ASN's Mission

To create a world without kidney diseases, the ASN Alliance for Kidney Health elevates care by educating and informing, driving breakthroughs and innovation, and advocating for policies that create transformative changes in kidney medicine throughout the world.

learn more

Contact ASN

1401 H St, NW, Ste 900, Washington, DC 20005

email@asn-online.org

202-640-4660

The Latest on Twitter

Kidney Week

Abstract: FR-PO674

From ESRD to CKD5: An Unlikely Path for an Anti-Glomerular Basement Membrane (Anti-GBM) Patient

Session Information

Category: Glomerular Diseases

  • 1303 Glomerular Diseases: Clinical‚ Outcomes‚ and Trials

Authors

  • Middleman, Christopher F., Walter Reed National Military Medical Center, Bethesda, Maryland, United States
  • Watson, Maura A., Walter Reed National Military Medical Center, Bethesda, Maryland, United States
Introduction

Anti-GBM is a rare small vessel vasculitis with estimated fewer than two cases per one million population. The disease is caused by circulating antibodies directed against the glomerular basement membrane and alveolar basement membrane, leading to glomerulonephritis (GN) and/or alveolar hemorrhage.

Case Description

A 60 year old female was admitted for acute renal failure with a rapidly rising serum creatinine. Lab work-up revealed a highly elevated anti-GBM titer. Kidney biopsy revealed 100% crescentic disease with 15% interstitial fibrosis and tubular atrophy due to anti-GBM. Plasmapheresis plus immunosuppressive therapy (cyclophosphamide and steroids) vs. IV steroids followed by oral taper were considered. Given her biopsy findings conveyed low likelihood of renal recovery the patient elected treatment with IV, followed by oral, steroids and initiated chronic dialysis May 2020. Her anti-GBM antibody activity was monitored monthly (peak of 153 units May 2020) and down-trended over time. She reported increased urine output in early summer 2021 and 24 hour urine studies, completed between dialysis sessions, revealed an up-trending creatinine clearance. Her dialysis needs decreased to twice-weekly and she was ultimately liberated from dialysis entirely 16 months after initiation.

Discussion

Necrotizing anti-GBM associated GN with 100% crescent formation carries a poor renal prognosis and it is not unreasonable to forgo immunosuppressive therapy and plasmapheresis, as risks of these therapies often outweigh potential benefits. This case illustrates a patient who, not having received PLEX or cyclophosphamide, eventually recovered enough renal function to become a dialysis-free CKD5 patient. Other such case reports exist in literature and beckon us to ask: should we change our perspective of crescentic disease in anti-GBM and more strongly consider available therapies? Further research is needed to determine optimal treatment in anti-GBM patients with 100% crescent formation on biopsy.

The views expressed in this Abstract are those of the authors and do not reflect the official policy of the Department of the Army/Navy/Air Force, the Department of Defense, or the United States government