L

S

Agreement %

1

Overarching principle

4

D

100

All children with suspected JSSc should be referred to a specialized paediatric rheumatology centre for multi-disciplinary care.

2

Vascular involvement

3

C

80

All patients with isolated RP should have a nailfold capillary assessment and ANA testing. If capillaroscopy is abnormal and/or ANA are positive, then regular follow-up is recommended.

3

Cutaneous involvement

4

D

100

A standardized skin score tool should be used for clinical assessment in JSSc. The modified Rodnan skin score is suitable, but needs to be adapted and validated for use in childhood.

4

Internal organ involvement

4

D

100

The Juvenile Systemic Sclerosis Severity Score (J4S) can be used as a severity assessment tool in JSSC, but needs to be validated.

5

Pulmonary function tests, including DLCO, and HRCT in children with JSSc are sensitive tools to detect presence and severity of interstitial lung disease. Pulmonary function tests are also indicated for the respiratory function monitoring.

3

C

100

6

Patients with JSSc should have assessment of pulmonary function tests with DLCO, cardiac echo, renal function and modified Rodnan skin score at least every 6 months.

4

D

90