Cardiovascular Risk In Systemic Sclerosis Research Articles

Systemic Sclerosis and Atherosclerosis: Potential Cellular Biomarkers and Mechanisms.

Systemic sclerosis (SSc) is a rare systemic autoimmune disease of unknown etiology, which is characterized by endothelial dysfunction, pathologic vasculopathy, and increased tissue fibrosis. Traditionally, SSc has been regarded as a prototypical fibrotic disease in the family of systemic autoimmune diseases. Traditionally, emphasis has been placed on the three components of the pathogenesis of SSc: vascular, immune, and mesenchymal. Microvascular lesions, including endothelial dysfunction and smooth muscle cell migration into the intima of vessels in SSc, resemble the atherosclerotic process. Although microvascular disease is a hallmark of SSc, understanding the role of atherosclerotic vascular lesions in patients with SSc remains limited. It is still unknown whether the increased cardiovascular risk in SSc is related to specific cardiac complications (such as myocardial fibrosis) or the accelerated development of atherosclerosis. Different immune cell types appear to be involved in the immunopathogenesis of SSc via the activation of other immune cells, fibrosis, or vascular damage. Macrophages, B cells, T cells, dendritic cells, neutrophils, and endothelial cells have been reported to play the most important role in the pathogenesis of SSc and atherosclerosis. In our article, we reviewed the most significant and recent studies on the pathogenetic links between the development of SSc and the atherosclerotic process.

Peripheral microcirculatory abnormalities are associated with cardiovascular risk in systemic sclerosis: a nailfold video capillaroscopy study.

Microvascular dysfunction is the key element in the pathogenesis of systemic sclerosis (SSc), whereas the contribution of large and medium size vessel abnormalities is yet to be established. The aim of the present study is to assess the association between micro- and macrovascular function by utilizing a broad spectrum of assessments of vascular performance. We included consecutive, consenting SSc patients who underwent nailfold video capillaroscopy (NVC) for microcirculation evaluation. Peripheral and central systolic and diastolic blood pressure, carotid intima-media thickness (cIMT), aortic augmentation index (AIx) corrected for a heart rate of 75 beats per minute (AIx-75), and carotid-femoral pulse wave velocity (PWV) were also performed to assess macrovascular function. Cardiovascular risk disease (CVD) algorithms were also calculated and included in the analysis. A total of 81 patients (6 males) were studied with mean age 55.44 ± 13.40years. Reduced capillary density was inversely correlated with arterial stiffness (Alx-75) and augmentation pressure (r = - 0.262, p = 0.018, and r = - 0.249, p = 0.025 respectively). Alx was significantly lower in the early compared to late pattern (28.24 ± 11.75 vs 35.63 ± 10.47, p = 0.036). A significant trend was found among NVC patterns with Alx-75 values being higher with the progression of microangiopathy towards the "late" group (26.36 ± 10.90 vs 30.81 ± 11.59 vs 35.21 ± 7.90, p = 0.027 for trend). Similarly, Framingham risk score and Atherosclerotic Cardiovascular Disease score were progressively higher across the worsening NVC patterns (4.10 ± 4.13 vs 2.99 ± 2.72 vs 6.36 ± 5.65, p = 0.023, and 6.99 ± 7.18 vs 5.63 ± 4.41 vs 12.09 ± 9.90, p = 0.019, respectively, for trends). Finally, QRISK3 (10-year cardiovascular disease risk) and ASCVD (Atherosclerotic Cardiovascular Disease) scores were inversely correlated with the number of capillaries (r = - 0.231, p = 0.048, and r = - 0.260, p = 0.038 respectively). These data suggest that CVD risk scores and macrovascular parameters are strongly correlated with microvasculopathy in patients with SSc. Key Points • Microangiopathy is the hallmark of SSc, but the relationship between subclinical atherosclerosis and small vessel disease remains unknown. • Arterial stiffening and CVD risk scores are positively associated with the degree of progression of peripheral microvasculopathy assessed with NVC. • The results of the study suggest an association between NVC abnormalities and higher CVD risk in SSc patients.

Cardiovascular Risk in Systemic Sclerosis

Systemic sclerosis (SSc) is a systemic inflammatory, autoimmune disorder characterized by diffuse fibrosis of the skin and visceral organ involvement. Endothelial dysfunction and microvascular injury dominate the pathophysiology and clinical manifestations of the disease, while the impact of macrovascular atherosclerotic disease on cardiovascular (CVD) morbidity and mortality is yet to be established. In this article, we aim to review current knowledge about CVD as well as cardiac complications in SSc and discuss the potentially implicated pathogenetic mechanisms. Systemic inflammation has been identified as an important trigger and contributor for the development and progression of atherosclerosis, closely associated with high cardiovascular mortality in patients with autoimmune disorders, such as rheumatoid arthritis. A close interplay between traditional risk factors and factors related to the disease, including inflammation, endothelial injury, and immune-mediated cytotoxicity, sharing common pathogenetic features with microvasculopathy, may be responsible for large-vessel involvement and promotion of atherosclerosis in SSc. Cardiac complications, including heart failure due to impairment of coronary microcirculation and myocardial fibrosis, are listed among the primary cause of death in SSc. Evaluation of indirect surrogate markers of CVD, namely, arterial stiffness, carotid media thickness, and flow-mediated dilation, in small studies has provided inconsistent results regarding the association between SSc and atherosclerosis, highlighting the need for further research on this field. In this article, we aim to review current knowledge about large-vessel involvement and CVD in SSc and discuss the potentially implicated pathogenetic mechanisms. SSc conveys a higher risk for CVD associated with both vascular and fibrotic complications during the course of the disease. Increasing attention is given on the use of vasodilators, immunosuppressants, and more recently antifibrotic drugs that potentially improve myocardial function and reduce atherosclerotic disease burden.

B-type Natriuretic Peptide as a Marker of Different Forms of Systemic Sclerosis.

SummaryBackgroundSystemic sclerosis (SSc) is an autoimmune connective tissue disease which affects various tissues and organs, including skin, lungs, kidneys, gastrointestinal tract and cardiovascular system. Cardiac involvement is the most commonly recognized problem and a significant cause of morbidity. The brain natriuretic peptide (BNP) is a previously known marker of elevated cardiovascular risk in SSc, but the levels of BNP in various forms of SSc have not been investigated so far.AimThe aim of our study was to evaluate the influence of SSc on the function of the right ventricle and the right atrium using the echocardiographic parameters. Moreover, we examined the levels of BNP in different forms of SSc as well as the association of disease severity with the plasma concentrations of BNP.MethodsWe included 42 patients with newly diagnosed SSc and patients whose disease had been diagnosed earlier. SSc patients and non-SSc control patients were examined by using echocardiography and the concentrations of BNP were determined.ResultsWe analyzed differences in the parameters of right ventricle (RV) function and right atrium (RA) function between SSc patients and healthy controls. The two groups had similar distribution of gender, but SSc patients were significantly older than controls. RV wall thickness was increased in SSc patients (p<0.001), while right ventricular end-systolic area (RVESA; p=0.408) and right ventricular end-diastolic area (RVEDA; p=0.368) did not differ among the examinees. In contrast, RA minor-axis dimension (p=0.001) and the tricuspid annular plane systolic excursion (TAPSE) (p=0.001) were significantly higher in SSc patients. Also, we analyzed differences in brain natriuretic peptide (BNP) concentrations between diffuse cutaneous systemic sclerosis (DSSc) and limited cutaneous systemic sclerosis (LSSc) patients. DSSc patients had significantly higher concentrations of BNP. We found that levels of BNP were in significant positive correlations with age (p=0.007), disease duration (p=0.023), C reactive protein (CRP) (p=0.032), right ventricle fractional area change (FAC) (p=0.022), pulmonary vascular resistance (PVR) and Rodnan score (p=0.019).ConclusionsGiven the obtained results, the laboratory determination of BNP could be useful in differentiating different forms of systemic sclerosis as well as in predicting the severity of the disease and future cardiovascular complications.

Cardiovascular risk in systemic sclerosis: Micro- and Macro-vascular involvement

Systemic sclerosis (SSc) is an autoimmune disease characterized by fibrosis of multiple organs (kidney, heart, lung, gastrointestinal tract, and skin), endothelial damage leading to vascular disease, and autoantibody production. Although the microvascular disease is well-understood, mechanistic insights explaining the presence and extent of macrovascular disease in SSc patients has been a matter of intense debate, especially in the past few years. Patients with systemic sclerosis have an increased risk for atherosclerotic cardiovascular disease (CVD), possibly mediated by inflammatory and fibrotic mechanisms. The excess cardiovascular risk in SSc is suggested by increased arterial stiffness, carotid intima thickening, and reduced flow-mediated dilatation. Given the involvement of the microvasculature, the differentiation between primary and ischemic heart disease is difficult. There is a relative paucity of data regarding clinical and preclinical CVD in SSc. Therefore, large cohort studies are required to clarify whether CVD is predominantly associated with atherosclerosis or microvascular involvement. The aim of this review is to discuss primary and ischemic heart disease and their contribution to CVD in SSc.

A critical view on cardiovascular risk in systemic sclerosis.

Systemic Sclerosis (SSc) is an autoimmune disorder characterized by microvascular injury and diffuse fibrosis of the skin and internal organs. While macrovascular disease and higher risk for cardiovascular events are well documented in other systemic rheumatic diseases such as rheumatoid arthritis and systemic lupus erythematosus, the presence and extent of atherosclerosis among patients with SSc is yet to be established. Primary cardiac involvement, due to impairment of coronary microvascular circulation and myocardial fibrosis, considerably affects prognosis and life expectancy of individuals with SSc, representing one of the leading causes of death in this population. On the other hand the existence and prevalence of atherosclerotic coronary disease remains an issue of debate as studies comparing structural and morphological markers of atherosclerosis and cardiovascular events between SSc patients and the general population have yielded controversial results. The aim of this review is to summarize recent literature about the prevalence of cardiovascular disease in SSc, review the surrogate markers of CVD that have been evaluated and examine whether common pathogenic mechanisms exist between SSc and macrovascular disease.

THU0250 Cardiovascular risk assessment in systemic sclerosis using the score chart and reclassification by presence of plaques on ultrasonography

BackgroundIncreased cardiovascular (CV) risk and atherosclerosis have been described in patients with rheumatic diseases including systemic sclerosis (SSc). Besides traditional CV risk factors, endothelial dysfunction, and disease-specific factors, such as...

Cardiovascular disease in systemic sclerosis - an emerging association?

Microvascular disease is a prominent feature of systemic sclerosis (SSc) and leads to Raynaud's phenomenon, pulmonary arterial hypertension, and scleroderma renal crisis. The presence of macrovascular disease is less well established, and, in particular, it is not known whether the prevalence of coronary heart disease in SSc is increased. Furthermore, in terms of cardiac involvement in SSc, there remains conjecture about the relative contributions of atherosclerotic macrovascular disease and myocardial microvascular disease. In this review, we summarize the literature describing cardiovascular disease in SSc, discuss the pathophysiological mechanisms common to SSc and atherosclerosis, and review the surrogate markers of cardiovascular disease which have been examined in SSc. Proposed mediators of the vasculopathy of SSc which have also been implicated in atherosclerosis include endothelial dysfunction, a reduced number of circulating endothelial progenitor cells, and an increased number of microparticles. Excess cardiovascular risk in SSc is suggested by increased arterial stiffness and carotid intima thickening and reduced flow-mediated dilatation. Cohort studies of adequate size are required to resolve whether this translates into an increased incidence of cardiovascular events in patients with SSc.