EFFACED BUT NOT ERASED - A CASE OF CONSECUTIVE RELAPSES OF LUPUS PODOCYTOPATHY IN RENAL TRANSPLANTS

Introduction:

Lupus podocytopathy is considered a distinct subtype of lupus nephritis. Histologically, it can present as minimal change disease (MCD) or focal segmental glomerulosclerosis (FSGS) on microscopy, and with podocyte effacement. We present a case in which lupus podocytopathy relapsed in a patient on consecutive renal transplants.

Methods:

During initial presentation, the patient had serology positive for lupus, urine protein/creatinine ratio (UPCR) of 10, and 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, while patient was maintained on Belatacept, mycophenolate, and prednisone. The patient was noted to have malar rash, serology positive for antinuclear antibodies 1:320, and double-stranded DNA. Wedge allograft biopsy 3 days post-transplant showed recurrent lupus podocytopathy. The patient was treated with Cytoxan, plasmapheresis, Plaquenil, and steroids; discharged off dialysis.

The patient was then readmitted 2 weeks later for acute renal injury and had to be restarted on dialysis. 24-hour urine collection showed 25 grams of protein. Repeat allograft biopsy showed collapsing variant of FSGS (Figures 1, 2, 3). The patient was treated with plasmapheresis and immunosuppression without clinical response, and then progressed to graft failure with dialysis dependence.

The patient received a second living unrelated kidney transplant 3 years later with Thymoglobulin induction. As a means to reduce the risk of recurrence, the patient received pre- and post-transplant plasmapheresis and 4 doses of rituximab. She was discharged with creatinine 1 mg/dL but nephrotic proteinuria with UPCR of 6. A protocol biopsy showed recurrent podocytopathy/focal segmental glomerulosclerosis. The patient was maintained on plasmapheresis, with stable allograft function.

Results:

Lupus podocytopathy is not a benign lesion. Patients with lupus podocytopathy in the transplant kidney, such as ours with recurrent podocytopathy/focal segmental glomerulosclerosis, require more vigilance and may benefit from measures such as plasmapheresis and immunosuppression to help prevent graft failure. Classically, patients with the FSGS subtype are less likely to respond to treatment, particularly with the collapsing variant. Even those with MCD features may have relapsing events. 

Conclusions:

It is important to be able to diagnose lupus podocytopathy, due to its response to treatment and the chance of relapse in transplant patients. Because lupus podocytopathy can increase the risk of graft failure, it is important to consider clinical markers of recurrence, such as proteinuria, and to determine which immunosuppression regimens can help prevent it.

This abstract has been submitted to American Society of Nephrology in the past. Re-submission is permitted.

I have no potential conflict of interest to disclose.

I did not use generative AI and AI-assisted technologies in the writing process.