Treatment of Limited Cutaneous Systemic Sclerosis in Pulmonary Arterial Hypertension

Clinical Scenario

This protocol addresses patients with limited cutaneous systemic sclerosis (lcSSc) who have been diagnosed with pulmonary arterial hypertension (PAH) and have not previously received any PAH-specific therapy.

Comorbidity: Pulmonary Arterial Hypertension

Pulmonary arterial hypertension is a recognised complication of limited cutaneous systemic sclerosis (SSc-PAH). Its presence defines a distinct sub-population that requires a specific therapeutic approach, separate from other forms of PAH, applied at the point of diagnosis.

Treatment Approach — Partial Overview

First-line management involves an upfront combination of a PDE5 inhibitor and an endothelin receptor antagonist. The complete regimen — including precise agent selection, monitoring schedule, and decision criteria — is available in the full structured protocol.

Dosing, sequencing, and full clinical algorithm are not shown here. Access the protocol below for the complete evidence-based regimen.

Treatment Goals

The aim is to achieve WHO functional class II or better and an ERS low or intermediate risk assessment at 3–6 months after initiation of therapy.

Instant Access to Structured Evidence-Based Regimens

References

DOI: 10.1136/ard-2024-226430

Combination of PDE5i and endothelin receptor antagonists should be considered as first-line treatment of SSc PAH.
The task force unanimously agreed to recommend the use of first-line combination treatment at diagnosis of PAH.

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