2022 ACR/EULAR Classification Criteria for Eosinophilic Granulomatosis with Polyangiitis (EGPA)

The American College of Rheumatology (ACR) and EULAR reported criteria for eosinophilic granulomatosis with polyangiitis (EGPA).

Parameters:

(1) maximum absolute eosinophil count

(2) obstructive airway disease

(3) nasal polyps

(4) c-ANCA or anti-proteinase3-ANCA positivity

(5) extravascular eosinophilic predominant inflammation

(6) mononeuritis multiplex/motor neuropathy not due to radiculopathy

(7) hematuria

Parameter	Finding	Points
max absolute eosinophil count	< 1 * 10^9/L	0
	>= 1 * 10^9/L	5
obstructive airway disease	no	0
	yes	3
nasal polyps	no	0
	yes	3
c-ANCA or anti-proteinase3 ANCA	no	0
	yes	-3
extravascular eosinophilic inflammation	no	0
	yes	2
mononeuritis multiplex/motor neuropathy not radiculopathy	no	0
	yes	1
hematuria	no	0
	yes	-1

total score =

= SUM(points for all of the parameters)

Interpretation:

• minimum score: -4

• maximum score: 14

• A score >= 6 indicates EGPA in a patient with a small/medium vessel vasculitis, with exclusion of vasculitis mimics.

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