2022 ACR/EULAR ANCA-Associated Vasculitis Classification Criteria (GPA / MPA / EGPA)

Computes the 2022 ACR/EULAR weighted classification scores for all three ANCA-associated vasculitides (GPA, MPA, EGPA) at once from clinical, ANCA, imaging, and biopsy findings, for research classification only once small/medium-vessel vasculitis is established and mimics excluded — not a diagnostic test.

Entry gate: diagnosis of small- or medium-vessel vasculitis established AND vasculitis mimics excluded?

ANCA result (IIF pattern and/or antigen-specific ELISA)

Nasal involvement: bloody discharge, ulcers, crusting, congestion, blockage, or septal defect/perforation

Cartilaginous involvement: ear/nose cartilage inflammation, hoarse voice or stridor, endobronchial involvement, or saddle-nose deformity

Conductive or sensorineural hearing loss

Pulmonary nodules, mass, or cavitation on chest imaging

Granuloma, extravascular granulomatous inflammation, or giant cells on biopsy

Inflammation, consolidation, or effusion of nasal/paranasal sinuses, or mastoiditis on imaging

Pauci-immune glomerulonephritis on biopsy

Lung fibrosis or interstitial lung disease on chest imaging

Obstructive airway disease (e.g., asthma)

Nasal polyps

Mononeuritis multiplex / motor neuropathy not due to radiculopathy

Extravascular eosinophilic-predominant inflammation on biopsy

Hematuria

Maximum blood eosinophil count (x10⁹/L; = cells/microliter divided by 1000)