Table 2

Summary of included studies evaluating treatment with PAH agents in patients with CTD-PAH

Source (official acronym)PAH agentNumber of patients with CTD-PAHNumber (%) of patients with SSc–PAHStudy designInterventionControlPeriod (weeks)
Badesch et al34 (SUPER-1)Sildenafil8438 (45)RCT, DB20 mg×3/day, 40 mg×3/day and 80 mg×3/dayPlacebo12
Galiè et al18 (PHIRST)Tadalafil95NRRCT, DB2.5, 10, 20 and 40 mgPlacebo16
Denton et al35Bosentan6652 (79)RCT, DB62.5 mg×2/day for 4 weeks, then 125 or 250 mg×2/dayPlacebo12 or 16
Launay et al36Bosentan4949 (100)Single-arm, open-label62.5 mg×2/day for 4 weeks, then 125 or 250 mg×2/dayNone28
Badesch37 (ARIES)Ambrisentan124NRRCT, DB2.5, 5 and 10 mgPlacebo12
Badesch et al38 (ARIES-3)Ambrisentan40NRSingle-arm, open-label5 mgNone24
Badesch et al26Epoprostenol111111 (100)RCT, open-labelDosage established according to signs and symptoms from initial low doseConventional therapy12
Kunieda et al39Beraprost19NRSingle-arm, open-labelInitial dose of 120 mg/day, then titrated to maximum dose of 360 mg/dayNone12
Oudiz et al40Treprostinil9045 (50)RCT, DBInitial dosage of 2.5 or 5.0 ng/kg/min, then titrated to maximum dosage of 20 ng/kg/minPlacebo 8
  • CTD, connective tissue disease; DB, double-blind; NR, not reported; PAH, pulmonary arterial hypertension; RCT, randomised controlled trial; SSc, systemic sclerosis.