Final EULAR/PRINTO/PRES c-WG criteria (with glossary) and classification definition (sample 939)
| Criterion | Glossary | Sensitivity (%) | Specificity (%) | AUC (%) |
|---|---|---|---|---|
| 1. Histopathology | Granulomatous inflammation within the wall of an artery or in the perivascular or extravascular area | 54 | 99.6 | 76.8 |
| 2. Upper airway involvement | Chronic purulent or bloody nasal discharge or recurrent epistaxis/crusts/granulomata | 83 | 99 | 91 |
| Nasal septum perforation or saddle nose deformity | ||||
| Chronic or recurrent sinus inflammation | ||||
| 3. Laryngo-tracheo-bronchial involvement | Subglottic, tracheal or bronchial stenoses | 22 | 99.8 | 60.7 |
| 4. Pulmonary involvement | Chest x-ray or CT showing the presence of nodules, cavities or fixed infiltrates | 78 | 92 | 85.2 |
| 5. ANCA | ANCA positivity by immunofluorescence or by ELISA (MPO/p or PR3/c ANCA) | 93 | 90 | 91.7 |
| 6. Renal involvement | Proteinuria >0.3 g/24 h or >30 mmol/mg of urine albumin/creatinine ratio on a spot morning sample | 65 | 69.6 | 67.3 |
| Haematuria or red blood cell casts: >5 red blood cells/high power field or red blood cells casts in the urinary sediment or ≥2+ on dipstick | ||||
| Necrotising pauci-immune glomerulonephritis | ||||
| c-WG EULAR/PRINTO/PRES Ankara 2008 classification definition: κ 0.90 (95% CI 0.84 to 0.97) | At least three of the six following criteria: | 93.3 | 99.2 | 96.3 |
| Histopathology | ||||
| Upper airway involvement | ||||
| Laryngo-tracheo-bronchial stenoses | ||||
| Pulmonary involvement | ||||
| ANCA positivity | ||||
| Renal involvement |
ANCA, antineutrophilic cytoplasmic antibody; AUC, area under the curve; c-WG, c-Wegener granulomatosis; EULAR, European League Against Rheumatism; PRES, Paediatric Rheumatology European Society, PRINTO, Paediatric Rheumatology International Trials Organisation.