Kidney Week

Abstract: PUB498

Collapsing Focal Segmental Glomerulosclerosis 5 Years after Transplant: Diagnosis, Treatment, and Outcome

Session Information

• Publication Only

Category: Transplantation

• 2102 Transplantation: Clinical

Authors

• Rodriguez Medina, Ulises, University of New Mexico School of Medicine, Albuquerque, New Mexico, United States

Ulises Rodriguez Medina, MD, PhD

• Aragon, Claudia N., University of New Mexico School of Medicine, Albuquerque, New Mexico, United States

Claudia N. Aragon, MD

• Onuoha, Kingsley I., University of New Mexico School of Medicine, Albuquerque, New Mexico, United States

Kingsley I. Onuoha, MD

• Avila, Paul Brandon, University of New Mexico School of Medicine, Albuquerque, New Mexico, United States

Paul Brandon Avila, MD

• Singh, Namita, University of New Mexico School of Medicine, Albuquerque, New Mexico, United States

Namita Singh, MBBS, MD, FASN

• Singh, Pooja P., University of New Mexico School of Medicine, Albuquerque, New Mexico, United States

Pooja P. Singh, MD, FASN

• Holanda, Danniele Gomes, Arkana Laboratories, Little Rock, Arkansas, United States

Danniele Gomes Holanda, MD

• Garcia, Pablo, University of New Mexico School of Medicine, Albuquerque, New Mexico, United States

Pablo Garcia, MD, FASN

Introduction

Collapsing FSGS involves segmental and global collapse of glomerular capillaries. Its etiology is often unclear; risk factors include genetic predisposition, immune abnormalities, and environmental exposure. Predominantly affects AA and rapidly progresses to ESKD. Recurrence rate after KT is about 20-50%, often leading to allograft loss. We present a case of recurrent FSGS after KT.

Case Description

A 39-yo Hispanic KT recipient with ESKD secondary to FSGS developed nephrotic syndrome five years post-transplant. Urine protein-creatinine was 8.8 g/g with eGFR of 105 ml/min. KT biopsy revealed collapsing FSGS with rare tubular cells showing weak nuclear staining for SV40. Tests for CMV, parvovirus, and HIV were negative. Genetic testing for APOL1 was negative in both patient and donor. Given the presence of SV40, JC virus PCR was checked and was positive. Patient was treated with 9 PLEX sessions and a prednisone taper, reducing proteinuria to 3.7 g/g. One month later, proteinuria increased to 5 g/g. Given the persistent positive JC virus, we discussed potential risks/benefits and treated him with rituximab 1g. Proteinuria declined significantly to 0.6 g/g after two months, and eGFR has remained stable at 110 ml/min.

Discussion

This case highlights the recurrence of FSGS five years after KT, relatively late for post-KT recurrent FSGS. Efficacy of treatment modalities in post-transplant FSGS, especially collapsing FSGS, remains challenging. However, our case suggests that combining PLEX, steroids, and rituximab is effective. JC virus infection can potentially lead to PML. Further studies are warranted to understand this population's risk-benefit profile with rituximab.

Bright Field Image, 400x (Jones Methenamine Silver Stain): Glomerular lesion with collapsing features, showing compressive podocyte hyperplasia and hypertrophy.