Lung involvement in childhood onset granulomatosis with polyangiitis

Table 1 EULAR/PRINTO/PRES criteria for childhood granulomatosis with polyangiitis [6]

1. Histopathology	Granulomatous inflammation within the wall of an artery or in the perivascular or extravascular area
2. 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
3. Laryngo-tracheo-bronchial involvement	Subglottic, tracheal or bronchial stenosis
4. Pulmonary involvement	Chest x-ray or CT showing the presence of nodules, cavities or fixed infiltrates
5. ANCA	ANCA positivity by immunofluorescence or by ELISA (MPO/p or PR3/c ANCA)
6. Renal involvement	Proteinuria >0.3 g/24 h 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 cells casts in the urinary sediment or ≥2+ on dipstick
	Necrotising pauci-immune glomerulonephritis

The presence of at least three of the six criteria has a sensitivity of 93.3% and a specificity of 99.2% in confirming the diagnosis

ISSN: 1546-0096