Pulmonary Arterial Hypertension in Limited Cutaneous Systemic Sclerosis — When Initial PAH Therapy Has Not Reached Target
Clinical scenario
A patient with limited cutaneous systemic sclerosis (SSc) has developed pulmonary arterial hypertension (SSc-PAH) and is already receiving a PDE5 inhibitor and/or bosentan — a regimen initiated in part for digital ulcers. Despite this therapy, functional and risk-stratification targets have not been met, raising the question of what the next treatment step should be.
Previous therapy — failure condition
The preceding regimen included riociguat, or prostacyclin analogues such as selexipag, added to the existing background therapy. Escalation to this protocol is indicated when that approach has not achieved WHO functional class II or better, or has not placed the patient at low or intermediate ERS risk after 3–6 months of treatment.
Next-line approach — partial overview
Evidence supports the consideration of intravenous prostacyclin analogue therapy for patients with SSc-PAH who have reached an advanced functional stage. The complete treatment algorithm — including agent selection, monitoring, and sequencing — is detailed in the full protocol.
Dosing, route, duration, and full decision logic are not shown here.
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 ↗