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Kidney Week

Abstract: FR-PO0780

Dialysis-Dependence in Dual-Positive Anti-GBM/Myeloperoxidase (MPO)-ANCA Crescentic Glomerulonephritis Despite Early Aggressive Therapy

Session Information

Category: Glomerular Diseases

  • 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics

Authors

  • Hameed, Haya, Marshall University, Huntington, West Virginia, United States
  • Kheetan, Murad, Marshall University, Huntington, West Virginia, United States
  • Al-Baqain, Khaled, Marshall University, Huntington, West Virginia, United States
Introduction

Dual-positive anti-GBM disease and MPO-ANCA-associated vasculitis is an increasingly recognized overlap syndrome characterized by rapidly progressive glomerulonephritis. Recent studies suggest that concurrent anti-GBM and MPO-ANCA positivity may occur more frequently than previously appreciated. However, management of dual-positive disease remains a therapeutic challenge.Some studies suggest that standardized aggressive induction therapy may result in improved renal recovery compared with isolated anti-GBM disease.

Case Description

A 76-year-old female with hypertension and rheumatoid arthritis presented with severe acute kidney injury (serum creatinine 7.0 mg/dL, baseline 1.1 mg/dL), hyperkalemia ,and active urine sediment with hematuria and proteinuria (urine protein-to-creatinine ratio 1.1 g/g).

Renal biopsy showed diffuse active crescentic glomerulonephritis with diffuse linear IgG staining along the glomerular basement membrane,80% of glomeruli showed global glomerulosclerosis with predominantly fibrous crescents and a small fibrocellular crescent, along with diffuse interstitial inflammation. Serologic testing was positive for MPO-ANCA.

The patient was initiated on pulse-dose corticosteroids, plasma exchange, and cyclophosphamide.Despite early initiation of therapy, renal function continued to worsen. On hospital day 3 , she developed altered mental status attributed to uremia and was started on hemodialysis with concurrent continuation of therapy.
Following completion of seven plasma exchange sessions, anti-GBM antibody titers had become undetectable and MPO-ANCA titers decreased to 2.5 AU/ml. However, renal recovery was not achieved, and the patient remained dialysis-dependent at discharge.

Discussion

Renal biopsy demonstrated extensive chronic injury with 80% global glomerulosclerosis. Nevertheless, the decision to treat was made to attempt salvage of residual kidney function, consistent with KDIGO guidance that induction treatment should not be withheld solely on the basis of unfavorable histologic findings.
Despite successful clearance of circulating anti-GBM antibodies, the patient remained dialysis-dependent. This highlights the therapeutic dilemma in dual-positive disease and raises questions regarding predictors of renal recovery and the potential limitations of treatment once advanced kidney injury has occurred.