Treatment recommendations with different agreements between experts stratified according to drug/treatment option availability.
| Recommendations | Drug Available | Drug Not Available | p |
|---|---|---|---|
| PDE-5 inhibitors should be considered in treatment of SSc-RP | 7.9 (2.5), n = 129 | 6.4 (2.7), n = 88 | < 0.0001 |
| Fluoxetine might be considered in treatment of SSc-RP attacks | 4.9 (2.9), n = 138 | 4.1 (2.5), n = 81 | < 0.05 |
| Intravenous iloprost should be considered for severe SSc-RP | 8.3 (2.6), n = 170 | 6.8 (2.7), n = 44 | < 0.001 |
| Intravenous iloprost should be considered in the treatment of DU in patients with SSc | 8.8 (2.2), n = 176 | 7.5 (2.6), n = 37 | < 0.0001 |
| Bosentan should be considered for reduction of number of new DU in patients with SSc | 8.3 (2.4), n = 165 | 6.5 (3.0), n = 48 | < 0.0001 |
| Riociguat should be considered to treat SSc-related PAH | 8.1 (2.4), n = 99 | 6.6 (3.0), n = 66 | < 0.0001 |
| HSCT should be considered for treatment of selected patients with rapidly progressive SSc at risk of organ failure | 7.4 (2.8), n = 136 | 6.5 (2.8), n = 71 | < 0.05 |
Values are mean (SD) unless otherwise specified. PDE-5: phosphodiestherase type 5; SSc: systemic sclerosis; RP: Raynaud phenomenon; DU: digital ulcers; PAH: pulmonary arterial hypertension; HSCT: hematopoietic stem cell transplant.