Pulmonary Arterial Hypertension in Systemic Sclerosis — What to Do When No PAH Therapy Has Been Given

This protocol addresses patients with systemic sclerosis who have developed pulmonary arterial hypertension (SSc-PAH) and have not yet received any PAH-specific treatment.

Clinical Situation

Pulmonary arterial hypertension is a recognised complication of systemic sclerosis. In PAH-naïve patients with SSc-PAH, the choice of initial therapy is pivotal for achieving meaningful functional improvement and reducing disease burden.

Treatment Approach

Evidence supports starting with an upfront combination strategy using two drug classes with distinct mechanisms as first-line treatment for SSc-PAH.

The specific agents, sequencing, monitoring parameters, and complete algorithm are set out in the full protocol below.

Treatment Goals

WHO functional class II or better
Low or intermediate ERS risk assessment at 3–6 months

Instant Access to Structured Evidence-Based Regimens

References

Combination of PDE5i and endothelin receptor antagonists should be considered as first-line treatment of SSc PAH.

In AMBITION, 500 participants with PAH (connective tissue disease, CTD and Non CTD) all in WHO functional class II or III, were randomised to ambrisentan 10 mg and tadalafil 40 mg in combination or the single intervention with placebo in a 2:1:1 ratio.

The task force unanimously agreed to recommend the use of first-line combination treatment at diagnosis of PAH.

DOI: 10.1136/ard-2024-226430

View source ↗