Table 2

Final EULAR/PRINTO/PRES c-PAN criteria (with glossary) and classification definition (sample 1099)

CriterionGlossarySensitivity (%)Specificity (%)AUC (%)
A systemic inflammatory disease characterised by:
Histopathology Evidence of necrotising vasculitis in medium or small sized arteries84.899.792.3
Angiographic abnormalities Angiography showing aneurysm, stenoses or occlusion of a medium or small sized artery, not due to fibromuscular dysplasia, or other non-inflammatory causes.81.099.990.5
Conventional angiography is the preferred imaging modality.
(Histopathology or angiography are mandatory criterion)90.699.695.1
 1. Skin involvementLivedo reticularis: purplish reticular pattern usually irregularly distributed around subcutaneous fat lobules, often more prominent with cooling78.894.386.6
Skin nodules: tender subcutaneous nodules
Superficial skin infarctions: superficial skin ulcers (involving skin and superficial subcutaneous tissue) or other minor ischaemic changes (nailbed infarctions, splint haemorrhages, digital pulp necrosis)
Deep skin infarctions: deep skin ulcers (involving deep subcutaneous tissue and underlying structures), digital phalanx or other peripheral tissue (nose and ear tips) necrosis/gangrene
 2. Myalgia or muscle tendernessMuscle pain or tenderness72.583.678.1
 3. HypertensionSystolic/diastolic blood pressure greater than 95th centile for height29.78959.4
 4. Peripheral neuropathySensory peripheral neuropathy: neuropathy resulting in glove or stocking distribution of sensory loss26.099.862.9
Motor mononeuritis multiplex Neuritis of named peripheral nerve, only scored if motor involvement
 5. Renal involvementProteinuria >0.3 g/24 h or >30 mmol/mg of urine albumin/creatinine ratio on a spot morning sample37.336.737
Haematuria or red blood cell casts: >5 red blood cells/high power field or red blood cells casts in the urinary sediment or ≥2+ on dipstick
Impaired renal function: measured or calculated GFR (Schwartz formula) <50% normal
c-PAN EULAR/PRINTO/PRES Ankara 2008 classification definition: κ 0.92 (95% CI 0.86 to 0.98)Histopathology or angiographic abnormalities (mandatory) plus one of the five following criteria:89.699.694.6
Skin involvement
Myalgia/muscle tenderness
Peripheral neuropathy
Renal involvement
  • AUC, area under the curve; c-PAN, childhood polyarteritis nodosa; EULAR, European League Against Rheumatism; GFR, glomerular filtration rate; PRES, Paediatric Rheumatology European Society, PRINTO, Paediatric Rheumatology International Trials Organisation.