Pulmonary arterial hypertension (PAH) is a serious complication of limited cutaneous systemic sclerosis (lcSSc). When PAH persists despite an existing PDE5 inhibitor and/or bosentan regimen — agents often initiated for digital ulcers — further escalation of pulmonary vasodilator therapy is indicated. This protocol defines the structured approach for that specific clinical situation.

Clinical Scenario

Pulmonary arterial hypertension complicating lcSSc, in a patient already receiving a PDE5 inhibitor and/or bosentan. Treatment goals have not been met and escalation of pulmonary vasodilator management is required.

Treatment Approach (partial overview)

The protocol involves adding or switching to a different class of pulmonary vasodilator to address residual PAH burden. The complete decision algorithm, full set of therapeutic options, and sequencing criteria are available in the structured protocol below.

Treatment Goals

WHO functional class II or better, and ERS low or intermediate risk stratification, reassessed at 3–6 months.

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.
Riociguat can be considered for 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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Pulmonary Arterial Hypertension in Limited Cutaneous Systemic Sclerosis: Treatment When Already on PDE5 Inhibitor or Bosentan

References