Systemic Sclerosis with Pulmonary Arterial Hypertension: What to Do When Add-On Therapy Has Not Met Goals

Clinical scenario

This protocol addresses patients with systemic sclerosis (SSc) complicated by pulmonary arterial hypertension (SSc-PAH) who have already received add-on therapy but have not achieved the expected functional and haemodynamic targets.

Prior therapy — targets not achieved

Add-on therapy with prostacyclin analogues or agonists (selexipag) or riociguat did not result in sufficient improvement in 6-minute walk distance, WHO functional class, or haemodynamic parameters. This failure of goals triggers escalation to the present protocol.

Next-step approach (partial overview)

For SSc-PAH patients with advanced disease — particularly those in higher WHO functional classes — an intravenous therapeutic approach is among the interventions considered at this stage. The complete treatment pathway and selection criteria are available in the full protocol.

Instant Access to Structured Evidence-Based Regimens

References

DOI: 10.1136/ard-2024-226430

Combination of PDE5i and endothelin receptor antagonists should be considered as first-line treatment of SSc PAH.
Intravenous epoprostenol should be considered for the treatment of SSc patients with advanced PAH (class III and IV).

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