From: Monogenic polyarteritis: the lesson of ADA2 deficiency
Histopathology or angiographic abnormalities (mandatory) plus one of the five following criteria: | - Histology: necrotising vasculitis in medium or small-sized arteries. - Angiography: aneurysm, stenosis or occlusion of a medium or small sized artery, | |
EULAR/PRINTO/PRES classification criteria for childhood Polyarteritis nodosa (c-PAN) | 1. Skin involvement | Livedo reticularis, skin nodules, superficial ulcers, peripheral tissue necrosis |
2. Myalgia/muscle tenderness | Muscle pain or tenderness | |
3. Hypertension | Blood pressure > 95th centile | |
4. Peripheral neuropathy | Sensory or motor neuropathy | |
5. Renal involvement | Proteinuria, haematuria, impaired function |