EULAR/PRINTO/PRES paediatric criteria - ≥3 of 6 features classify GPA.
The Granulomatosis with Polyangiitis (Wegener) is a rheumatology scoring tool used in paediatric practice. EULAR/PRINTO/PRES paediatric criteria - ≥3 of 6 features classify GPA. It scores 6 criteria and interprets the total across 2 bands, so the number always arrives with the severity it implies rather than on its own. In PediAid each criterion is shown as a labelled option with the weight it carries, so the total can be checked rather than taken on trust.
6. They are Histopathology - granulomatous inflammation within the vessel wall or peri/extravascular area; Upper airway involvement - nasal discharge, recurrent epistaxis, crusts, sinus inflammation, subglottic/tracheal stenosis; Laryngo-tracheo-bronchial stenosis; Pulmonary involvement - chest X-ray or CT showing nodules, cavities or fixed infiltrates; ANCA positivity (by immunofluorescence or ELISA - MPO/p-ANCA or PR3/c-ANCA); Renal involvement - proteinuria, haematuria/red-cell casts, necrotising pauci-immune glomerulonephritis.
The total is interpreted as: any score - Criteria not met: fewer than 3 criteria - does not classify as childhood GPA; 3 criteria or more - Criteria MET: ≥3 of 6 criteria - classifies as childhood granulomatosis with polyangiitis (sensitivity 93%, specificity 99%). Induction with corticosteroids plus rituximab or cyclophosphamide.
No. It is a structured way to record and communicate severity. A clinician concerned about a child should act on that concern regardless of the total.
Source: Ozen S et al. EULAR/PRINTO/PRES Ankara 2008 criteria. Ann Rheum Dis 2010;69:798-806.
Yes. Score definitions are bundled with the PediAid app, so the score computes without a connection.