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.