Article Figures & Data
- Table 1.
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).
- Table 2.
Treatment recommendations with different agreements between European SSc experts and non-European SSc experts.
Recommendations European Experts, n = 157 Non-European Experts, n = 63 p Intravenous iloprost should be considered for severe SSc-RP 8.5 (2.2) 6.5 (3.1) < 0.0001 Availability, % 90 55 Intravenous iloprost should be considered in the treatment of DU in patients with SSc 9.0 (1.9) 7.4 (2.7) < 0.0001 Availability, % 93 59 Bosentan should be considered for reduction of number of new DU in patients with SSc 8.5 (2.3) 6.3 (3.2) < 0.0001 Availability, % 86 57 MTX may be considered for skin manifestations of early diffuse SSc 7.9 (2.7) 6.8 (3.0) < 0.05 Availability, % 95 92 Cyclophosphamide should be considered for treatment of SSc-ILD, in particular for patients with progressive ILD 8.3 (2.4) 7.4 (2.9) < 0.05 Availability, % 99 100 HSCT should be considered for treatment of selected patients with rapidly progressive SSc at risk of organ failure 7.3 (2.7) 6.4 (3.2) < 0.05 Availability, % 66 66 Values are mean (SD) unless otherwise specified. SSc: systemic sclerosis; RP: Raynaud phenomenon; DU: digital ulcers; MTX: methotrexate; ILD: interstitial lung disease; HSCT: hematopoietic stem cell transplant.
- Table 3.
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.
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