Abstract: PUB596
Double Trouble: Combined ANCA Vasculitis Relapse and Antibody-Mediated Rejection in a Kidney Transplant Recipient
Session Information
Category: Trainee Case Report
- 1203 Glomerular Diseases: Clinical, Outcomes, and Trials
Authors
- Scott, Jennifer, Trinity Health Kidney Centre, Dublin, Ireland
- Dorman, Anthony M., Beaumont Hospital, Dublin, Ireland
- Little, Mark Alan, Trinity College Dublin, Dublin, Ireland
- de Freitas, Declan G., Beaumont Hospital, Dublin, Ireland
Introduction
A female kidney transplant recipient, presented with graft dysfunction associated with headache, polyarthralgia, sinusitis, epistaxis, fatigue and vomiting for 2 weeks. She had a history of PR3 positive anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV), diagnosed 6 years prior, resulting in end-stage renal disease, despite treatment with the CYCLOPS protocol and plasma exchange. She received a ‘standard immunological risk’ deceased donor kidney transplant 3 years later. Maintenance immunosuppression (IS) included Prograf 3mg BD, Mycophenolate Mofetil 750mg BD and prednisolone 5mg OD. The patient denied medication non-adherence.
Case Description
Examination was unremarkable. Initial investigations revealed severe non-oliguric acute kidney injury (creatinine 685 µmol/L from 110 µmol/L baseline). Urinalysis showed 4+ blood and 1+ protein. Anti-PR3 antibody level rose to 81 IU/ml from 5 IU/ml ten months prior. Urine CD163 was elevated at 1225 ng/mmol (<0.3). There was a significant development in the strength and breadth of both class I and II donor specific antibodies. Renal biopsy demonstrated focal necrotising crescentic glomerulonephritis, tubulointerstitial inflammation and strong global C4d positivity in peritubular capillaries, consistent with AAV relapse and antibody-mediated rejection (ABMR). The Molecular Microscope Diagnostic Report® independently identified severe TCMR and ABMR (figure 1). She was treated with pulsed methylprednisolone (500mg x 3 days), followed by tapering prednisolone, Rituximab 375mg/m2 x 4 and continued maintenance IS. Management was influenced by prior IS exposure and the risk of- and subsequent development of infection. Unfortunately, she remained dialysis dependent and subsequently required graft nephrectomy for persistent rejection symptoms.
Discussion
While AAV relapse after kidney transplantation is rare, this case is a reminder that it can recur. The diagnostic biopsy in this case was supported by 2 novel techniques, a urine CD 163 and the Molecular Microscope Diagnostic Report®, identifying concomitant rejection and vasculitis. Despite treatment, the synchronous immunological mechanisms resulted in irreversible kidney damage. The wealth of objective evidence was key to personalising the risk-benefit of further IS in this case.