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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
  1. ANCA, anti-neutrophil cytoplasmic antibody; WG, Wegener granulomatosis; EULAR, European League Against Rheumatism; PReS, Paediatric Rheumatology European Society; PRINTO, Paediatric Rheumatology International Trials Organisation