EULAR/PReS issued criteria for the diagnosis of vasculitides in pediatric patients. Criteria for Wegener's granulomatosis are a modification of the ACR criteria.
Patient selection: pediatric patient with vasculitis
Distribution: predominantly small vessels
Criteria:
(1) abnormal urinalysis (hematuria and/or significant proteinuria)
(2) granulomatous inflammation on biopsy
(2a) renal biopsy showing necrotizing pauci-immune glomerulonephritis
(3) inflammation of the nasal sinuses
(4) subglottic, tracheal or endobronchial stenosis
(5) abnormal chest imaging study (X-ray, CT)
(6) PR3 ANCA or C-ANCA staining
A diagnosis of Wegener's granulomatosis requires 3 or more of these criteria to be present.
where:
• Items 1, 3 and 5 seem to be somewhat nonspecific.
• Granulomatous inflammation as a term could be refined.