Summary of included studies evaluating treatment with PAH agents in patients with CTD-PAH
| Source (official acronym) | PAH agent | Number of patients with CTD-PAH | Number (%) of patients with SSc–PAH | Study design | Intervention | Control | Period (weeks) |
|---|---|---|---|---|---|---|---|
| Badesch et al34 (SUPER-1) | Sildenafil | 84 | 38 (45) | RCT, DB | 20 mg×3/day, 40 mg×3/day and 80 mg×3/day | Placebo | 12 |
| Galiè et al18 (PHIRST) | Tadalafil | 95 | NR | RCT, DB | 2.5, 10, 20 and 40 mg | Placebo | 16 |
| Denton et al35 | Bosentan | 66 | 52 (79) | RCT, DB | 62.5 mg×2/day for 4 weeks, then 125 or 250 mg×2/day | Placebo | 12 or 16 |
| Launay et al36 | Bosentan | 49 | 49 (100) | Single-arm, open-label | 62.5 mg×2/day for 4 weeks, then 125 or 250 mg×2/day | None | 28 |
| Badesch37 (ARIES) | Ambrisentan | 124 | NR | RCT, DB | 2.5, 5 and 10 mg | Placebo | 12 |
| Badesch et al38 (ARIES-3) | Ambrisentan | 40 | NR | Single-arm, open-label | 5 mg | None | 24 |
| Badesch et al26 | Epoprostenol | 111 | 111 (100) | RCT, open-label | Dosage established according to signs and symptoms from initial low dose | Conventional therapy | 12 |
| Kunieda et al39 | Beraprost | 19 | NR | Single-arm, open-label | Initial dose of 120 mg/day, then titrated to maximum dose of 360 mg/day | None | 12 |
| Oudiz et al40 | Treprostinil | 90 | 45 (50) | RCT, DB | Initial dosage of 2.5 or 5.0 ng/kg/min, then titrated to maximum dosage of 20 ng/kg/min | Placebo | 8 |
CTD, connective tissue disease; DB, double-blind; NR, not reported; PAH, pulmonary arterial hypertension; RCT, randomised controlled trial; SSc, systemic sclerosis.