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Kidney Week

Abstract: FR-PO0773

Rapid Recovery from AKI with Plasmapheresis in Crescentic Anti-GBM Glomerulonephritis in a Pediatric Patient

Session Information

Category: Glomerular Diseases

  • 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics

Authors

  • Zhang, William, University of California San Diego, La Jolla, California, United States
  • Nourbakhsh, Noureddin D., University of California San Diego, La Jolla, California, United States
  • Ellington, Natalie, University of California San Diego, La Jolla, California, United States
  • Perens, Elliot, University of California San Diego, La Jolla, California, United States
Introduction

Anti-Glomerular Basement Membrane (GBM) disease is exceedingly rare in children. Accordingly, treatment guidelines remain limited, and the applicability of adult-derived protocols is uncertain. Prompt treatment, however, is critical as adult series demonstrate significantly worse outcomes in patients presenting with advanced kidney failure.

Case Description

A 14-year-old female presented with 4 days of gross hematuria following an upper respiratory illness. Initial serum creatinine was 0.85 mg/dL, urine protein-to-creatinine ratio 6.5 mg/mg, albumin 2.8 g/dL, and normal complement levels. By day 11, creatinine increased to 1.11 mg/dL and kidney biopsy revealed necrotizing glomerulonephritis, cellular crescents in 82% of glomeruli, and linear IgG (3+) staining along the GBM, prompting initiation of high-dose intravenous methylprednisolone. On day 13, serum anti-GBM IgG returned positive (3.0 AI), creatinine peaked at 1.33 mg/dL, and plasmapheresis was initiated. After 8 sessions, creatinine decreased to 0.87 mg/dL. She was also treated with oral cyclophosphamide and prednisone, with further improvement in her creatinine.

Discussion

This case illustrates that adult-derived anti-GBM treatment protocols (plasmapheresis, pulse corticosteroids, and cyclophosphamide) can produce a striking recovery of kidney function in a pediatric patient, even with severe crescentic histology. The favorable outcome is consistent with adult data showing patients treated before dialysis dependence have substantially better kidney survival. Also of note, this patient’s initial presentation—mild AKI without pulmonary involvement—was not immediately suggestive of crescentic anti-GBM disease. Although the rarity of pediatric anti-GBM may not justify routine GBM antibody screening, the combination of this patient’s mild presentation and the effectiveness of early, intense intervention supports a low threshold for anti-GBM testing and/or kidney biopsy in children with normal complement glomerulonephritis and unexplained AKI or significant proteinuria.