How to Assess Pulmonary Circulation and Right Heart Chambers in Systemic Sclerosis Patients?

Systemic sclerosis (SSc) is a rare autoimmune connective tissue disease characterized by a widespread accumulation of extracellular matrix components leading to fibrosis of the skin and internal organs. Vascular changes occur in all involved tissues and are responsible for several distinctive clinic...

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Main Authors: Michele Correale, Ester Maria Lucia Bevere, Lucia Tricarico, Deborah Villani, Mattia Granato, Erminia Guerriero, Raffaele Capasso, Luciano Rossi, Cinzia Rotondo, Francesco Paolo Cantatore, Addolorata Corrado, Massimo Iacoviello, Natale Daniele Brunetti
Format: Article
Language:English
Published: MDPI AG 2025-04-01
Series:Diagnostics
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Online Access:https://www.mdpi.com/2075-4418/15/8/1029
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Summary:Systemic sclerosis (SSc) is a rare autoimmune connective tissue disease characterized by a widespread accumulation of extracellular matrix components leading to fibrosis of the skin and internal organs. Vascular changes occur in all involved tissues and are responsible for several distinctive clinical manifestations of the disease. This review focuses on the usefulness of various diagnostic tools in clinical practice for the early identification of clinical, functional, and/or structural RV impairment in SSc patients at risk of PH. It aims to identify specific causes of RV dysfunction, describe potential differences in outcome measures, and, ultimately, determine different cut-off values compared to subjects with PH not related to SSc.
ISSN:2075-4418