Dual therapy in IPAH and SSc-PAH. A qualitative systematic review

Respiratory Medicine, 04/05/2012

Johnson SR et al. – The evidence suggests a beneficial effect of dual therapy in idiopathic pulmonary arterial hypertension and systemic sclerosis–associated pulmonary arterial hypertension, particularly those who are deteriorating on monotherapy. Research should focus on subsets of patients to identify the optimal timing and combination of dual therapy.


  • A systematic review of MEDLINE (1950–2011), EMBASE (1980–2011) and CINAHL (inception–2011) was conducted to identify studies that evaluated the effect of any dual combination of ERA, PDE–5 inhibitors or prostaglandin analogues on 6–min walk distance (6MWD), functional class (FC), haemodynamics, quality–of–life (QoL) or time–to–clinical–worsening in IPAH or SSc–PAH.
  • A standardized form was used to design, sample size, aetiology, outcome and treatment effect.


  • Twenty–six observational studies and 6 randomized trials were identified.
  • Using combination PDE–5 inhibitor and prostaglandin analogues, 6/7 studies reported improvement in 6MWD, 6/8 studies reported improvement in FC, 6/6 studies reported improvement in haemodynamics and 1 trial demonstrated improvement in QoL and time–to–clinical–worsening.
  • Using combination ERA and prostaglandin analogues, 4/6 studies and 1 trial reported improvement in 6MWD, 3/3 studies and 1 trial reported improvement in FC, 4/5 studies and 1 trial reported improvement in PAP.
  • Using combination ERA and PDE–5 inhibitor, 4/7 studies reported an improvement in 6MWD, and 2/6 report improvement in FC.

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