Pulmonary arterial hypertension (PAH) is a serious complication of systemic sclerosis. When a treatment-naive SSc-PAH patient is started on first-line combination therapy and does not reach the expected clinical benchmarks after 3–6 months, a structured escalation pathway applies.
Pulmonary arterial hypertension complicating systemic sclerosis in a patient who was treatment-naive at first-line initiation. This protocol addresses the next therapeutic step when that initial regimen does not produce a sufficient response.
Upfront combination of a PDE5 inhibitor and an endothelin receptor antagonist is the recommended first-line approach for SSc-PAH.
The prior first-line regimen — upfront combination of a PDE5 inhibitor (tadalafil) and an endothelin receptor antagonist (ambrisentan) — is considered insufficient when the following targets are not achieved after 3–6 months of treatment:
Non-achievement of these benchmarks is the trigger for escalation to this protocol.
When first-line targets are not reached, escalation involves add-on therapy from the prostacyclin pathway — one of the agent classes involved in the structured next step. The complete selection criteria, specific agents, and sequencing are available in the full protocol.
References
DOI: 10.1136/ard-2024-226430
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.
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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