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Abstract: SA-PO0869

Personalized Approach to Antineutrophil Cytoplasmic Antibody-Associated Vasculitis Leading to Rapidly Progressive Glomerulonephritis Treatment with Glucocorticoids, Plasma Exchange, and Hemodialysis

Session Information

Category: Glomerular Diseases

  • 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics

Authors

  • Soto, Marquise, Massachusetts General Hospital, Boston, Massachusetts, United States
  • Zoeb, Shabnam, Stony Brook University Hospital, Stony Brook, New York, United States
  • Daccueil, Farah, Stony Brook University Hospital, Stony Brook, New York, United States
Introduction

ANCA-associated vasculitis is an autoimmune condition causing inflammation in small vessels that can lead to end-organ damage. First line treatment is combination of high dose glucocorticoids with rituximab or cyclophosphamide. We present an approach to managing RPGN in the setting of active infection.

Case Description

A 50-year-old woman with a past medical history of untreated hepatitis C infection presented with fever, abdominal pain, hematuria, oliguria, and lower extremity edema. Vitals were significant for hypotension, with physical exam concerning right lower extremity cellulitis. Laboratory results of BUN 64, Cr 4.8, Fena 2.2%, urine protein to Creatinine ratio 2.4 g, myeloperoxidase IgG 106 AU, ANCA titer 1:640, and positive lupus anticoagulant. Kidney biopsy revealed proliferative glomerulonephritis with crescents. Patient started on high-dose glucocorticoids. Creatinine peaked at 6.03 and the patient became uremic. The decision was made to start with plasma exchange and hemodialysis in setting of active infection. Upon discharge creatinine stabilized at 2.58. Patient continued 60mg prednisone with goal to taper outpatient and begin treatment with rituximab.

Discussion

First-line therapy for the induction of ANCA-RPGN was not pursued in this case. This case emphasizes the use of plasma exchange in conjunction with steroids without immunosuppressant in the setting of infection. Our patient met criteria for plasma exchange with a serum creatinine ≥ 5.7 mg/dL and risk for progression to end stage renal disease. This case may provide support/guidance on the utility of using Plasmapheresis and steroids in the treatment of RPGN in cases where there may be concerns about starting immunosuppressive therapy with rituximab or cyclophosphamide.

Digital Object Identifier (DOI)