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Table 1 PRES scleroderma working group recommendations assessed within UK audit

From: Multi-centre national audit of juvenile localised scleroderma: describing current UK practice in disease assessment and management

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
  1. JLS Juvenile localised scleroderma, PGA-A Physician global assessment-activity, PGA-D Physician global assessment-damage, LoSCAT Localised scleroderma cutaneous activity tool, PROM Patient-reported outcome measures, MMF Mycophenalate mofetil, ETN Etanercept, ANA Anakinra, TCZ Tocilizumab
  2. aAudit only recorded whether shared care clinic dermatology/rheumatology
  3. bPatients where at least one of PGA-A, PGA-D or LoSCAT was used
  4. cPatients where at least one form of PROM was used
  5. dPatients with joint contractures
  6. e data unavailable for 10 patients