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Kidney Week

Abstract: SA-PO880

Under the Hood: Nephrosis in Autosomal Polycystic Kidney Disease

Session Information

Category: Transplantation

  • 2002 Transplantation: Clinical

Authors

  • Sharifi, Bobak, UVA Health, Charlottesville, Virginia, United States
  • Cathro, Helen P., UVA Health, Charlottesville, Virginia, United States
  • Nishio Lucar, Angie G., UVA Health, Charlottesville, Virginia, United States
  • Leeds, Joseph T., UVA Health, Charlottesville, Virginia, United States
Introduction

About 50% of patients with autosomal dominant polycystic kidney disease (APKD) will develop end-stage kidney disease (ESKD). The presence of multiple bilateral cysts is a relative contraindication to percutaneous kidney biopsy. Thus, APKD patients with chronic kidney disease (CKD) rarely undergo a native kidney biopsy. Concomitant proteinuria may appear in advanced CKD but nephrotic syndrome is rare. We report the case of an APKD patient presenting with proteinuria and progressive allograft dysfunction after kidney transplantation (KT) suggesting recurrence of native glomerular disease.

Case Description

A 62-year-old female with ESKD secondary to ADPKD underwent an uncomplicated decreased-donor KT in 2020. During her third month post-transplant, her serum creatinine increased to 1.9mg/dL (post-transplant nadir of 1.2 mg/dL) and developed progressive proteinuria (urine protein quantification 2.5g/g). Serologic tests (ANA, ANCA, C3/C4, anti-GBM, SPEP/UPEP/SFLC) were unremarkable. An allograft biopsy revealed no rejection or glomerular abnormalities on light microscopy. No C4d staining seen on immunofluorescence (IF). Capillary loops stained for IgG (3+), kappa (2+), and lambda (3+). Electron microscopy noted moderately effaced foot processes with sub-epithelial immune complex deposits. Serum phospholipase A2 receptor antibody (PLA2RAb) was high at 544 RU/ml. IF was positive (1+) for PLA2RAb granular capillary staining confirming the diagnosis of recurrent membranous nephropathy (MN). She received Rituximab therapy with improvement in her creatinine and proteinuria. Creatinine returned to nadir. Repeat PLA2RAb reduced to 11 RU/ml and proteinuria to 0.06 g/g. interestingly, the patient’s pre transplant serum revealed a PLA2RAb of 1247 RU/ml.

Discussion

There are few reports of MN associated with APKD and none in the context of KT. MN can present as de novo or recurrent disease after KT in 10-45% of cases. Although rare, limited literature suggest that 15-20% of APKD patients may have concomitant glomerular disease. This case emphasizes the importance of avoiding anchoring cognitive bias and remembering that primary causes of CKD may not be mutually exclusive.