Abstract
Pulmonary arterial hypertension, categorized as group 1 pulmonary hypertension by the World Health Organization classification system, represents a major complication of systemic sclerosis resulting from pulmonary vascular involvement of the disease. The high mortality seen in systemic sclerosis-associated pulmonary arterial hypertension is likely due to the impairment of right ventricular systolic function and the coexistence of other non-group-1 pulmonary hypertension phenotypes that may negatively impact clinical response to pulmonary arterial hypertension-targeted therapy. This review highlights two areas of recent advances regarding the management of systemic sclerosis patients with pulmonary hypertension: the tolerability of pulmonary arterial hypertension-targeted therapy in the presence of mild to moderate interstitial lung disease and the potential clinical significance of the antifibrotic effect of soluble guanylate cyclase stimulators demonstrated in preclinical studies.
Highlights
Systemic sclerosis (SSc) is a systemic rheumatic disease characterized by autoimmunity, vascular injury, and unchecked collagen synthesis leading to fibrosis of the skin and internal organs[1,2]
A recent subgroup analysis of the two trials demonstrated in SSc-pulmonary arterial hypertension (PAH) patients that riociguat was associated with improvement in cardiac index and reduction in pulmonary vascular resistance (PVR) at 12 weeks, and possibly with an increase in the 6-minute walk distance compared to placebo[37]
Given that riociguat is effective in CTEPH93 and SSc patients are predisposed to venous thromboembolism (VTE), another interesting question regards the potential role of Soluble guanylate cyclase (sGC) stimulation in SSc patients affected by chronic thromboembolic pulmonary hypertension (CTEPH)
Summary
Systemic sclerosis (SSc) is a systemic rheumatic disease characterized by autoimmunity, vascular injury, and unchecked collagen synthesis leading to fibrosis of the skin and internal organs[1,2]. The Ambrisentan and Tadalafil in Patients with Pulmonary Arterial Hypertension (AMBITION) trial was a recently published randomized double-blind multicenter study that compared upfront ambrisentan and tadalafil combination therapy to monotherapy in 500 treatment-naïve participants with WHO functional class II–III PAH35. A recent subgroup analysis of the two trials demonstrated in SSc-PAH patients that riociguat was associated with improvement in cardiac index and reduction in PVR at 12 weeks, and possibly with an increase in the 6-minute walk distance compared to placebo[37]. Another case series described three patients whose SSc-PAH improved after phosphodiesterase-5 inhibitors were replaced with riociguat[90]. Given that riociguat is effective in CTEPH93 and SSc patients are predisposed to VTE, another interesting question regards the potential role of sGC stimulation in SSc patients affected by CTEPH
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