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Rheumatology

GPA

Classification

2022 American College of Rheumatology/European Alliance of Associations for Rheumatology Classification Criteria for Eosinophilic Granulomatosis with Polyangiitis

Clinical criteria

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

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Cartilaginous involvement (inflammation of ear or nose cartilage, hoarse voice or stridor, endobronchial involvement, or saddle nose deformity)

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Conductive or sensorineural hearing loss

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Laboratory and biopsy criteria

Positive test for cytoplasmic antineutrophil cytoplasmic antibodies (cANCA) or antiproteinase 3 (anti-PR3) antibodies

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Pulmonary nodules, mass, or cavitation on chest imaging

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Granuloma, extravascular granulomatous inflammation, or giant cells on biopsy

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

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Pauci-immune glomerulonephritis on biopsy

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Positive test for perinuclear antineutrophil cytoplasmic antibodies (pANCA) or antimyeloperoxidase (anti-MPO) antibodies

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Blood eosinophil count ≥ 1 x109/litre

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Considerations

The tool is for educational use only, is not meant to be a substitute for professional advice and should not be used for medical diagnosis and/or medical treatment.

  • These classification criteria should be applied to classify a patient as having granulomatosis with polyangiitis when a diagnosis of small- or medium-vessel vasculitis has been made
  • Alternate diagnoses mimicking vasculitis should be excluded prior to applying the criteria
Reference

Robson JC, Grayson PC, Ponte C, et al 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for granulomatosis with polyangiitis Annals of the Rheumatic Diseases Published Online First: 02 February 2022. doi: 10.1136/annrheumdis-2021-221795

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