New recommendations on the management of juvenile localised scleroderma


  • Mary Corcoran
  • Univadis Medical News
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An international committee of experts in paediatric rheumatology has developed new consensus-based recommendations on the management of juvenile localised scleroderma (JLS). 

Key recommendations include:

  • Localised Scleroderma Skin Severity Index is highly recommended.            
  • Infrared thermography can assess activity, but skin atrophy can give false-positive results.      
  • Ultrasound, using standardised assessment and colour Doppler, can assess disease activity, extent of JLS and response to treatment.   
  • Use magnetic resonance imaging when the lesion crosses the joint.          
  • MRI of the head and orthodontic and maxillofacial evaluation for all patients with JLS involving face and head, with or without signs of neurological involvement.         
  • Ophthalmological assessment during diagnosis and follow-up for all patients.              
  • When starting systemic corticosteroids in the active inflammatory phase, methotrexate or alternative disease-modifying anti-rheumatic drug should be started.          
  • Oral or subcutaneous methotrexate at 15 mg/m2/week for active, potentially disfiguring or disabling JLS.
  • If effective, methotrexate should be maintained for ≥12 months before tapering.              
  • Mycophenolate mofetil may be used for severe JLS or methotrexate-refractory or methotrexate-intolerant patients.               
  • Medium-dose UVA1 phototherapy may improve skin softness in circumscribed morphoea lesions.
  • Topical imiquimod to decrease skin thickening of circumscribed morphoea.

The recommendations were developed as part of the Single Hub and Access point for paediatric Rheumatology in Europe initiative.