Department of Experimental Medicine, Division of Rheumatology, University of Florence, Via Delle Oblate 4, 50141, Florence, Italy.
Department of Medicine, The University of Melbourne at St Vincent's Hospital, 41 Victoria Pde Fitzroy VIC, 3065, Australia; Department of Rheumatology, St Vincent's Hospital, Melbourne 41 Victoria Pde Fitzroy VIC, 3065, Australia.
Best Pract Res Clin Rheumatol. 2021 Sep;35(3):101668. doi: 10.1016/j.berh.2021.101668. Epub 2021 Mar 15.
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.
原发性系统性硬化症心脏受累（pSHI）是一种重要的疾病表现，占系统性硬化症（SSc）相关死亡率的很大比例。pSHI 存在广泛的临床谱，从无症状的灌注异常到舒张功能障碍或急性心肌炎和充血性心力衰竭。随着心脏检查敏感性的提高，越来越多的人认识到 SSc 患者存在大量亚临床心脏疾病。pSHI 的早期迹象可能很微妙，确定心脏异常的病因是具有挑战性的，因为这些病因可能来自于其他原因，如高血压、缺血性心脏病（IHD）和肺血管疾病。早期识别 pSHI 可能为临床医生提供干预机会，以避免进展为心力衰竭。然而，目前缺乏最佳的筛查和治疗指南，这是一个急需进一步临床研究的领域。
