L

S

Agreement %

1

Skin involvement

4

D

100

Systemic corticosteroids, in combination with a DMARD, are useful in the active inflammatory phase of JSSc.

2

At the time of diagnosis of JSSc, a systemic immunomodulatory treatment, like methotrexate, should be considered.

4

D

100

3

In case of non-response to methotrexate an additional immunomodulatory agent (i.e. MMF) should be considered.

3

D

90

4

Pulmonary involvement

4

D

100

Cyclophosphamide may be used to treat cardiac and/or pulmonary involvement.

5

Vascular involvement

3

C

90

Iloprost may be used to treat ischaemic digits and digital ulcerations.

6

In New York stage II pulmonary hypertension, and/or digital ulcerations refractory to other therapies, bosentan should be considered.

3

D

100

7

Experimental treatment

4

D

100

Biological agents, in particular tocilizumab or rituximab, should be considered in severe or refractory cases.

8

Autologous stem cell transplantation may be an option to treat patients with JSSc with progressive disease refractory to immunosuppressive therapy.

2a 2b

B

100