Kidney Week

Abstract: SA-PO862

A Case of Pediatric Steroid-Resistant FSGS Evolving into a Steroid-Sensitive Profile in Young Adulthood

Session Information

• Glomerular Diseases: Case Reports - 2
  October 26, 2024 | Location: Exhibit Hall, Convention Center
  Abstract Time: 10:00 AM - 12:00 PM

Category: Glomerular Diseases

• 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics

Authors

• Fontanella, Antonio Miguel, University of Miami Miller School of Medicine, Miami, Florida, United States

Antonio Miguel Fontanella

• Grewal, Meghan Rai, University of Miami Miller School of Medicine, Miami, Florida, United States

Meghan Rai Grewal

• Ortigosa Serrano, Veronica A., The William J. Harrington Medical Training Programs for Latin America and the Caribbean and Global Observaerships, University of Miami, International Medicine Institute, Miami, United States

Veronica A. Ortigosa Serrano, MD

• Burke, George William, University of Miami Miller School of Medicine, Miami, Florida, United States

George William Burke, MD

• Fornoni, Alessia, University of Miami Miller School of Medicine, Miami, Florida, United States

Alessia Fornoni, MD, PhD, FASN

• Roth, David, University of Miami Miller School of Medicine, Miami, Florida, United States

David Roth, MD

Introduction

Recurrent focal segmental glomerulosclerosis (rFSGS) is a challenging complication affecting 30-40% of transplanted FSGS patients, which can lead to loss of graft function and end-stage kidney disease. In this case report, I, the first author, will share my recent experience with rFSGS and hope to showcase the use of glomerular biomarkers to monitor disease evolution and guide treatment.

Case Description

I received a kidney transplant at 14 years old due to FSGS and PKD and developed rFSGS within 24 hours. Post-reperfusion renal biopsy showed that I was B7-1 positive, and I have been effectively treated with abatacept at the time and during various episodes of recurrence until the current case.

I presented to transplant follow-up with fatigue, edema, and abdominal discomfort. Urine dipstick had shown +2 proteinuria, and labs showed a UPCR of 6.5, with a SCr of 0.9. Over the next 2 months, I received 4 doses of abatacept 1 g IV to no effect, with UPCR increasing to 10.8 and serum albumin falling to 1.8 with worsening symptoms.

A cytokine panel found significant elevations in multiple cytokines. (Table 1). Standard treatment with plasmapheresis and rituximab 700 mg IV showed little effect, leading us to pursue allograft biopsy. The glomeruli demonstrated non-collapsing segmental sclerosis (tip variant) and mild mesangial expansion, as well as foot process effacement, consistent with rFSGS. B7-1 staining was negative, marking a shift from my previous status from B7-1 positive. [Figure 1]

Plasmapheresis was continued for a total of 10 exchanges, accompanied by bolus steroid dosing followed by oral prednisone taper, and a repeat dose of rituximab 700 mg IV. There was a rapid response with decreasing proteinuria and clinical improvement in symptoms. Follow-up labs obtained 1 week after completion of treatment showed a UPCR of 0.2 with an albumin of 4.1 and SCr of 1.2. [Figure 2]

Discussion

Here, we report the first case of a pediatric patient with steroid resistant B7-1 positive rFSGS who transitioned to a B7-1 negative profile responsive to steroids in adulthood. It is crucial to observe the growing cohort of pediatric rFSGS patients reaching adulthood to determine the effect of age on pathology, outcomes, and treatment response.