Kidney Week

Abstract: PUB234

Recurrence of Granulomatosis with Polyangiitis in a Kidney Transplant Recipient

Session Information

• Publication Only

Category: Transplantation

• 2002 Transplantation: Clinical

Authors

• Vega-Martinez, Krystal C., Vanderbilt Health, Nashville, Tennessee, United States

Krystal C. Vega-Martinez, MD

• Shawar, Saed, Vanderbilt Health, Nashville, Tennessee, United States

Saed Shawar, MD

• Richardson, Trey Howard, Vanderbilt Health, Nashville, Tennessee, United States

Trey Howard Richardson, MD

• Wang, Yihan, Vanderbilt Health, Nashville, Tennessee, United States

Yihan Wang, MD

Introduction

Granulomatosis with polyangitis (GPA) is an ANCA-associated vasculitis characterized by necrotizing inflammation of small vessels and rapidly progressive organ damage, particularly the kidneys and lungs. Progression to ESKD is seen in about ~20-25% cases, and patients who receive transplantation have a lower risk of death than those who remain on dialysis. Despite improvements in survival with transplantation, disease recurrence, although rare, could threaten graft survival and lead to graft loss, requiring greater vigilance post-transplantation.

Case Description

58 y/o F with H/o of ESRD secondary to GPA s/p kidney transplant on 10/2022 (Myfortic/FK/Prednisone), RCC s/p R nephrectomy (4/2022), DM, HTN, and pAfib who presented for Tx kidney biopsy given worsening renal function. The patient had a kidney bx 4 mo prior to presentation, consistent with ATN and mild CAN. She reported arthralgia, SOB, and a week of diarrhea 3-5x per day associated with N/V in the setting of a recent Myfortic dose change. She denied fever, chills, decreased UOP, dysuria, hematuria, or any other associated symptoms. On presentation, vital signs were normal. The physical exam was unremarkable. BMP showed bicarbonate 20 mmol/L, BUN 45 mg/dL, SCr 2.46 mg/dL (Baseline ~1). UA showed proteinuria 20 mg/dL, large blood, 5 WBCs, 337 RBCs. Infectious workup was negative, including GIPP, C. diff, CMV, and BKV. Serologic workup was negative for anti-GBM and anti-MPO antibodies but elevated for anti-PR3 (1.4 AI), with C3 141, C4 31, IgG 696, and TMPT 30.5 units/mL. Biopsy showed focal pauci-immune necrotizing crescentic glomerulonephritis, consistent with renal involvement of recurrent GPA, with moderate CAN and features suggestive of Calcineurin inhibitor toxicity. She was treated with a course of IVMP, Rituximab, IVIG for hypoglammaglobulinemia, and Myfortic was changed to Azathioprine. She was given prophylactic Valcyte for 4 months and pentamidine for 3 months. Cr at discharge was 2.8, and the most recent labs showed SCr 2.49, a negative repeat serologic panel, and improved proteinuria and hematuria.

Discussion

GPA recurrence is rare but can cause rapid glomerulonephritis, allograft dysfunction, or loss. Early recognition prevents misdiagnosis and treatment delays, as it may mimic rejection, infection, or drug toxicity. Awareness of recurrence is key for optimizing transplant timing and immunosuppression, as immunosuppression lowers recurrence risk but can mask vasculitis.