Vasculitis & polymyalgia rheumatica
Avacopan in a compassionate use programme for severe anti-neutrophil cytoplasmic antibody-associated vasculitis in Sweden
Authors' conclusion
The use of AVAC in a case series with mainly GPA patients led to rapid clinical improvement, reduction of corticosteroid doses, and improvement in fatigue.
Study context
- Population
- Patients with severe AAV in a compassionate use programme in Sweden; 13 (81%) granulomatosis with polyangiitis (GPA), 3 (19%) MPA; mean age 51 (range 16–74) years
- Participants
- 16 patients
- Exposure
- Baseline vs 6 months on avacopan
- AVACn = 16
- Outcome
- BVAS score, median (IQR)
- Follow-up
- 6 months
Methodological detailscompassionate use programme
- Compassionate use programme
Main finding
As reported in the abstract
Birmingham Vasculitis Activity Score (BVAS) at baseline, median (IQR)
10
3.25–13.25
BVAS at 6 months, median (IQR)
0
0–0
Observational data: associations, not effects.
Further findings
| Outcome | Mean difference | P value |
|---|---|---|
| eGFR in renal AAV (n = 10), median (IQR) · 6 months6 months vs Baseline · increased from 50 (13-75) to 58 (15.5-88) | P = 0.055 | |
| MAF fatigue score · 6 months6 months vs Baseline · MAF score decreased | 6.65 | P = 0.05 |
- Prednisolone dose, median (IQR) · 6 months — Discontinued prednisolone · 6 months vs Baseline · tapered from 25 (20-60) to 2.5 (0-5) mg/day
- 8 · P = 0.0001
- eGFR in renal AAV (n = 10), median (IQR) — Progressed to end-stage kidney disease
- 1
- eGFR in renal AAV (n = 10), median (IQR) — Able to discontinue haemodialysis
- 1
Safety
- Serious adverse events in five of the 16 patients
- 31.3%
Original publication
Avacopan in a compassionate use programme for severe anti-neutrophil cytoplasmic antibody-associated vasculitis in Sweden. Scand J Rheumatol. Published 22 May 2026. doi:10.
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