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Abstract: FR-PO766

ANCA-Associated Vasculitis Recurrence After Kidney Transplantation

Session Information

Category: Transplantation

  • 2102 Transplantation: Clinical


  • Bin Homam, Wadhah Mohammed, University of Arkansas for Medical Sciences, Little Rock, Arkansas, United States
  • Ayub, Fatima, University of Arkansas for Medical Sciences, Little Rock, Arkansas, United States
  • Elkalashy, Ahmed, University of Arkansas for Medical Sciences, Little Rock, Arkansas, United States
  • Manchala, Venkata Ramana, University of Arkansas for Medical Sciences, Little Rock, Arkansas, United States

Group or Team Name

  • UAMS Internal Medicine/Nephrology.

ANCA-associated vasculitis (AAV) is a well-recognized cause of End Stage Kidney Disease (ESKD). It is a necrotizing vasculitis that affects small vessels without immune complex deposition. About 20-25% of patients will progress to ESKD in a few years after diagnosis. AAV may recur in about 5-10% of cases after kidney transplantation despite standard immunosuppression. Here we present a case of AAV recurrence in a kidney transplant recipient.

Case Description

A 49-year-old white female with a past medical history of HTN and ESKD secondary to p-ANCA positive, pauci-immune glomerulonephritis received DDKT 3 years ago with post-transplant course complicated by cryptococcus meningitis and mycobacterium avium infection. She was maintained on cyclosporine and prednisone. Her creatinine rose from baseline of 0.9 to 2.3 along with new onset of microscopic hematuria and proteinuria and UPCR of 2.6 g/g. Her p-ANCA titer is 1:640. Cyclosporine levels ranged from 100-144 ng/ml. Kidney biopsy showed focal crescentic and necrotizing glomerulonephritis, borderline acute TCMR, and diffuse acute tubular injury. Of 22 glomeruli, 3 glomeruli contain cellular, 3 glomeruli contain fibrocellular, 2 glomeruli contain fibrous crescents and 2 glomeruli with cellular crescents also have fibrinoid necrosis. A focal segmental granular mesangial and loop staining is seen with IGG (trace), IGM (1+), C3 (1+), C1q (1+), kappa (trace), and lambda (trace). There is 1+ arteriolar and 2+ focal segmental tubular basement membrane staining with C3. Tubular casts are equally positive with IGA, kappa, and lambda. Electron microscopy with no deposits in any locations. The patient received a 3-days of pulse methylprednisolone, one dose of rituximab and is scheduled to receive a second dose of rituximab in two weeks. The patient was discharged on prednisone with plans for gradual taper over 4-6 months.


The diagnosis of AAV flare post transplantation is difficult as relapses may mimic infections and other complications of immunosuppression. Disease recurrence of AAV should be considered in the differential diagnosis of sudden worsening of kidney function and must be confirmed by biopsy. Rituximab is the therapy of choice both in ESKD and kidney transplant patients. It is recommended to maintain continuous vigilance in all AAV transplant recipients for early detection of relapses.