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| 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. |
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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. |
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5 | Vascular involvement | 3 | C | 90 |
| Iloprost may be used to treat ischaemic digits and digital ulcerations. |
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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. |
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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 |