Towards an integrated and proactive management of pulmonary hypertension in systemic sclerosis: a practical approach for early diagnosis and optimal patient management

Pulmonary arterial hypertension (PAH) is a severe vascular complication of SSc and a leading cause of disease-related mortality. Despite the availability of validated screening tools and treatment recommendations, diagnosis delay and suboptimal therapeutic implementation remain frequent in real-world practice. The 2022 European Society of Cardiology/European Respiratory Society guidelines and 2025 EULAR recommendations advocate systematic annual screening and initial combination therapy with an endothelin receptor antagonist and a phosphodiesterase type 5 inhibitor at PAH diagnosis. In SSc patients already receiving a dual combination, escalation to triple therapy including selexipag, or in selected cases switching to riociguat, should be promptly considered. Given the rapid progression and poorer prognosis of SSc-PAH, follow-up within 3 months of diagnosis is critical. Structured referral networks, implementation of the DETECT algorithm and involvement of a dedicated case manager and/or nurse can further improve timely diagnosis and continuity of care. Optimizing SSc-PAH management requires a proactive, integrated approach that bridges rheumatology and cardiology expertise.