Calculators & Tools

Juvenile Systemic Sclerosis Criteria

PRES/ACR/EULAR paediatric criteria - the mandatory major criterion plus ≥2 of 20 minor criteria.

1 What is the Juvenile Systemic Sclerosis Criteria?

The Juvenile Systemic Sclerosis Criteria is a rheumatology scoring tool used in paediatric practice. PRES/ACR/EULAR paediatric criteria - the mandatory major criterion plus ≥2 of 20 minor criteria. It scores 11 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 11 criteria: MAJOR (mandatory) - Proximal skin sclerosis/induration of the skin proximal to MCP or MTP joints; Cutaneous - sclerodactyly; Vascular - Raynaud phenomenon; Vascular - nailfold capillary abnormalities or digital-tip ulcers; Gastrointestinal - dysphagia or gastro-oesophageal reflux; Renal - renal crisis or new-onset arterial hypertension; Cardiac - arrhythmia or heart failure; Respiratory - pulmonary fibrosis on HRCT/radiograph, reduced DLCO, or pulmonary hypertension; Musculoskeletal - tendon friction rubs, arthritis or myositis; Neurological - neuropathy or carpal tunnel syndrome; Serological - antinuclear antibodies, or SSc-selective antibodies (anti-centromere, anti-topoisomerase I/Scl-70, anti-RNA polymerase, anti-fibrillarin, anti-PM-Scl). Totals are then banded: any score - Criteria not met: the major criterion (proximal skin sclerosis) is mandatory - without it juvenile systemic sclerosis cannot be classified; 4 criteria or more - Major only: major criterion present but fewer than 2 minor criteria - at least 2 minor criteria are required; 6 criteria or more - Criteria MET: major criterion plus ≥2 minor criteria - classifies as juvenile systemic sclerosis (sensitivity 90%, specificity 96%). Screen for pulmonary, cardiac and renal involvement at baseline.
  • Onset must be before the 16th birthday for the juvenile classification.
  • Source: Zulian F et al. The Pediatric Rheumatology European Society/ACR/EULAR provisional classification criteria for juvenile systemic sclerosis. Arthritis Rheum 2007;57:203-12.

4 Frequently asked questions

How many criteria does the Juvenile Systemic Sclerosis Criteria use?

11. They are MAJOR (mandatory) - Proximal skin sclerosis/induration of the skin proximal to MCP or MTP joints; Cutaneous - sclerodactyly; Vascular - Raynaud phenomenon; Vascular - nailfold capillary abnormalities or digital-tip ulcers; Gastrointestinal - dysphagia or gastro-oesophageal reflux; Renal - renal crisis or new-onset arterial hypertension; and others.

What do the bands of the Juvenile Systemic Sclerosis Criteria mean?

The total is interpreted as: any score - Criteria not met: the major criterion (proximal skin sclerosis) is mandatory - without it juvenile systemic sclerosis cannot be classified; 4 criteria or more - Major only: major criterion present but fewer than 2 minor criteria - at least 2 minor criteria are required; 6 criteria or more - Criteria MET: major criterion plus ≥2 minor criteria - classifies as juvenile systemic sclerosis (sensitivity 90%, specificity 96%). Screen for pulmonary, cardiac and renal involvement at baseline.

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?

Onset must be before the 16th birthday for the juvenile classification.

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.