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Abstract: PO1756

Rituximab Rescue in Anti-GBM Nephritis

Session Information

Category: Trainee Case Report

  • 1202 Glomerular Diseases: Immunology and Inflammation

Authors

  • Shailly, Shikha, Nassau University Medical Center, East Meadow, New York, United States
  • Berman, Lorin, Nassau University Medical Center, East Meadow, New York, United States
  • Rubinstein, Sofia, Nassau University Medical Center, East Meadow, New York, United States
Introduction

Anti-GBM nephritis is a rare, severe autoimmune disease. If left untreated or in patients requiring dialysis at presentation, it has a renal survival of 8% at 1 year. Conventional therapy includes corticosteroid, cyclophosphamide and plasmapheresis. An anti-B cell agent, rituximab is more recently being used in refractory cases (defined as no response after 4 weeks of standard therapy).

Case Description

59-year-old female with hypertension presented with 1 month of fever, generalized malaise, and cough following recent travel to Iraq. Laboratory evaluation showed serum creatinine 1.3 mg/dL with hematuria and proteinuria (0.8g/day). ANA, ANCA, RF, Hepatitis B/C, HIV, RPR, and streptozyme panel were negative. Renal and pulmonary imaging were unremarkable. With creatinine rising rapidly, renal biopsy was performed revealing acute focal segmental necrotizing and crescentic glomerulonephritis involving 50% of glomeruli. Anti-GBM antibody level was 8U. Plasmapheresis daily, cyclophosphamide and steroids were initiated. She remained non-oliguric, but developed edema requiring intermittent diuresis. On day 15, plasmapheresis was reduced to every 48 hours. Anti-GBM antibody failed to decline, therefore 1 gr of rituximab infusion was initiated 3 weeks later. Standard therapy was continued until the second dose of 1gr of rituximab 2 weeks later. Patient was discharged with creatinine stable at 4 mg/dL, anti-GBM antibody level at 1.4U and on prednisone taper. On follow up day 75, antibody levels were undetectable and on day 147 she remained dialysis free.

Discussion

Our patient presentated with favorable prognostic markers including non-oliguria, low creatinine and anti-GBM antibody levels, negative ANCA, involvement of 50% of glomeruli, and no dialysis requirement. Despite these factors, she did not respond to standard therapy alone. Our patient was initiated on rituximab earlier than reported refractory cases while continuing standard therapy until the 2nd dose of rituximab, with a favorable outcome of remaining dialysis free. We suggest early use of rituximab with overlapping of the standard regimen is safe and effective in older age group.