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ACR/EULAR classification criteria for granulomatosis with polyangiitis

Entry criteria
Evidence of small- or medium-vessel vasculitis on imaging
Alternative diagnoses mimicking vasculitis are excluded
Clinical criteria
Nasal involvement: bloody discharge, ulcers, crusting, congestion, blockage, or septal defect/perforation
Cartilaginous involvement: inflammation of ear or nose cartilage, hoarse voice or stridor, endobronchial involvement, or saddle nose deformity
Conductive or sensorineural hearing loss
Laboratory, imaging, and biopsy criteria
Positive test for c-ANCA or anti-PR3 antibodies
Pulmonary nodules, mass, or cavitation on chest imaging
Granuloma, extravascular granulomatous inflammation, or giant cells on biopsy
Inflammation, consolidation, or effusion of the nasal/paranasal sinuses, or mastoiditis on imaging
Pauci-immune glomerulonephritis on biopsy
Positive test for p-ANCA or anti-MPO antibodies
Blood eosinophil count ≥ 1×10⁹/L
Diagnostic criteria are not met
Reference
Joanna C Robson, Peter C Grayson, Cristina Ponte et al. 2022 American College of Rheumatology / European Alliance of Associations for Rheumatology classification criteria for granulomatosis with polyangiitis. Ann Rheum Dis. 2022 Mar;81(3):315-320.
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