Criteria

2012 revised International Chapel Hill Consensus Conference nomenclature of vasculitides[36]

Granulomatosis with polyangiitis (GPA) is defined by the 2012 International Chapel Hill Consensus Conference as necrotising granulomatous inflammation usually involving the upper and lower respiratory tract, and necrotising vasculitis affecting predominantly small to medium vessels (e.g., capillaries, venules, arterioles, arteries, and veins). Necrotising glomerulonephritis is common.

2022 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for eosinophilic granulomatosis with polyangiitis[21]

These classification criteria should be applied to classify a patient as having GPA when a diagnosis of small or medium vessel vasculitis has been made. Alternative diagnoses mimicking vasculitis should be excluded prior to applying this criteria.

Clinical criteria

  • Nasal involvement: bloody discharge, ulcers, crusting, congestion, blockage, or septal defect/perforation +3 points

  • Cartilaginous involvement (inflammation of ear or nose cartilage, hoarse voice or stridor, endobronchial involvement, or saddle nose deformity) +2 points

  • Conductive or sensorineural hearing loss +1 point

Laboratory, imaging, and biopsy criteria

  • Positive test for cytoplasmic anti-neutrophil cytoplasmic antibodies (cANCA) or anti-proteinase 3 (anti-PR3) antibodies +5 points

  • Pulmonary nodules, mass, or cavitation on chest imaging +2 points

  • Granuloma, extravascular granulomatous inflammation, or giant cells on biopsy +2 points

  • Inflammation, consolidation, or effusion of the nasal/paranasal sinuses or mastoiditis on imaging +1 point

  • Pauci-immune glomerulonephritis on biopsy +1 point

  • Positive test for peri-nuclear anti-neutrophil cytoplasmic antibodies (pANCA) or anti-myeloperoxidase (anti-MPO) antibodies -1 point

  • Blood eosinophil count ≥ 1 x 10⁹/L -4 points

A score of ≥5 is needed for a classification of GPA.

2021 American College of Rheumatology/Vasculitis Foundation guideline for the management of anti-neutrophil cytoplasmic antibody-associated vasculitis​[37]​

This guideline defines levels of disease activity and disease severity.

  • Active disease: new, persistent, or worsening clinical signs and/or symptoms attributed to GPA and not related to prior damage.

  • Severe disease: vasculitis with life- or organ-threatening manifestations (e.g., alveolar haemorrhage, glomerulonephritis, central nervous system vasculitis, mononeuritis multiplex, cardiac involvement, mesenteric ischaemia, limb/digit ischaemia).

  • Non-severe disease: vasculitis without life- or organ-threatening manifestations (e.g., rhinosinusitis, asthma, mild systemic symptoms, uncomplicated cutaneous disease, mild inflammatory arthritis).

  • Remission: absence of clinical signs or symptoms attributed to GPA, on or off immunosuppressive therapy.

  • Refractory disease: persistent active disease despite an appropriate course of immunosuppressive therapy.

  • Relapse: recurrence of active disease following a period of remission.

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