This protocol addresses patients with limited cutaneous systemic sclerosis (lcSSc) who have developed pulmonary arterial hypertension (PAH) and whose initial combination therapy has not achieved adequate disease control after 3–6 months.
The patient has established pulmonary arterial hypertension in the context of limited cutaneous systemic sclerosis (SSc-PAH). An upfront combination of a PDE5 inhibitor and an endothelin receptor antagonist was initiated as first-line treatment.
The prior regimen — PDE5 inhibitor combined with endothelin receptor antagonist — did not reach the following targets at 3–6 months:
This protocol defines the recommended escalation step following that failure.
The next step involves adding a further therapeutic agent to the ongoing regimen. Options from the prostacyclin pathway are among those considered. The complete selection criteria, sequencing, and decision algorithm are available in the full protocol.
Combination of PDE5i and endothelin receptor antagonists should be considered as first-line treatment of SSc PAH.
Other prostacyclin analogues or agonists should be considered for the treatment of SSc PAH.
Riociguat can be considered for treatment of SSc PAH.
The 170 patients with SSc-PAH (77 on treatment vs 93 on placebo) had a similar maximum tolerated dose despite slight difference in proportion of background therapy and showed an overall reduction in risk of a morbidity/mortality event of 44%.
SSc-PAH patients receiving riociguat reported a 4 min improvement in 6MWD at week 12 vs a 37 min worsening in the placebo group.
This was associated with haemodynamic and WHO functional class improvement that persisted in the long-term analysis of PATENT-2.
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