Cardiac involvement in systemic sclerosis: Getting to the heart of the matter

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.

Automated summary

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.