Recommendation | Number of patients meeting recommendation (%) |
---|---|
Extra-cutaneous manifestations | |
In craniofacial JLS, brain MRI is recommended | 37/52 (71.2%) |
In craniofacial JLS, uveitis screening every 6 months is recommended for the first 4 years of the disease | 1/52 (1.9%) |
In non-facial JLS, uveitis screening every 12 months is recommended for the first four years | 6/97 (6.2%) |
Dental assessment is suggested for any child with craniofacial JLS | 12/52 (23.1%) |
Assessment | |
All patients with JLS, except for those with solitary small superficial plaque scleroderma, should be seen at baseline and minimum of every 12 months by a paediatric rheumatologist and (paediatric) dermatologist, ideally in a combined clinic | 107/149 (71.8%)a |
PGA-A, PGA-D and the LoSCAT, are acceptable tools for capturing cutaneous disease activity and damage | 83/149 (55.7%)b |
PROMs should be considered as an adjunct to clinical outcome measures and the following are recommended for serial measurements: Child/parent global assessment of disease severity on a visual analogue scale of 0–10 cm; Children’s Dermatology Life Quality Index | 86/149 (57.7%)c |
Treatment | |
All types of active JLS require systemic immunosuppression, except for circumscribed small superficial non-progressive lesions, which are not crossing a joint and occur in non-cosmetically sensitive areas, where topical treatments alone may be appropriate | 143/143 (100%) |
First line treatment should be with methotrexate 15 mg/m2/week, max 25 mg/week either orally or subcutaneously | 127/133 (95.5%)e |
Bridging therapy with glucocorticoids should be used for patients requiring immunosuppression, particularly in rapidly progressive and severe disease, such as lesions crossing the joints and cosmetically disfiguring disease. | 123/143 (86.0%) |
Physiotherapy and/or occupational therapy are recommended for any patients with decreased range of motion in any joints | 24/30 (80.0%)d |