiro Koyama ◽  
Kosaku Nitta ◽  
Ichiro Nakajima ◽  
...  
Keyword(s):  
Renal Failure ◽  
Kidney Transplantation ◽  
Focal Segmental Glomerulosclerosis ◽  
Low Density Lipoprotein ◽  
Density Lipoprotein ◽  
Low Density ◽  
Ldl Apheresis ◽  
Segmental Glomerulosclerosis ◽  
Living Related ◽  
Observation Period

Background. Focal segmental glomerulosclerosis (FSGS) often develops rapidly and frequently progresses to renal failure, while the recurrence rate after kidney transplantation is 20–50%. We performed low-density lipoprotein (LDL) apheresis before kidney transplantation in FSGS patients to prevent recurrence. Methods. Five adult patients with chronic renal failure due to FSGS undergoing living related donor kidney transplantation were investigated retrospectively. LDL apheresis was done 1-2 times before transplantation. Postoperative renal function and recurrence of FSGS were assessed. Results. The patients were two men and three women aged 24 to 41 years. The observation period ranged from 60 days to 22 months. Preoperative LDL apheresis was performed once in one patient and twice in four patients. Blood LDL cholesterol levels were normal before LDL apheresis and remained normal both after LDL apheresis and after kidney transplantation. Additional LDL apheresis was performed once in one patient with mild proteinuria after transplantation. The renal graft survived in all patients and there was no evidence of recurrent FSGS. Conclusions. Although the observation period was short, FSGS did not recur in all 5 patients receiving preoperative LDL apheresis. These results suggest that LDL apheresis can be effective in preventing recurrence of FSGS after kidney transplantation.

scholarly journals RISK FACTORS AND TREATMENT OF RECURRENT FOCAL SEGMENTAL GLOMERULOSCLEROSIS AFTER KIDNEY TRANSPLANTATION

2020 ◽  
Vol 104 (S3) ◽  
pp. S470-S470
Author(s):  
Guodong Chen ◽  
Chang Wang ◽  
Zixuan Wu ◽  
Xiaomian Liu ◽  
Qihao Li ◽  
...  
Keyword(s):  
Risk Factors ◽  
Kidney Transplantation ◽  
Focal Segmental Glomerulosclerosis ◽  
Segmental Glomerulosclerosis

P1757RITUXIMAB FOR RECURRENCE OF PRIMARY FOCAL SEGMENTAL GLOMERULOSCLEROSIS AFTER KIDNEY TRANSPLANTATION: RESULTS OF A NATIONWIDE STUDY

2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
Camille Lanaret ◽  
Dany Anglicheau ◽  
Audard Vincent ◽  
Celine Lambert ◽  
Lionel Couzi ◽  
...  
Keyword(s):  
Kidney Transplantation ◽  
Focal Segmental Glomerulosclerosis ◽  
Standard Of Care ◽  
High Dose ◽  
Infectious Risk ◽  
Segmental Glomerulosclerosis ◽  
Group 2 ◽  
Retrospective Multicenter Study ◽  
Group 1 ◽  
Rejection Rates

Abstract Background and Aims The indication of rituximab (RTX) in the treatment of primary focal segmental glomerulosclerosis (FSGS) recurrence after kidney transplantation (KT) remains controversial. The objective of our study was to evaluate the benefit and tolerability of adding RTX to the standard of care (SOC) comprising plasmapheresis (PP), corticosteroids, and high-dose anticalcineurins for the treatment of FSGS recurrence after KT. Method This retrospective, multicenter study reports on 148 patients, transplanted between 31 December 2004 and 31 December 2018, aged 39.9 + 13.4 years, who developed FSGS recurrence at 7 [3–23] days. In all 109 patients received a SOC (Group 1). RTX was introduced in this group after more than 28 days of SOC for failure or for therapeutic intensification (n = 19, Group 1a), or for early discontinuation of PP (n = 12, Group 1b); 39 patients received RTX associated at the outset with SOC (Group 2). Results We observed 46.6% complete remission (CR) and 33.1% partial remission (PR). Ten-year graft survival was 65.6% [51.4–76.6] and 13.4% [3.4–30.0] in responders and non-responders respectively. There was no difference in CR + PR rate between G1 (82.5%) and G2 (71.8%), p = 0.08, confirmed by propensity score +4.3% (95% CI [−9.0%-17.5%], p = 0.53). Following addition of RTX (Group 1a), we observed a CR rate of 26.3% and a PR rate of 31.6%. Patients with and without RTX experienced similar rejection rates (18.6% and 28.2%, p = 0.17) and infection rates (71.4% and 79.5%, p = 0.40). In multivariate analysis, the infections were associted with hypogammaglobulinemia &lt;5g/l (OR = 8.04, 95% CI [1.65,39.25], p = 0.01). Conclusion Rituximab could be used in cases of SOC failure or in remission patients for early weaning of plasmapheresis, without increasing infectious risk.

scholarly journals Galactose therapy reduces proteinuria in patients with recurrent focal segmental glomerulosclerosis after kidney transplantation

Nephrology ◽  
2015 ◽  
Vol 20 ◽  
pp. 13-16 ◽  
Author(s):  
Kate Robson ◽  
Prudence Hill ◽  
David Langsford ◽  
Karen Dwyer ◽  
David Goodman ◽  
...  
Keyword(s):  
Kidney Transplantation ◽  
Focal Segmental Glomerulosclerosis ◽  
Segmental Glomerulosclerosis

scholarly journals Successful Treatment of Focal Segmental Glomerulosclerosis after Kidney Transplantation with Plasma Exchange and Abatacept in a Patient with Juvenile Rheumatoid Arthritis

2016 ◽  
Vol 2016 ◽  
pp. 1-4 ◽  
Author(s):  
Hannelore Sprenger-Mähr ◽  
Emanuel Zitt ◽  
Afschin Soleiman ◽  
Karl Lhotta
Keyword(s):  
Rheumatoid Arthritis ◽  
Kidney Transplantation ◽  
Renal Transplantation ◽  
Plasma Exchange ◽  
Juvenile Rheumatoid Arthritis ◽  
Focal Segmental Glomerulosclerosis ◽  
Caucasian Patient ◽  
Segmental Glomerulosclerosis ◽  
Allograft Biopsy ◽  
Progression Of Disease

Recurrent focal segmental glomerulosclerosis (FSGS) after renal transplantation is difficult to treat. Recently a series of four patients unresponsive to plasma exchange (PE) and rituximab, who were successfully treated with abatacept, has been reported. We present a 26-year-old Caucasian patient who suffered from juvenile rheumatoid arthritis and developed severe proteinuria eleven days after transplantation. An allograft biopsy was suggestive of recurrent focal segmental glomerulosclerosis. He did not respond to PE therapy. A first dose of abatacept produced partial remission. Four weeks later proteinuria again increased and a second biopsy showed progression of disease. After another ineffective course of PE he was given a second dose of abatacept, which was followed by rapid, complete, and sustained resolution of proteinuria. This treatment caused a significant increase in BK and JC viremia. Whether abatacept ameliorated proteinuria via an effect on podocytes or on the patient’s primary disease remains speculative.

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