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Abstract: FR-PO619

ANCA Negative Pauci-Immune Necrotizing Glomerulonephritis in a Liver Transplant Patient

Session Information

Category: Glomerular Diseases

  • 1302 Glomerular Diseases: Immunology and Inflammation

Authors

  • Kumar, Arjun, University of Cincinnati, Cincinnati, Ohio, United States
  • McCartney, Audrey, University of Cincinnati, Cincinnati, Ohio, United States
  • Zimmermann, Nives, University of Cincinnati, Cincinnati, Ohio, United States
  • Brathwaite, Latoya L., University of Cincinnati, Cincinnati, Ohio, United States
  • Patel, Niralee, University of Cincinnati, Cincinnati, Ohio, United States
Introduction

Pauci-immune necrotizing glomerulonephritis (PING), a common cause of rapidly progressive glomerulonephritis, is associated with antineutrophil cytoplasmic antibodies (ANCA); yet up to 10% of cases may be ANCA-negative. We present a case of ANCA-negative PING in a patient with an orthotopic liver transplant (OLT) on immunosuppressants.

Case Description

40-year-old male with history of OLT 4 years ago from congenital cytomegalovirus cirrhosis and well-controlled post-transplant diabetes had an urgent kidney biopsy due to an acute rise in creatinine (Cr) from 2.5 to 3.5 mg/dL, new nephrotic range proteinuria (urine protein Cr ratio 3.8g/g), and microscopic hematuria. His immunosuppression was tacrolimus (FK) and mycophenolate mofetil (MMF). Kidney ultrasound showed small echogenic kidneys. Biopsy (Figure 1) revealed glomeruli with cellular crescents with focal necrotizing lesions with majority of glomeruli with global sclerosis, and 50% interstitial fibrosis and tubular atrophy. Immunofluorescence staining was negative for immunoglobulins, C3, C1q, and light chains. Electron microscopy did not exhibit any deposits. Serological labs, including ANCA, were negative except for a low (1:80) antinuclear antibody. Given active cellular crescents, despite the signs of chronicity, he was treated with 3 days of high dose methylprednisolone followed by prednisone taper and 2 doses of rituximab in 2 weeks, in addition to chronic FK and MMF. 2 months later, renal dysfunction persists.

Discussion

ANCA-negative PING tends to have poorer kidney prognosis, with 25% requiring dialysis at diagnosis and a high mortality compared to ANCA-positive PING. ANCA-negative PING may represent an entirely separate disease and has been associated with malignancy and bacterial infections. Data on the underlying pathophysiology is limited; yet ANCA-negative PING is managed similarly to ANCA-positive cases with corticosteroids, rituximab or cyclophosphamide. The unique de novo presentation of a patient on immunosuppressives suggests there may be an atypical underlying pathophysiology and further investigations are warranted.