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

Abstract

The evidence suggests a beneficial effect of dual therapy in IPAH and SSc-PAH, particularly those who are deteriorating on monotherapy. Research should focus on subsets of patients to identify the optimal timing and combination of dual therapy.

Medical Therapies for the Treatment of Pulmonary Arterial Hypertension: How Do We Choose?

“…There is strong evidence to support sequential combination therapy, with data from metaanalyses showing improved functional class, exercise capacity and haemodynamics, but no mortality benefit [70]. In patients with IPAH and SSc-PAH, the combination of either an ERA or PDE5i with prostanoid appears to be more effective than ERA and PDE5i in combination [71], particularly in those deteriorating on monotherapy.…”

“…Upfront combination therapy is likely to have a role in the future for severe PAH and possibly for young fitter patients in whom a more aggressive treatment stance may be taken. This approach is likely to affect clinician preference in initial drug choice within a class, as certain combinations seem to be more effective [71]. Further research should focus on PAH subgroups to identify the most appropriate timings and combinations for specific patient populations.…”

Update on Systemic Sclerosis

“…For severe disease, first-line treatment with prostanoids is recommended. Dual-combination therapy appears to be beneficial in improving exercise capacity, functional class, and quality of life in patients' clinical decline on monotherapy [64].…”

New insights into the pathogenesis and management of juvenile systemic sclerosis

“…However to date, none of the limited number of randomized, controlled clinical trials with these DMARDs in adult or childhood systemic sclerosis have been able to demonstrate a therapeutic effect in either preventing disease progression or reversing fibrosis [49][50][51][52][53][54][55][56][57][58]. As a result of these discouraging findings, therapy in SSc remains largely organ-specific encompassing endothelin receptor antagonists, phospho-diesterase inhibitors or prostanoids for pulmonary arterial hypertension and angiotensin-converting enzyme inhibitors (ACEi) for renal disease [59][60][61][62][63].…”