Table 1 EULAR/PRINTO/PReS Criteria for Childhood Granulomatosis with Polyangiitis
A patient is said to have GPA when three of the following six criteria are present: | |
|---|---|
Upper Airway Involvement | Chronic purulent or bloody nasal discharge, or recurrent epistaxis/crusts/granulomata Nasal septal perforation or saddle-nose deformity Chronic or recurrent sinus inflammation |
Pulmonary Involvement | Chest X-ray or CT scan showing the presence of nodules, cavities, or fixed infiltrates |
Renal Involvement | Proteinuria > 0.3 g/24H, or greater than 30 umol/mg of urine albumin/creatinine ratio on a spot morning sample Hematuria or red blood cell casts: > 5 red blood cells per high-power field, or red blood cell casts in urinary sediment, or > 2+ on dipstick Necrotizing pauci-immune glomerulonephritis |
Granulomatous Inflammation | Granulomatous inflammation within wall of artery or in perivascular or extravascular area of artery or arteriole |
Laryngotracheobronchial stenosis | Subglottic, tracheal, or bronchial stenosis |
ANCA | ANCA positivity by immunofluorescence or by ELISA (MPO/p or PR3/c ANCA) |