This protocol addresses pulmonary arterial hypertension complicating limited cutaneous systemic sclerosis in patients where the prior add-on therapy step has not delivered the expected clinical benefit. The scenario assumes no prior PAH treatment before the initial treatment line was started.
When add-on therapy with prostacyclin analogues or agonists (selexipag at maximum tolerated dose) or riociguat does not achieve improvement in 6-minute walk distance, WHO functional class, or haemodynamic parameters, escalation to this protocol is indicated.
For patients who have reached advanced disease severity — WHO functional class III or IV — this protocol involves an intravenous prostacyclin-based therapy. The complete regimen, including criteria, monitoring, and clinical decision points, is available in the full structured protocol.
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).