Eosinophils from Physiology to Disease: A Comprehensive Review

Table 5

Nosologic classification of EGPA.

Chapel Hill Consensus Conference 2012 definition of EGPA
Eosinophil-rich and necrotizing granulomatous inflammation often involving the respiratory tract, and necrotizing vasculitis predominantly affecting small to medium vessels, and associated with asthma and eosinophilia. ANCA is more frequent when glomerulonephritis is present
Classification and diagnostic criteria
Lanham’s (1984) diagnostic criteria	ACR (1990) classification criteria	MIRRA (2017) classification criteria	GERMOP/ERS (2017) diagnostic criteria
Mandatory criteria; (i) Asthma; (ii) Blood eosinophilia > 1500/mm^3 or >10% of total WBC; (iii) Evidence of vasculitis involving two or more organs	Criteria (at least 4/6 required); (i) Asthma; (ii) Blood eosinophilia > 10% of total WBC; (iii) Neuropathy; (iv) Pulmonary infiltrates nonfixed; (v) Paranasal sinus abnormalities; (vi) Extravascular eosinophils	Mandatory criteria: (i) Asthma; (ii) Eosinophilia (>1.0 × 10^9/L and/or >10% of total blood leucocytes); Minor criteria (at least two required): (i) Positive biopsy showing histopathological evidence of eosinophilic vasculitis, or perivascular eosinophilic infiltration, or eosinophil-rich granulomatous inflammation.; (ii) Neuropathy: either mononeuritis or polyneuropathy demonstrated by a motor deficit or nerve conduction abnormality; (iii) (nonfixed) pulmonary infiltrates; (iv) Sino-nasal abnormality; (v) Cardiomyopathy at echocardiography or cardiac magnetic resonance imaging; (vi) Glomerulonephritis; (vii) Hematuria, red cell casts, proteinuria; (viii) Alveolar haemorrhage, confirmed by bronchoalveolar lavage; (ix) Palpable purpura; (x) Positive ANCA (MPO or PR-3)	Entry criteria for EGPA and HASM; (i) Asthma; (ii) Blood eosinophils ≥ 1.5 × 10^9/l or ≥10% of total WBC; Eosinophilic granulomatosis with polyangiitis (EGPA); Criteria (at least 1/4 required): (i) Definite vasculitis features; (a) Biopsy-proven necrotising vasculitis of any organ; (b) Biopsy-proven necrotising glomerulonephritis or crescentic glomerulonephritis; (c) Alveolar haemorrhage; (d) Palpable purpura; (e) Myocardial infarction due to proven coronaritis; (ii) Definite surrogates of vasculitis; (a) Haematuria associated with red casts or >10% dysmorphic erythrocytes or haematuria and 2+ proteinuria on urine analysis; (b) Leukocytoclastic capillaritis and/or eosinophilic infiltration of the arterial wall at biopsy; (iii) Mononeuritis or mononeuritis multiplex; (iv) ANCA and any systemic manifestation; Hypereosinophilic asthma with systemic manifestations (HASM); Mandatory criteria; (i) Any systemic manifestation other than definite polyangiitis or surrogate of vasculitis or mononeuritis; (ii) Absence of ANCA