Agreement with the EULAR/EUSTAR recommendations. Data are mean agreement (SD, range) and percent in the top 3 ratings of the survey scale (7–9).
| Recommendations | Combined North America and Europe, n = 59 | North America, n = 41 | Europe, n = 18 | p | |||
|---|---|---|---|---|---|---|---|
| SSc-related digital vasculopathy (RP, digital ulcers) | |||||||
| 1. Dihydropyridine-type calcium antagonists, usually oral nifedipine, should be considered for first-line therapy for SSc-RP, and intravenous iloprost, or other available intravenous prostanoids, for severe RP. | 7.3 (1.6, 3–9) | 74.5 | 7.2 (1.6, 4–9) | 77.6 | 7.5 (1.6, 3–9) | 72.2 | 0.673 |
| 2. Intravenous prostanoids (in particular iloprost) should be considered in the treatment of active digital ulcers in patients with SSc. | 6.7 (2.5, 0–9) | 67.8 | 6.1 (2.7, 0–9) | 58.6 | 7.8 (1.8, 2–9) | 88.9 | 0.001* |
| 3. Bosentan should be considered in diffuse SSc with multiple digital ulcers after failure of calcium antagonists and, usually, prostanoid therapy. | 5.8 (2.5, 0–9) | 47.5 | 5.6 (2.4, 0–9) | 43.9 | 6.3 (2.6, 1–9) | 55.6 | 0.523 |
| SSc-PAH | |||||||
| 4. Bosentan should be strongly considered to treat SSc-PAH. | 7.7 (1.8, 3–9) | 76.3 | 7.7 (1.8, 3–9) | 83.0 | 7.5 (1.9, 4–9) | 61.1 | 0.547 |
| 5. Sitaxentan may also be considered to treat SSc-PAH. | 7.7 (1.8, 0–9) | 83.1 | 7.5 (1.9, 0–9) | 85.4 | 7.9 (1.5, 5–9) | 77.8 | 0.445 |
| 6. Sildenafil may be considered to treat SSc-PAH. | 8.1 (1.5, 2–9) | 89.8 | 8.1 (1.5, 2–9) | 95.1 | 8.1 (1.5, 4–9) | 89.0 | 0.696 |
| 7. Intravenous epoprostenol should be considered for the treatment of patients with severe SSc-PAH. | 7.8 (2.0, 0–9) | 83.1 | 8.5 (1, 5–9) | 95.0 | 6.5 (2.7, 0–9) | 55.5 | 0.006* |
| SSc-related skin involvement | |||||||
| 8. Methotrexate may be considered for treatment of skin manifestations of early diffuse SSc. | 5.3 (2.8, 0–9) | 40.3 | 5.2 (3, 0–9) | 41.1 | 5.5 (2.6, 0–9) | 38.9 | 0.962 |
| SSc-ILD | |||||||
| 9. Cyclophosphamide should be considered for treatment of SSc-ILD. | 7.7 (1.4, 1–9) | 84.7 | 7.7 (1.5, 1–9) | 87.6 | 7.7 (1.2, 6–9) | 77.8 | 0.755 |
| SSc-SRC | |||||||
| 10. ACE inhibitors should be used in the treatment of SRC. | 8.9 (0.6, 5–9) | 98.3 | 8.9 (0.3, 9) | 100 | 8.8 (0.9, 5–9) | 94.5 | 0.187 |
| 11. Patients on steroids should be carefully monitored for blood pressure and renal function. | 8.6 (1.0, 4–9) | 96.7 | 8.6 (1.1, 4–9) | 95.2 | 8.6 (0.7, 7–9) | 100 | 0.626 |
| SSc-related gastrointestinal disease | |||||||
| 12. PPI should be used for the prevention of SSc-related gastroesophageal reflux, esophageal ulcers and strictures.** | 8.3 (1.5, 2–9) | 89.9 | 8.2 (1.7, 2–9) | 87.9 | 8.5 (0.8, 6–9) | 94.5 | 0.518 |
| 13. Prokinetic drugs should be used for the management of SSc-related symptomatic motility disturbances (dysphagia, GERD, early satiety, bloating, pseudo-obstruction, etc.). | 7.1 (1.9, 0–9) | 71.1 | 7.1 (1.9, 1–9) | 70.8 | 7.1 (2.0, 0–9) | 72.3 | 0.828 |
| 14. When malabsorption is caused by bacterial overgrowth, rotating antibiotics may be useful in SSc patients. | 8.3 (1.1, 4–9) | 93.3 | 8.4 (1.1, 4–9) | 95.2 | 8.1 (1.1, 6–9) | 88.9 | 0.585 |
↵* Mean North American rheumatologist agreement vs European rheumatologist agreement that was statistically different (p < 0.05).
↵** Recommendations that had significantly different agreement (p < 0.05) by respondents who were EULAR/EUSTAR recommendation authors vs respondents who were not EULAR/EUSTAR recommendation authors. EULAR: European League Against Rheumatism; EUSTAR: EULAR Scleroderma Trials and Research group; SSc: systemic sclerosis; RP: Raynaud’s phenomenon; PAH: pulmonary arterial hypertension; ILD: interstitial lung disease; SRC: scleroderma renal crisis; ACE: angiotensin-converting enzyme; PPI: proton pump inhibitors; GERD: gastroesophageal reflux disease.