Pulmonary Arterial Hypertension in Systemic Sclerosis — Next Step When Riociguat or Selexipag Has Not Achieved Treatment Targets

This protocol applies to patients with systemic sclerosis who have pulmonary arterial hypertension and are already on a PDE5 inhibitor and/or bosentan (for digital ulcers), and who were subsequently escalated to riociguat or prostacyclin analogues (selexipag) — yet have not reached the expected treatment goals at re-evaluation.

Previous line — failure condition

The prior escalation step involved adding or switching to riociguat, or adding prostacyclin analogues (selexipag). Escalation to this protocol is triggered when the patient has not achieved WHO functional class II or better, or an ERS low or intermediate risk assessment, after 3–6 months of that therapy.

Next escalation step

The approach at this stage centres on an intravenous prostacyclin analogue for advanced pulmonary arterial hypertension. The complete structured protocol — including agent selection, patient criteria, and the full regimen — is available via the link below.

Instant Access to Structured Evidence-Based Regimens

References

DOI: 10.1136/ard-2024-226430

Intravenous epoprostenol should be considered for the treatment of SSc patients with advanced PAH (class III and IV).

Other prostacyclin analogues or agonists should be considered for the treatment of SSc PAH.

View source ↗