Calculators & Tools

Childhood Polyarteritis Nodosa Criteria

EULAR/PRINTO/PRES - mandatory histology or angiographic abnormality PLUS ≥1 of five features.

1 What is the Childhood Polyarteritis Nodosa Criteria?

The Childhood Polyarteritis Nodosa Criteria is a rheumatology scoring tool used in paediatric practice. EULAR/PRINTO/PRES - mandatory histology or angiographic abnormality PLUS ≥1 of five features. It scores 6 criteria and interprets the total across 3 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.

2 When to use it

  • Grading severity at the bedside in rheumatology
  • Recording an objective severity band in the case notes
  • Deciding when a total crosses the threshold for escalation
  • Re-scoring to track whether a child is improving or deteriorating
  • Checking the exact criteria of a score used infrequently

3 Formula & method

The score sums 6 criteria: MANDATORY - Necrotising vasculitis on biopsy OR angiographic abnormality (aneurysm/stenosis/occlusion); Skin involvement - livedo reticularis, nodules, superficial or deep infarcts; Myalgia or muscle tenderness; Hypertension for age; Peripheral neuropathy - sensory or motor mononeuritis multiplex; Renal involvement - proteinuria, haematuria or impaired renal function. Totals are then banded: any score - Criteria not met: the mandatory histological or angiographic criterion is absent - childhood PAN cannot be classified; 4 criteria or more - Mandatory only: histology/angiography present but no additional feature yet - at least one of the five clinical criteria is required; 5 criteria or more - Criteria MET: classifies as childhood polyarteritis nodosa (sensitivity 89.6%, specificity 99.6%). Treat with corticosteroids plus cyclophosphamide in severe disease; assess for hepatitis B.
  • Source: Ozen S et al. EULAR/PRINTO/PRES Ankara 2008 criteria. Ann Rheum Dis 2010;69:798-806.

4 Frequently asked questions

How many criteria does the Childhood Polyarteritis Nodosa Criteria use?

6. They are MANDATORY - Necrotising vasculitis on biopsy OR angiographic abnormality (aneurysm/stenosis/occlusion); Skin involvement - livedo reticularis, nodules, superficial or deep infarcts; Myalgia or muscle tenderness; Hypertension for age; Peripheral neuropathy - sensory or motor mononeuritis multiplex; Renal involvement - proteinuria, haematuria or impaired renal function.

What do the bands of the Childhood Polyarteritis Nodosa Criteria mean?

The total is interpreted as: any score - Criteria not met: the mandatory histological or angiographic criterion is absent - childhood PAN cannot be classified; 4 criteria or more - Mandatory only: histology/angiography present but no additional feature yet - at least one of the five clinical criteria is required; 5 criteria or more - Criteria MET: classifies as childhood polyarteritis nodosa (sensitivity 89.6%, specificity 99.6%). Treat with corticosteroids plus cyclophosphamide in severe disease; assess for hepatitis B.

Does the score replace clinical judgement?

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.

Where does this score come from?

Source: Ozen S et al. EULAR/PRINTO/PRES Ankara 2008 criteria. Ann Rheum Dis 2010;69:798-806.

Does it work offline?

Yes. Score definitions are bundled with the PediAid app, so the score computes without a connection.

For qualified clinicians. This page and the PediAid app are a clinical aid only. Calculations and reference data must be verified against the patient's clinical context, the source guideline and your local protocols before any treatment decision is made.