Table 2 Final EULAR/PRINTO/PReS criteria for granulomatosis with polyangiitis (GPA, Wegener’s) [8]
From: ANCA-associated vasculitis in childhood: recent advances
Criterion | Glossary | Sensitivity (%) | Specificity (%) |
|---|---|---|---|
1-Histopathology | Granulomatous inflammation within the wall of an artery or in perivascular or extravascular area | 54 | 99.6 |
2-Upper airway involvement | Chronic purulent or bloody nasal discharge or recurrent epistaxis/crusts/granulomata Nasal septum perforation o saddle nose deformity Chronic or recurrent sinus inflammation | 83 | 99 |
3-Laryngo-tracheo-bronchial involvement | Subglottic, tracheal or bronchial stenoses | 22 | 99.8 |
4-Pulmonary involvement | Chest x-ray or CT showing the presence of nodules, cavities or fixed infiltrates | 78 | 92 |
5-ANCA | ANCA positivity by immunofluorescence or by ELISA (MPO/p or PR3/c ANCA) | 93 | 90 |
6-Renal involvement | Proteinuria >0.3 g/24 ore or > 30 mmol/mg of urine albumin/creatinine ratio on a spot morning sample Haematuria or red blood cell casts: >5 red blood cells/high power field or red blood cell casts in the urinary sediment or ≥2+ on dipstick Necrotising pauci-immune glomerulonephritis | 65 | 69.6 |
Childhood-WG EULAR/PRINTO/PRES Ankara 2008 classification | At least three of the six following criteria: Histopathology Upper airway involvement Laryngo-tracheo-bronchial stenosis Pulmonary involvement ANCA positivity Renal involvement | 93.3 | 99.2 |