ANCA Vasculitis
ANCA vasculitis (GPA, MPA, EGPA): small-vessel necrotizing vasculitis; anti-PR3 or anti-MPO IgG primes neutrophils via C5a/C5aR1 → NET formation → endothelial injury. Cyclophosphamide/rituximab induction; avacopan (steroid-sparing; FDA Oct 2021) and rituximab maintenance.
Entry Metadata
| Field | Value |
|---|---|
| ID | anca-vasculitis |
| Name | ANCA Vasculitis |
| Status | draft |
| Last reviewed | 2026-06-07 |
| Atlas | 01-human |
| Scale | 07-system |
Cross-Atlas Connections
Sources
- Jayne DRW, Merkel PA, Schall TJ, Bekker P. Avacopan for the Treatment of ANCA-Associated Vasculitis. N Engl J Med. 2021;384(7):599-609. · PubMed 33596356
- Stone JH, Merkel PA, Spiera R, et al. Rituximab versus cyclophosphamide for ANCA-associated vasculitis. N Engl J Med. 2010;363(3):221-232. · PubMed 20647199
- Charles P, Terrier B, Perrodeau É, et al. Comparison of individually tailored versus fixed-schedule rituximab regimen to maintain ANCA-associated vasculitis remission. Ann Rheum Dis. 2018;77(8):1143-1149. · PubMed 29549154
- Yates M, Watts RA, Bajema IM, et al. EULAR/ERA-EDTA recommendations for the management of ANCA-associated vasculitis. Ann Rheum Dis. 2016;75(9):1583-1594. · PubMed 27338776