Abstract: SA-PO770
Case Series of Avacopan in Dual-Positive Anti-glomerular Basement Membrane (GBM) and ANCA Disease
Session Information
- ANCA-Associated Vasculitis, Anti-GBM Disease, and Other RPGN
October 26, 2024 | Location: Exhibit Hall, Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Glomerular Diseases
- 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics
Authors
- Floyd, Lauren, Lancashire Teaching Hospitals NHS Foundation Trust, Preston, Lancashire, United Kingdom
- Moore, Louise Rachel, Lancashire Teaching Hospitals NHS Foundation Trust, Preston, Lancashire, United Kingdom
- Sen, Ranjoy, Lancashire Teaching Hospitals NHS Foundation Trust, Preston, Lancashire, United Kingdom
- Dhaygude, Ajay Prabhakar, Lancashire Teaching Hospitals NHS Foundation Trust, Preston, Lancashire, United Kingdom
- McAdoo, Stephen Paul, Imperial College London, London, United Kingdom
- Morris, Adam, Lancashire Teaching Hospitals NHS Foundation Trust, Preston, Lancashire, United Kingdom
Introduction
Anti-glomerular basement membrane(GBM) disease is characterised by rapidly progressive glomerulonephritis(RPGN) & pulmonary haemorrhage & up to 40% can have dual positivity with circulating ANCA antibodies. Treatment include steroids, plasma exchange(PLEX) & cyclophosphamide(CYC). The recent ADVOCATE trial showed Avacopan as a steroid-sparing agent but its effectiveness in other RPGNs including dual positive patients remains uncertain.
Case Description
We present 3 cases of dual positive ANCA & anti-GBM disease treated with Avacopan. The first was a 73yr old lady presenting with severe non-oliguric AKI(eGFR 5ml/min) and dual positivity with MPO-ANCA(Fig1). Renal biopsy showed focal segmental necrotising GN with added tubular injury. She was treated with CYC, rituximab & PLEX. Prior to admission she had a significant GI bleed with erosive gastritis & duodenal ulcers. Consequently, she was managed steroid free, opting for Avacopan as a steroid sparing agent.
Case 2 was a 64yr old male presenting with AKI (eGFR 8ml/min) and dual antibody positive with MPO-ANCA. The biopsy showed fibrocellular & cellular crescents and linear IgG. He was treated with PLEX, CYC, rituximab & a rapidly weaning course of steroids alongside Avacopan(Fig.2)
The final case was a 22yr old lady presenting with myalgia & cough progressing to pulmonary haemorrhage(Fig 3). She was anti-GBM & PR3-ANCA positive with significant anaemia & proteinuria. She received CYC, PLEX, rituximab & Avacopan alongside a 6week tapering course of steroids. She made a good recovered & is in remission with Avacopan as maintenance treatment
Discussion
We demonstrated the role of Avacopan in anti-GBM disease to facilitate rapid steroid weaning, even in those with severe disease. One patient was managed without steroids, a rare approach in the management of anti-GBM disease & all maintained independent renal function. We suggest Avacopan should be considered in the management of patients with dual positive antibodies