期刊
BEST PRACTICE & RESEARCH IN CLINICAL RHEUMATOLOGY
卷 35, 期 3, 页码 -出版社
ELSEVIER SCI LTD
DOI: 10.1016/j.berh.2021.101668
关键词
Systemic sclerosis (scleroderma); Organ involvement; Cardiac disease; Heart involvement
类别
资金
- Gruppo Italiano Lotta alla Sclerodermia (GILS)
- Fondazione Italiana Ricerca sull'Artrite (FIRA)
- European Scleroderma Trial and Research (EUSTAR) group
- New Horizon Fellowship Foundation
- Foundation for Research in Rheumatology (FOREUM)
- Musculoskeletal Australia PhD Scholarship
- Australian Government Research and Training Scholarship
Primary systemic sclerosis heart involvement is an important manifestation of systemic sclerosis, with a broad clinical spectrum ranging from asymptomatic perfusion abnormalities to heart failure. Early signs may be subtle, and early identification can provide an opportunity for intervention to prevent heart failure, although optimal screening and treatment guidelines are currently lacking.
Primary systemic sclerosis heart involvement (pSHI) is an important disease manifestation that accounts for a significant proportion of systemic sclerosis (SSc)-associated mortality. A broad clinical spectrum of pSHI exists, which ranges from asymptomatic perfusion abnormalities to diastolic dysfunction or acute myocarditis and congestive heart failure. With improving sensitivity of cardiac investigations, it is increasingly recognized that there is a large burden of subclinical cardiac disease in patients with SSc. Early signs of pSHI can be subtle and determining the etiology of cardiac abnormalities from other causes of cardiomyopathy such as hypertension, ischemic heart disease (IHD), and pulmonary vascular disease remain challenging. Early identification of pSHI potentially provides clinicians with a window of opportunity for intervention to avert progression to heart failure. However, optimal screening and treatment guidelines are lacking, and it is an area of much needed further clinical research. (C) 2021 Elsevier Ltd. All rights reserved.
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