January 2020 - Presented by Dr. Guofeng Gao (Mentored by Dr. Grace F. Monis)
A 24 year old Caucasian female with chronic kidney disease stage V had been on peritoneal dialysis for two years. Last year, she was evaluated for kidney transplantation by our adult transplant nephrology clinic.
This patient first presented at age 11 with multiple urinary tract infections and was subsequently found to have left Ureteropelvic Junction obstruction. She had no known interventions. At age 14 she presented with nephrotic syndrome with periorbital and leg swelling and low serum albumin (as low as 1.0 mg/dL, normal albumin range: 3.4 to 5.4 g/dL). She had a right native kidney biopsy done at age 16, which showed focal segmental glomerulosclerosis (FSGS). High dose prednisone was offered but patient did not pursue. At age 20, she started following up at UCDHS. At that point, her serum creatinine was 2.0 mg/dL with nephrotic range protein about 10 gm/day. She was treated with a trial of prednisone 1 mg/kg with no improvement of function and proteinuria. Follow-up kidney biopsy showed FSGS with extensive global glomerulosclerosis and advanced renal interstitial fibrosis and tubular atrophy, and thus no further immunosuppression was offered. She progressed to End Stage Renal Disease and started peritoneal dialysis.
At age 23 she received a kidney transplant from a living donor (her cousin). After transplant, she had immediate graft function with baseline serum creatinine (sCr) 1.3 -1.5 mg/dL (normal sCr range: 0.84 to 1.21 mg/dL). However, a month later, because her spot urine protein/creatinine ratio rose to 5.2 mg/mg Cr (the upper limit of normal spot urine protein/creatinine ratio: <0.3 mg/mg Cr), she was admitted for transplant kidney biopsy. The biopsy showed diffuse foot process effacement on electron microscope and no FSGS on light microscopy, confirming recurrent podocytopathy. Then she received daily Therapeutic Plasma Exchange (TPE), followed by weaning and oral prednisone (also rituximab). Repeat transplant kidney biopsy two months later showed no FSGS on light microscopy. No electron microscope study was done due to lack of sample. Proteinuria worsened after TPE was tapered down to once a week, so she was restarted back on TPE twice a week and her prednisone dose also increased, followed by weaning. Repeat transplant kidney biopsy a year later showed recurrent FSGS on light microscopy and diffuse foot process effacement on electron microscope. Now she undergoes regular TPE every 2 weeks.
D.) ASFA Category I (first-line therapy) and ASFA Grading Recommendations 1B (Strong recommendation, moderate-quality evidence)
> Learn more about focal segmental glomerulosclerosis.