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Kidney Week

Abstract: PO2586

Unexpected Recurrence of Undiagnosed ANCA-Associated Vasculitis in a Kidney Transplant Recipient

Session Information

Category: Trainee Case Report

  • 1902 Transplantation: Clinical

Authors

  • Naik, Ruchi Harshadrai, Vanderbilt University Medical Center, Nashville, Tennessee, United States
  • Schaefer, Heidi M., Vanderbilt University Medical Center, Nashville, Tennessee, United States
  • Shawar, Saed, Vanderbilt University Medical Center, Nashville, Tennessee, United States
Introduction

ANCA Associated Vasculitis (AAV) is one of the leading causes of End-stage Kidney disease (ESKD). The relapses of AAV after kidney transplant are relatively rare. As per the literature, the positive ANCA status in not a contraindication for transplant and patients usually get transplant once in clinical remission. Here, we are describing a unique case of ESKD due to renal limited AAV which was diagnosed retrospectively after the development of the recurrence two months post-transplant.

Case Description

A 69 years old Caucasian female with ESKD presumed due to hypertension received a deceased donor kidney transplant in Oct 2019 after being on dialysis for 4 years. She received alemtuzumab for induction followed by tacrolimus, mycophenolate mofetil, and prednisone for the maintenance immunosuppression. Her immediate post-transplant course was complicated by delayed graft function and her creatinine never went down below 2 mg/dl. Allograft biopsy was planned after 2 months due to persistent microscopic hematuria with progressive sub nephrotic range proteinuria. The immuno-histopathology and electron microscopy was suggestive of pauci-immune crescentic GN. Her serology workup was positive for ANA, ANCA with high titers of MPO. She lacked any other systemic involvement, and drugs induced ANCA was ruled out. She was treated with a pulse dose of methylprednisone and one dose of rituximab. Gradually her creatinine improved to 1.37 mg/dl with down trending MPO antibody titers within 4-6 weeks. The review of her chart retrospectively showed clinically asymptomatic ANCA positivity with high MPO titers 3.5 years back, suggestive of recurrence of AAV more than De novo AAV post-transplant.

Discussion

Physician should always be vigilant about the recurrence of the primary disease after transplant, especially when patients have undiagnosed primary kidney disease like AAV, where early diagnosis and treatment in the early stage of the disease is important to optimize results of renal transplantation. A randomized prospective study is needed to answer the question whether the ANCA positivity at the time of transplant adversely affect the outcomes.