Abstract
BackgroundSystemic sclerosis (SSc) is characterised by multi-organ tissue fibrosis including the myocardium. Diffuse myocardial fibrosis can be detected non-invasively by T1 and extracellular volume (ECV) quantification, while focal myocardial inflammation and fibrosis may be detected by T2-weighted and late gadolinium enhancement (LGE), respectively, using cardiovascular magnetic resonance (CMR). We hypothesised that multiparametric CMR can detect subclinical myocardial involvement in patients with SSc.Methods19 SSc patients (18 female, mean age 55 ± 10 years) and 20 controls (19 female, mean age 56 ± 8 years) without overt cardiovascular disease underwent CMR at 1.5T, including cine, tagging, T1-mapping, T2-weighted, LGE imaging and ECV quantification.ResultsFocal fibrosis on LGE was found in 10 SSc patients (53%) but none of controls. SSc patients also had areas of myocardial oedema on T2-weighted imaging (median 13 vs. 0% in controls). SSc patients had significantly higher native myocardial T1 values (1007 ± 29 vs. 958 ± 20 ms, p < 0.001), larger areas of myocardial involvement by native T1 >990 ms (median 52 vs. 3% in controls) and expansion of ECV (35.4 ± 4.8 vs. 27.6 ± 2.5%, p < 0.001), likely representing a combination of low-grade inflammation and diffuse myocardial fibrosis. Regardless of any regional fibrosis, native T1 and ECV were significantly elevated in SSc and correlated with disease activity and severity. Although biventricular size and global function were preserved, there was impairment in the peak systolic circumferential strain (-16.8 ± 1.6 vs. -18.6 ± 1.0, p < 0.001) and peak diastolic strain rate (83 ± 26 vs. 114 ± 16 s-1, p < 0.001) in SSc, which inversely correlated with diffuse myocardial fibrosis indices.ConclusionsCardiac involvement is common in SSc even in the absence of cardiac symptoms, and includes chronic myocardial inflammation as well as focal and diffuse myocardial fibrosis. Myocardial abnormalities detected on CMR were associated with impaired strain parameters, as well as disease activity and severity in SSc patients. CMR may be useful in future in the study of treatments aimed at preventing or reducing adverse myocardial processes in SSc.
Highlights
Systemic sclerosis (SSc) is characterised by multi-organ tissue fibrosis including the myocardium
Baseline characteristics of the patient population The SSc patients were well-matched with controls for age, sex and comorbidities and only a small minority of patients were on regular disease modifying anti-rheumatic drugs (Table 1)
In conclusion, subclinical myocardial involvement is common in SSc patients without cardiac symptoms, as measured by T2-weighted imaging, native T1, quantitative late gadolinium enhancement (LGE) and extracellular volume (ECV) measurement; and likely signifies a combination of myocardial inflammation and diffuse fibrosis which correlated with both SSc disease activity and skin fibrosis severity, as well as with subclinical impairment of systolic and diastolic strain despite the preserved Left ventricular (LV) ejection fraction
Summary
Systemic sclerosis (SSc) is characterised by multi-organ tissue fibrosis including the myocardium. We hypothesised that multiparametric CMR can detect subclinical myocardial involvement in patients with SSc. Systemic sclerosis (SSc) is an autoimmune connective tissue disorder characterised by vascular dysfunction and multi-organ fibrosis. The heart is one of the major organs commonly involved in SSc, with an estimated clinical prevalence of 15-35% [1]. Cardiovascular disease (CVD) in SSc may be direct (cardiac fibrosis, myocarditis, dilated cardiomyopathy, cardiac failure, premature coronary artery disease, conduction system abnormalities, valvular disease and pericardial disease) or indirect (pulmonary hypertension and renal crisis) [2,3]. In the majority of SSc patients, CVD often remains subclinical [4]. SSc patients with apparent cardiovascular clinical features are at greater risk of deterioration and premature cardiovascular death [5]. Early detection and monitoring of myocardial and vascular involvement is a crucial aspect of management [6]
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