Comparison of the American College of Rheumatology (ACR)13 and revised European League Against Rheumatism (EULAR)/Paediatric Rheumatology International Trials Organisation (PRINTO)/Paediatric Rheumatology European Society (PRES)11 classification criteria for granulomatosis with polyangiitis (GPA, formerly Wegener granulomatosis). Numbers describe patients in the ARChiVe cohort of 155 patients with ANCA-associated vasculitis who fulfilled criteria for GPA according to ACR or EULAR/PRINTO/PRES and patients meeting any individual criterion within these 2 subgroups.
| n | |
|---|---|
| 1990 ACR classification criteria | |
| A patient has GPA when 2 of the following 4 criteria are present: | 87 |
| Nasal or oral inflammation: Ulcers or purulent or bloody nasal discharge | 56 |
| Abnormal chest radiograph: Nodules, fixed infiltrates, or cavities | 70 |
| Abnormal urinary sediment: Microhematuria or red blood cell casts | 68 |
| Granulomatous inflammation on biopsy: Intra/peri/extravascular | 17 |
| 2008 EULAR/PRINTO/PRES classification criteria | |
| A patient has GPA when 3 of the following 6 criteria are present: | 98 |
| Upper airway involvement: Chronic purulent or bloody nasal discharge, or recurrent epistaxis/crusts/granulomata; nasal septum perforation or saddle nose deformity; chronic or recurrent sinus inflammation | 80 |
| Pulmonary involvement: Abnormal chest radiograph or chest computed tomography scan showing nodules, cavities, or fixed infiltrates | 72 |
| Renal involvement: Proteinuria, hematuria or red blood cell casts in the urinary sediment; or necrotizing pauciimmune glomerulonephritis | 79 |
| Laryngo-tracheobronchial involvement: Subglottic, tracheal, or bronchial stenosis | 20 |
| Granulomatous inflammation on biopsy: Intra/peri/extravascular | 17 |
| ANCA positivity: MPO/p or PR3/c ANCA | 94 |
ANCA: antineutrophil cytoplasmic antibody; MPO/p: myeloperoxidase and/or perinuclear; PR3/c: proteinase 3 and/or cytoplasmic.