4A44.A1
Granulomatosis with polyangiitis
Granulomatose com poliangiíte
CategoryDefinition
Necrotizing granulomatous inflammation usually involving the upper and lower respiratory tract, and necrotizing vasculitis affecting predominantly small to medium-sized vessels (e.g., capillaries, venules, arterioles, arteries and veins). Necrotizing glomerulonephritis is common. (Arthritis Rheum 1990;33:1101-1107)
Diagnostic Criteria
At least three out of six of the following criteria:
1. histopathology (granulomatous inflammation within the wall of an artery or in the peri-/extra-vascular area)
2. upper airway involvement
3. laryngo-tracheo-bronchial stenoses
4. pulmonary involvement
5. ANCA positivity
6. renal involvement
Inclusions
- Wegener granulomatosis
Index Terms
Granulomatosis with polyangiitisWegener granulomatosisNecrotising respiratory granulomatosis