Final EULAR/PRINTO/PRES c-PAN criteria (with glossary) and classification definition (sample 1099)
| Criterion | Glossary | Sensitivity (%) | Specificity (%) | AUC (%) |
|---|---|---|---|---|
| A systemic inflammatory disease characterised by: | ||||
| Histopathology | Evidence of necrotising vasculitis in medium or small sized arteries | 84.8 | 99.7 | 92.3 |
| 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. | 81.0 | 99.9 | 90.5 |
| Conventional angiography is the preferred imaging modality. | ||||
| (Histopathology or angiography are mandatory criterion) | 90.6 | 99.6 | 95.1 | |
| 1. Skin involvement | Livedo reticularis: purplish reticular pattern usually irregularly distributed around subcutaneous fat lobules, often more prominent with cooling | 78.8 | 94.3 | 86.6 |
| 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 tenderness | Muscle pain or tenderness | 72.5 | 83.6 | 78.1 |
| 3. Hypertension | Systolic/diastolic blood pressure greater than 95th centile for height | 29.7 | 89 | 59.4 |
| 4. Peripheral neuropathy | Sensory peripheral neuropathy: neuropathy resulting in glove or stocking distribution of sensory loss | 26.0 | 99.8 | 62.9 |
| Motor mononeuritis multiplex Neuritis of named peripheral nerve, only scored if motor involvement | ||||
| 5. Renal involvement | Proteinuria >0.3 g/24 h or >30 mmol/mg of urine albumin/creatinine ratio on a spot morning sample | 37.3 | 36.7 | 37 |
| 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: | 89.6 | 99.6 | 94.6 |
| Skin involvement | ||||
| Myalgia/muscle tenderness | ||||
| Hypertension | ||||
| 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.