What are the BVAS disease activity categories?

BVAS v3 scores 0-63; low activity 1-5 (minor relapse); moderate 6-11; severe ≥12 (major relapse/new onset); remission = BVAS 0 for ≥24 weeks on prednisolone ≤7.5 mg/day; VDI (Vasculitis Damage Index) measures irreversible damage.

When is rituximab preferred over cyclophosphamide for AAV?

RAVE and RITUXVAS trials: rituximab non-inferior to CYC for induction of remission; rituximab preferred for: relapsing AAV (superior to CYC), PR3-ANCA (higher remission rate), young patients (preserves fertility), prior bladder malignancy.

What is the induction protocol for severe ANCA vasculitis?

Rituximab 375 mg/m² weekly ×4 or 1g ×2 + high-dose prednisolone; or cyclophosphamide 15 mg/kg q3 weeks IV (renal dose-adjusted) + prednisolone; plasma exchange for severe renal involvement (creatinine >500 μmol/L) per PEXIVAS trial (no benefit on outcomes despite guideline inclusion).

What monitoring is required during AAV remission maintenance?

ANCA titers (PR3 or MPO) every 3-6 months; rising PR3-ANCA highly predictive of relapse; MPO-ANCA less predictive; serial urine PCR; creatinine; BVAS at every visit; consider rituximab retreatment if ANCA rises or CD19 B cells repopulate.

What eGPA features indicate poor prognosis?

Five-factor score (FFS) for eosinophilic GPA: cardiac involvement, GI involvement, renal insufficiency, CNS involvement, and absence of ENT features; FFS 0: <10% mortality; FFS 1: ~26%; FFS ≥2: ~46%; cardiac involvement is leading cause of death in eGPA.

BVAS (Birmingham Vasculitis Activity Score) Calculator

Calculates BVAS v3 for ANCA-associated vasculitis (GPA/MPA/eGPA) across 9 organ systems to assess disease activity and guide rituximab/cyclophosphamide induction and maintenance therapy decisions.

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