Effaced but Not Erased: A Case of Consecutive Recurrences of Lupus Podocytopathy in Kidney Transplants

Introduction: Lupus podocytopathy is considered a distinct subtype of lupus nephritis. Histologically it can present as minimal change disease (MCD) and/or Focal segmental glomerulosclerosis (FSGS). We present a case of lupus podocytopathy in a 29 year old woman that relapsed in 2 consecutive transplants. Case Description: At first presentation patient had serology positive for lupus, urine protein/creatinine ratio (UPCR) of 10, native kidney biopsy showed lupus podocytopathy with minimal change features. After living unrelated kidney transplant, there was delayed graft function and dialysis was initiated. Induction immunosuppression was changed from Simulect to thymoglobulin, and belatacept, mycophenolate and prednisone were chosen for calcineurin-inhibitor avoidance. Patient noted to have malar rash, serology positive for antinuclear anitbodies 1:320, and double stranded DNA. Wedge allograft biopsy 3 days post-transplant showed recurrent Lupus podocytopathy. Patient was treated with Cytoxan, plasmapheresis, plaquenil and steroids; discharged off dialysis. Patient was then readmitted 2 weeks later for acute renal injury; restarted on dialysis. 24 hour urine collection showed 25 grams protein. Repeat allograft biopsy showed collapsing variant of FSGS. Patient was treated with plasmapheresis and immunosuppression with no response, progressed to graft failure with dialysis dependence Patient received a second living unrelated kidney transplant 3 years later with Thymoglobulin induction. As a means to reduce the risk of recurrence, patient received pre and post-transplant plasmapheresis and 4 doses of rituximab. She was discharged with creatinine 1 mg/dL but nephrotic proteinuria present with UPCR 6. A protocol biopsy showed recurrent podocytopathy/ focal segmental glomerulosclerosis. Patient was maintained on plasmapheresis, with stable allograft function. Discussion: Lupus podocytopathy is not a benign lesion. It requires vigilance for specific diagnosis and aggressive immunosuppression for treatment. Classically patients with FSGS subtype are less likely to respond to treatment, particularly with the collapsing variant. Even those with MCD features, may have relapsing events.