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Abstract: SA-PO738

Avacopan for ANCA-Associated Vasculitis in Ireland: A Report on All Patients Who Received Avacopan in the Irish Health Care System

Session Information

Category: Glomerular Diseases

  • 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics

Authors

  • Nic an Riogh, Eithne Muireann, Trinity Translational Medicine Institute, Trinity College Dublin, Dublin, Ireland
  • Cummiskey, Andy Gerard, Mater Misericordiae University Hospital, Dublin, Ireland
  • Stoneman, Sinead, Cork University Hospital, Cork, Cork, Ireland
  • Clarkson, Michael, Cork University Hospital, Cork, Cork, Ireland
  • Moran, Sarah Margaret, Cork University Hospital, Cork, Cork, Ireland
  • Magee, Ciara N., Beaumont Hospital, Dublin, Dublin, Ireland
  • McAnallen, Susan Marie, St James's Hospital, Dublin, Ireland
  • Little, Mark Alan, Trinity Translational Medicine Institute, Trinity College Dublin, Dublin, Ireland
Background

Avacopan is a selective C5a receptor antagonist that inhibits neutrophil chemoattraction and activation. It is indicated for the treatment of severe, active granulomatosis polyangiitis or microscopic polyangiitis in combination with rituximab or cyclophosphamide and is considered a steroid sparing alternative. The aim of this report is to describe the first clinical experience with avacopan in ANCA Associated Vasculitis (AAV) in Ireland.

Methods

To date ten patients have received avacopan for AAV between December 2017 and April 2024 through the Irish early access programme and ADVOCATE trial. For these patients, disease relevant information was extracted from the RITA Ireland Vasculitis (RIV) database and through collaboration with the treating nephrologists. Data collected included commencement date and indication for avacopan initiation, duration of treatment, ANCA subtype, organ involvement, number of flares, immunosuppression medication usage and adverse events experienced.

Results

The average age of patients receiving avacopan in Ireland is 51 years (range 18-69 years). 50% were male and 50% were female. Six patients were MPO positive and four were PR3 positive. The majority of patients had renal involvement (90%). Avacopan was predominately commenced in cases where steroid sparing regimens were sought, e.g. severe osteoporosis, history of steroid induced psychosis, patient preference not to take steroids. Avacopan treatment duration ranged from 1 to 20 months, with an average duration of 4 months. 50% of patients remain on avacopan. On average patients received a total of 2.37 gm of Methylprednisolone prior to avacopan commencement (dose range 0mg to 6.5gm). While most patients tolerated avacopan and had no adverse events, reported side effects in this Irish cohort included headache, limited maculopapular rash as well as neutropenia and persistent liver dysfunction.

Conclusion

This is the first report of real-world experience of all patients who have received avacopan in Ireland. Two minor relapses and no major relpases have been reported in this cohort. Although the number of patients receiving avacopan in Ireland is small, avacopan appears to have a good safety profile in this population.