Table 1

Recommendations regarding diagnosis and assessment

LSAgreement
(%)

Overarching principle

All children with suspected localised scleroderma should be referred to a specialised paediatric rheumatology centre.
4D100
1LoSSI, 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.3C90
2LoSDI, which is part of LoSCAT, is a good clinical instrument to assess damage in JLS and is highly recommended in clinical practice.3C90
3Infrared thermography can be used to assess activity of the lesions in JLS, but skin atrophy can give false-positive results.4D90
4A 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.4D100
5All patients with JLS at diagnosis and during follow-up should be carefully evaluated with a complete joint examination, including the temporomandibular joint.2aC100
6MRI can be considered a useful tool to assess musculoskeletal involvement in JLS, especially when the lesion crosses the joint.3C100
7It 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.3C90
8All patients with JLS involving face and head should undergo an orthodontic and maxillofacial evaluation at diagnosis and during follow-up.2bB90
9Ophthalmological 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.2aC100
10Ophthalmological 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.3C100
  • 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.