The American College of Rheumatology (ACR) and EULAR reported criteria for eosinophilic granulomatosis with polyangiitis (GPA).
Parameters:
(1) bloody nasal discharge, nasal crusting or sino-nasal congestion
(2) cartilaginous involvement
(3) conductive or sensorineural hearing loss
(4) cANCA or anti-proteinase3-ANCA positivity
(5) pulmonary nodules, mass or cavitation on chest imaging
(6) granuloma or giant cells on biopsy
(7) inflammation or consolidation of the nasal/paranasal sinuses on imaging
(8) pauci-immune glomerulonephritis
(9) p-ANCA or antimyeloperoxidase ANCA activity
(10) absolute eosinophil count
|
Parameter |
Finding |
Points |
|
bloody nasal discharge, crusting, congestion |
no |
0 |
|
|
yes |
3 |
|
cartilaginous involvement |
no |
0 |
|
|
yes |
2 |
|
hearing loss |
no |
0 |
|
|
yes |
1 |
|
pulmonary nodules/mass/cavitation |
no |
0 |
|
|
yes |
2 |
|
c-ANCA or anti-proteinase3 ANCA |
no |
0 |
|
|
yes |
5 |
|
p-ANCA or anti-myeloperoxidase ANCA |
no |
0 |
|
|
yes |
-1 |
|
granuloma or giant cells on biopsy |
no |
0 |
|
|
yes |
2 |
|
inflammation nasal/paranasal sinuses |
no |
0 |
|
|
yes |
1 |
|
pauci-immune glomerulonephritis |
no |
0 |
|
|
yes |
1 |
|
max absolute eosinophil count |
< 1 * 10^9/L |
0 |
|
|
>= 1 * 10^9/L |
-4 |
total score =
= SUM(points for all of the parameters)
Interpretation:
• minimum score: -5
• maximum score: 17
• A score >= 5 indicates GPA in a patient with a small/medium vessel vasculitis, with exclusion of vasculitis mimics.
Performance:
• In the validation cohort the sensitivity was 93% and specificity 94%.
Specialty: Immunology/Rheumatology
