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Treatment of Focal Segmental Glomerulosclerosis Recurrence in the Renal Allograft: A Report of Two Cases.

Tran MH, Chan C, Pasch W, Carpenter P, Ichii H, Foster C - Case Rep Nephrol Dial (2016)

Bottom Line: Spot urine protein:creatinine ratios were monitored and treatment was continued until a target of <0.5 was achieved.In patient number two, a second peak in proteinuria and azotemia was ultimately attributable to ureteral stenosis and these values normalized following repair.In conclusion, therapeutic plasma exchange is an effective treatment for FSGS recurring following renal transplant.

View Article: PubMed Central - PubMed

Affiliation: Department of Pathology and Laboratory Medicine, UC Irvine Health School of Medicine, USA.

ABSTRACT
Focal segmental glomerulosclerosis (FSGS) causes glomerular lesions that can progress to end-stage renal disease. It is suspected to be caused by a circulating factor that is amenable to plasmapheresis removal and exhibits a risk for recurrence in the renal allograft. We present two patients with FSGS recurrence in their allograft kidneys diagnosed by biopsy after significant proteinuria developed in the posttransplant setting. Treatment with therapeutic plasma exchange induced long-term remission in both patients. Spot urine protein:creatinine ratios were monitored and treatment was continued until a target of <0.5 was achieved. In patient number two, a second peak in proteinuria and azotemia was ultimately attributable to ureteral stenosis and these values normalized following repair. In conclusion, therapeutic plasma exchange is an effective treatment for FSGS recurring following renal transplant.

No MeSH data available.


Related in: MedlinePlus

Case 2, treatment course. Arrows depict therapeutic plasma exchange procedures.
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Figure 3: Case 2, treatment course. Arrows depict therapeutic plasma exchange procedures.

Mentions: A 37-year-old Asian male with ESRD due to primary FSGS underwent living unrelated renal transplantation performed with thymoglobulin induction followed by standard steroid taper. On posttransplant day 3, significant proteinuria and a rise in serum creatinine developed. Therapeutic plasma exchange was initiated on posttransplant day 4 and continued forward as noted in figure 3. The patient also received a single dose of rituximab as well as IVIG as treatment for his recurrent FSGS. A kidney biopsy on posttransplant day 8 was negative for rejection, but demonstrated typical foot process effacement changes consistent with FSGS recurrent in the renal allograft. Following an initial complete response to therapeutic plasma exchange by posttransplant day 78, renal dysfunction and proteinuria recurred. A repeat allograft biopsy was obtained, which demonstrated improvement in the degree of foot process effacement and no evidence for rejection. The second bout of allograft dysfunction was ultimately discovered to be due to interval development of high-grade stenosis in the allograft ureter leading to a postobstructive nephropathy. Surgical correction of this abnormality led to normalization of his values. As of posttransplant day 317, blood urea nitrogen and creatinine (both as mg/dl) are 13 and 1.3, respectively, and there is continued absence of proteinuria.


Treatment of Focal Segmental Glomerulosclerosis Recurrence in the Renal Allograft: A Report of Two Cases.

Tran MH, Chan C, Pasch W, Carpenter P, Ichii H, Foster C - Case Rep Nephrol Dial (2016)

Case 2, treatment course. Arrows depict therapeutic plasma exchange procedures.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4836136&req=5

Figure 3: Case 2, treatment course. Arrows depict therapeutic plasma exchange procedures.
Mentions: A 37-year-old Asian male with ESRD due to primary FSGS underwent living unrelated renal transplantation performed with thymoglobulin induction followed by standard steroid taper. On posttransplant day 3, significant proteinuria and a rise in serum creatinine developed. Therapeutic plasma exchange was initiated on posttransplant day 4 and continued forward as noted in figure 3. The patient also received a single dose of rituximab as well as IVIG as treatment for his recurrent FSGS. A kidney biopsy on posttransplant day 8 was negative for rejection, but demonstrated typical foot process effacement changes consistent with FSGS recurrent in the renal allograft. Following an initial complete response to therapeutic plasma exchange by posttransplant day 78, renal dysfunction and proteinuria recurred. A repeat allograft biopsy was obtained, which demonstrated improvement in the degree of foot process effacement and no evidence for rejection. The second bout of allograft dysfunction was ultimately discovered to be due to interval development of high-grade stenosis in the allograft ureter leading to a postobstructive nephropathy. Surgical correction of this abnormality led to normalization of his values. As of posttransplant day 317, blood urea nitrogen and creatinine (both as mg/dl) are 13 and 1.3, respectively, and there is continued absence of proteinuria.

Bottom Line: Spot urine protein:creatinine ratios were monitored and treatment was continued until a target of <0.5 was achieved.In patient number two, a second peak in proteinuria and azotemia was ultimately attributable to ureteral stenosis and these values normalized following repair.In conclusion, therapeutic plasma exchange is an effective treatment for FSGS recurring following renal transplant.

View Article: PubMed Central - PubMed

Affiliation: Department of Pathology and Laboratory Medicine, UC Irvine Health School of Medicine, USA.

ABSTRACT
Focal segmental glomerulosclerosis (FSGS) causes glomerular lesions that can progress to end-stage renal disease. It is suspected to be caused by a circulating factor that is amenable to plasmapheresis removal and exhibits a risk for recurrence in the renal allograft. We present two patients with FSGS recurrence in their allograft kidneys diagnosed by biopsy after significant proteinuria developed in the posttransplant setting. Treatment with therapeutic plasma exchange induced long-term remission in both patients. Spot urine protein:creatinine ratios were monitored and treatment was continued until a target of <0.5 was achieved. In patient number two, a second peak in proteinuria and azotemia was ultimately attributable to ureteral stenosis and these values normalized following repair. In conclusion, therapeutic plasma exchange is an effective treatment for FSGS recurring following renal transplant.

No MeSH data available.


Related in: MedlinePlus