Table 1.

Recommendations regarding diagnosis and assessment

	L	S	Agreement (%)
	Overarching principle All children with suspected localised scleroderma should be referred to a specialised paediatric rheumatology centre.	4	D	100
1	LoSSI, which is part of LoSCAT, is a good clinical instrument to assess activity and severity in JLS lesions and is highly recommended in clinical practice.	3	C	90
2	LoSDI, which is part of LoSCAT, is a good clinical instrument to assess damage in JLS and is highly recommended in clinical practice.	3	C	90
3	Infrared thermography can be used to assess activity of the lesions in JLS, but skin atrophy can give false-positive results.	4	D	90
4	A specialised US imaging, using standardised assessment and colour Doppler, may be a useful tool for assessing disease activity, extent of JLS and response to treatment.	4	D	100
5	All patients with JLS at diagnosis and during follow-up should be carefully evaluated with a complete joint examination, including the temporomandibular joint.	2a	C	100
6	MRI can be considered a useful tool to assess musculoskeletal involvement in JLS, especially when the lesion crosses the joint.	3	C	100
7	It is highly recommended that all patients with JLS involving face and head, with or without signs of neurological involvement, have an MRI of the head at the time of the diagnosis.	3	C	90
8	All patients with JLS involving face and head should undergo an orthodontic and maxillofacial evaluation at diagnosis and during follow-up.	2b	B	90
9	Ophthalmological assessment, including screening for uveitis, is recommended at diagnosis for every patient with JLS, especially in those with skin lesions on the face and scalp.	2a	C	100
10	Ophthalmological follow-up, including screening for uveitis, should be considered for every patient with JLS, especially in those with skin lesions on the face and scalp.	3	C	100

JLS, juvenile localised scleroderma; L, level of evidence; LoSCAT, Localized Scleroderma Cutaneous Assessment Tool; LoSDI, Localized Scleroderma Skin Damage Index; LoSSI, Localized Scleroderma Skin Severity Index; S, strength of recommendation; US, ultrasound.