Table 2.

Recommendations regarding treatment

	L	S	Agreement (%)
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.	2b	C	100
All patients with active, potentially disfiguring or disabling forms of JLS should be treated with oral or subcutaneous methotrexate at 15 mg/m²/week.	1b	A	100
If acceptable clinical improvement is achieved, methotrexate should be maintained for at least 12 months before tapering.	3	C	100
Mycophenolate mofetil may be used to treat severe JLS or MTX-refractory or MTX-intolerant patients.	2a	B	100
Medium-dose UVA1 phototherapy may be used to improve skin softness in isolated (circumscribed) morphoea lesions.	1b	A	100
Topical imiquimod may be used to decrease skin thickening of circumscribed morphoea.	3	C	100

DMARD, disease-modifying antirheumatic drug; JLS, juvenile localised scleroderma; L, level of evidence; MTX, methotrexate; S, strength of recommendation; UVA1, ultraviolet A1.