Table 3

Final EULAR/PRINTO/PRES c-WG criteria (with glossary) and classification definition (sample 939)

CriterionGlossarySensitivity (%)Specificity (%)AUC (%)
 1. HistopathologyGranulomatous inflammation within the wall of an artery or in the perivascular or extravascular area5499.676.8
 2. Upper airway involvementChronic purulent or bloody nasal discharge or recurrent epistaxis/crusts/granulomata839991
Nasal septum perforation or saddle nose deformity
Chronic or recurrent sinus inflammation
 3. Laryngo-tracheo-bronchial involvementSubglottic, tracheal or bronchial stenoses2299.860.7
 4. Pulmonary involvementChest x-ray or CT showing the presence of nodules, cavities or fixed infiltrates789285.2
 5. ANCAANCA positivity by immunofluorescence or by ELISA (MPO/p or PR3/c ANCA)939091.7
 6. Renal involvementProteinuria >0.3 g/24 h or >30 mmol/mg of urine albumin/creatinine ratio on a spot morning sample6569.667.3
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:93.399.296.3
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.