Table 2

Recommendations regarding treatment

LSAgreement
(%)
Systemic corticosteroids may be useful in the active inflammatory phase of JLS. At the same time as starting systemic corticosteroids, MTX or an alternative DMARD should be started.2bC100
All patients with active, potentially disfiguring or disabling forms of JLS should be treated with oral or subcutaneous methotrexate at 15 mg/m²/week.1bA100
If acceptable clinical improvement is achieved, methotrexate should be maintained for at least 12 months before tapering.3C100
Mycophenolate mofetil may be used to treat severe JLS or MTX-refractory or MTX-intolerant patients.2aB100
Medium-dose UVA1 phototherapy may be used to improve skin softness in isolated (circumscribed) morphoea lesions.1bA100
Topical imiquimod may be used to decrease skin thickening of circumscribed morphoea.3C100
  • DMARD, disease-modifying antirheumatic drug; JLS, juvenile localised scleroderma; L, level of evidence; MTX, methotrexate; S, strength of recommendation; UVA1, ultraviolet A1.