Recommendations for treatment of systemic sclerosis; mean level of agreement among SSc experts (n = 209).
| Recommendations | Agreement, Mean (SD) |
|---|---|
| CCB should be considered as first-line therapy for SSc-RP | 8.2 (2.7) |
| PDE-5 inhibitors should be considered in treatment of SSc-RP | 7.3 (2.7) |
| Fluoxetine might be considered in treatment of SSc-RP attacks | 4.6 (2.8) |
| Intravenous iloprost should be considered for severe SSc-RP | 7.9 (2.7) |
| Intravenous iloprost should be considered in the treatment of DU in patients with SSc | 8.7 (2.3) |
| PDE-5 inhibitors should be considered in the treatment of DU in patients with SSc | 8.0 (2.5) |
| Bosentan should be considered for reduction of number of new DU in patients with SSc | 7.9 (2.8) |
| ERA should be considered to treat SSc-related PAH* | 8.8 (2.4) |
| PDE-5 inhibitors should be considered to treat SSc-related PAH* | 8.9 (2.3) |
| Riociguat should be considered to treat SSc-related PAH* | 7.4 (2.8) |
| Intravenous epoprostenol should be considered for treatment of patients with severe SSc-related PAH* | 8.3 (2.5) |
| Prostacyclin analogs should be considered to treat SSc-related PAH* | 8.0 (2.7) |
| MTX may be considered for skin manifestations of early diffuse SSc | 7.4 (2.8) |
| Cyclophosphamide should be considered for treatment of SSc-ILD, in particular for patients with progressive ILD | 8.0 (2.6) |
| HSCT should be considered for treatment of selected patients with rapidly progressive SSc at risk of organ failure | 7.1 (2.9) |
| Experts recommend immediate use of ACE inhibitors in the treatment of SRC | 9.2 (2.1) |
| Blood pressure and renal function should be carefully monitored in SSc patients treated with glucocorticoids | 9.0 (2.2) |
| PPI should be used for the treatment of SSc-related GERD, and prevention of esophageal ulcers and strictures | 9.0 (2.2) |
| Prokinetic drugs should be used for the management of SSc-related symptomatic motility disturbances | 8.0 (2.4) |
| Intermittent or rotating use of antibiotics may be useful to treat symptomatic bacterial overgrowth in patients with SSc | 8.5 (2.1) |
↵* N = 166: of n = 209 complete responders, 21% did not complete the questions regarding PAH-specific drugs because they stated that they felt uncomfortable answering questions regarding guidelines on treatment of SSc-related PAH. SSc: systemic sclerosis; CCB: dihydropyridine-type calcium channel blockers; PDE-5: phosphodiestherase type 5; ACE: angiotensin-converting enzyme; PPI: proton pump inhibitors; RP: Raynaud phenomenon; DU: digital ulcers; ERA: endothelin receptor antagonists; PAH: pulmonary arterial hypertension; MTX: methotrexate; ILD: interstitial lung disease; HSCT: hematopoietic stem cells transplant; SRC: scleroderma renal crises; GERD: gastroesophageal reflux disease (nonsignificant).