Pulmonary Arterial Hypertension in Systemic Sclerosis — Treatment When Already on a PDE5 Inhibitor or Bosentan

In systemic sclerosis, pulmonary arterial hypertension (PAH) frequently occurs alongside digital ulcer management, meaning many patients are already on a PDE5 inhibitor and/or bosentan when PAH is being actively treated. When current therapy is insufficient to reach PAH control targets, a structured therapeutic escalation is indicated.

Clinical Scenario

Patients with systemic sclerosis and pulmonary arterial hypertension who are receiving a PDE5 inhibitor and/or bosentan — agents that may have been started for digital ulcers — but have not achieved adequate PAH disease control. The concurrent regimen shapes which escalation steps are appropriate.

Treatment Approach (partial)

The protocol addresses therapeutic escalation by targeting a complementary pulmonary vascular pathway not covered by the current regimen.

The complete treatment options and sequencing are available in the full protocol below.

Clinical Goals

The aim is to achieve WHO functional class II or better and an ERS low or intermediate risk assessment within 3–6 months of treatment adjustment.

Instant Access to Structured Evidence-Based Regimens

References

DOI: 10.1136/ard-2024-226430

Riociguat can be considered for treatment of SSc PAH.
Other prostacyclin analogues or agonists should be considered for the treatment of SSc PAH.
Intravenous epoprostenol should be considered for the treatment of SSc patients with advanced PAH (class III and IV).
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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