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

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ID: 320549
2026
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Ranked #44 of 46 articles by views in Rheumatology Advances in Practice

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Abstract
Abstract Pulmonary arterial hypertension (PAH) is a severe vascular complication of systemic sclerosis (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 ESC/ERS guidelines and 2025 EULAR recommendations advocate systematic annual screening and initial combination therapy with an endothelin receptor antagonist and a PDE5 inhibitor at PAH diagnosis. In SSc patients already receiving 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 three 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.
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openalex_W7167894900 Use this key to autocite in the manuscript while using SciMatic Manuscript Manager or Thesis Manager
Authors Dilia Giuggioli, Francesco Del Galdo, Michele D’Alto, Marco Matucci‐Cerinic
Journal Rheumatology Advances in Practice
Year 2026
DOI
10.1093/rap/rkag077
URL
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