: Primary heart involvement (pHI) is frequent in systemic sclerosis (SSc), even though often underdiagnosed. SSc-pHI has been recently defined as cardiac abnormalities that are predominantly attributable to SSc rather than other causes and/or complications. SSc-pHI represents a major determinant of mortality in SSc, accounting alone for about 12% of disease-related deaths; its early recognition and promptly therapeutic interventions are therefore crucial. Both perfusion defects and myocardial inflammation contribute to the occurrence of myocardial fibrosis that precipitates myocardial remodeling, potentially leading to heart failure and arrhythmic complications. To date, clear evidence and guidelines for effectively managing SSc pHI are not established yet, resulting in a lack of a defined therapeutic algorithm. In this review we summarize the most recent scientific literature on the prevailing therapeutic strategies and interventions to manage SSc-pHI, with particular focus on therapeutic strategies to counteract the 3 major pathogenic events of the disease, i.e. microvascular damage, myocardial inflammation and myocardial fibrosis.

Therapeutic strategies for primary heart involvement in systemic sclerosis / Batani, Veronica; Dagna, Lorenzo; De Luca, Giacomo. - In: RHEUMATOLOGY AND IMMUNOLOGY RESEARCH. - ISSN 2719-4523. - 5:2(2024), pp. 72-82. [10.1515/rir-2024-0010]

Therapeutic strategies for primary heart involvement in systemic sclerosis

Batani, Veronica
Primo
;
Dagna, Lorenzo
Secondo
;
De Luca, Giacomo
Ultimo
2024-01-01

Abstract

: Primary heart involvement (pHI) is frequent in systemic sclerosis (SSc), even though often underdiagnosed. SSc-pHI has been recently defined as cardiac abnormalities that are predominantly attributable to SSc rather than other causes and/or complications. SSc-pHI represents a major determinant of mortality in SSc, accounting alone for about 12% of disease-related deaths; its early recognition and promptly therapeutic interventions are therefore crucial. Both perfusion defects and myocardial inflammation contribute to the occurrence of myocardial fibrosis that precipitates myocardial remodeling, potentially leading to heart failure and arrhythmic complications. To date, clear evidence and guidelines for effectively managing SSc pHI are not established yet, resulting in a lack of a defined therapeutic algorithm. In this review we summarize the most recent scientific literature on the prevailing therapeutic strategies and interventions to manage SSc-pHI, with particular focus on therapeutic strategies to counteract the 3 major pathogenic events of the disease, i.e. microvascular damage, myocardial inflammation and myocardial fibrosis.
2024
heart
myocardial fibrosis
myocarditis
primary heart involvement
scleroderma
systemic sclerosis
therapeutic strategies
therapy
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/20.500.11768/168479
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