Description

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.


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