Table 1.

Final EULAR/PRINTO/PRES childhood GPA criteria.

Criteria	Description	Sensitivity (%)	Specificity (%)
A PATIENT IS SAID TO HAVE GPA WHEN THREE OF THE FOLLOWING SIX CRITERIA ARE PRESENT:
Histopathology	Granulomatous inflammation within the wall of an artery or in the perivascular or extravascular area	54	99.6
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	83	99
Laryngo-tracheo-bronchial involvement	Subglottic, tracheal, or bronchial stenosis	22	99.8
Pulmonary involvement	Chest x-ray or CT showing the presence of nodules, cavities or fixed infiltrates	78	92
ANCA	ANCA positivity by immunofluorescence or by ELISA (MPO/p or PR3/c ANCA)	93	90
Renal	Proteinuria >0.3 g/24 h or >30 mmol/mg of urine albumin/creatinine ratio on a spot morning sample Hematuria 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 Necrotizing pauci-immune glomerulonephritis	93.2	99.2