Physiologic Abnormalities of Cardiac Function in Progressive Systemic Sclerosis with Diffuse Scleroderma

Follansbee, William P.; Curtiss, Edward I.; Medsger, Thomas A.; Steen, Virginia D.; Uretsky, Barry F.; Owens, Gregory R.; Rodnan, Gerald P.

doi:10.1056/nejm198401193100302

Cited by 222 publications

(113 citation statements)

References 29 publications

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“…Although the findings from Doppler echocardiographic studies (7-1 l), radionuclide ventriculography (12-14,16), and perfusion scintigraphy (15)(16)(17)(18)(19)(20)(21)(22) in the assessment of cardiac involvement in limited SSc have been evaluated in different reports, studies providing a comprehensive approach to these noninvasive investigations in limited SSc are not available. In the present study, when patients with limited SSc were compared with those in the control group (Tables 1 and 2), the most remarkable Doppler echocardiographic findings were the high prevalence of abnormal left and right diastolic parameters, mitral regurgitation, and mitral subvalvular thickening.…”

Section: Discussionmentioning

confidence: 99%

“…Some studies using Doppler echocardiography (12) and radionuclide ventriculography (11, [13][14][15] have disclosed diastolic function abnormalities in SSc patients. Radionuclide perfusion studies using thallium-201 (11, [16][17][18][19][20][21][22] or positron emission tomography (23) have shown perfusion defects that, in the absence of coronary lesions, may be attributed to a local coronary spasm. These sustained or repeated episodes of vasospasm may be responsible for the myocardial fibrosis and contraction band necrosis that can be found in patients with SSc.…”

mentioning

confidence: 99%

“…Most of these studies (9,10,12, 13,16,[19][20][21]23) were exclusively focused on diffuse forms of SSc. while limited SSc, which is found in up to 80% of such patients and has a better prognosis, has not been studied separately.…”

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Comprehensive noninvasive assessment of cardiac involvement in limited systemic sclerosis

Candell‐Riera

Armadans-Gil

Simeon

et al. 1996

Arthritis & Rheumatism

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Objective. To assess cardiovascular abnormalities in patients with limited systemic sclerosis (SSc), using noninvasive cardiac techniques.Methods. Sixty‐three patients with limited SSc were prospectively evaluated with Doppler echocardiography and thallium‐201 perfusion scintigraphy after a cold‐stress test and radionuclide ventriculography.Results. In the patients with limited SSc, there was a significantly high prevalence of abnormal left‐ and right‐diastolic function parameters (P = 0.001 and P = 0.0002, respectively), thickening of papillary muscles (46%; P = 0.003), and mild mitral regurgitation (49%; P < 0.0001), compared with controls. Systolic pulmonary arterial hypertension was detected in 9 patients (14%), and pericardial effusion in 11 patients (18%). In 64% of patients with limited SSc, an ischemic response was detected on the thallium cold‐stress scan; similarly, an ischemic response was detected in 57% of patients with primary Raynaud's phenomenon (P < 0.0001 versus controls).Conclusion. Although the frequency of cardiovascular symptoms was low in patients with limited SSc, a significant rate of cardiovascular abnormalities was found by noninvasive cardiac techniques.

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Section: Discussionmentioning

confidence: 99%

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confidence: 99%

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Comprehensive noninvasive assessment of cardiac involvement in limited systemic sclerosis

Candell‐Riera

Armadans-Gil

Simeon

et al. 1996

Arthritis & Rheumatism

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“…Impaired coronary artery reserve has previously been demonstrated in SSc patients by noninvasive imaging techniques (5)(6)(7). Studies published a few decades ago suggested an underlying microvascular disease of the myocardium, based on normal findings on coronary angiogram (8) and the absence of coronary atherosclerosis upon histologic examination at autopsy (9)(10)(11)(12). However, clinical and subclinical coronary artery disease (CAD) is increasingly reported in SSc patients (13)(14)(15), although the prevalence of coronary atherosclerosis and the predisposing factors in SSc patients remain unknown.…”

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Systemic sclerosis is an independent risk factor for increased coronary artery calcium deposition

Mok¹,

Lau²,

Chiu³

et al. 2011

Arthritis & Rheumatism

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Objective. Endothelial dysfunction and inflammation are pathogenic mechanisms common to systemic sclerosis (SSc) and atherosclerosis. This study was undertaken to examine the relationship between coronary atherosclerosis, as assessed by the coronary artery calcium score (CACS), and conventional cardiovascular and disease-specific risk factors in SSc patients.Methods. The CACS was measured by computed tomography, and cardiovascular risk factors were examined in SSc patients and compared with controls matched for age, sex, and glycemic status. Disease activity score, antiphospholipid antibodies, highsensitivity C-reactive protein level, and erythrocyte sedimentation rate were measured in SSc patients. Odds ratios (ORs) and 95% confidence intervals (95% CIs) were determined.Results. We recruited 53 SSc patients (50 women and 3 men) and 106 controls. The patients had a mean ؎ SD age of 53.1 ؎ 12.9 years and a median disease duration of 9 years. Compared to controls, SSc patients had significantly lower low-density lipoprotein (LDL) cholesterol levels (P ‫؍‬ 0.001), high-density lipoprotein cholesterol levels (P ‫؍‬ 0.01), diastolic blood pressure, waist circumference, and body mass index and were more likely to be receiving vasodilators (all P < 0.001). There was a significantly higher proportion of SSc patients among subjects with more severe coronary calcification (CACS >101) compared to those with lesser severity (CACS <100) (56.5% versus 29.4%; P ‫؍‬ 0.01). Multiple logistic regression analysis revealed SSc to be an independent determinant for a CACS >101 (OR 10.89 [95% CI 2.21-53.75], P ‫؍‬ 0.003) together with age and LDL cholesterol level after adjustment for other cardiovascular risk factors. Among diseasespecific factors, only disease duration (OR 1.14 [95% CI 1.02-1.27], P ‫؍‬ 0.02) was independently associated with more severe coronary calcification (CACS >101).Conclusion. Our findings indicate that SSc is an independent risk factor for coronary calcification, in addition to the conventional risk factors for coronary atherosclerosis, such as age and hypertension.

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“…Arteriolar vasospasm that is analogous to Raynaud's phenomenon (RP) may lead to reversible attacks of ischemia in response to cold provocation. Alterations of the microvasculature may play a crucial role in the development of SScMI [11][12][13][14][15][16][17]. Observations suggest that coronary flow reserve (CFR) is reduced in SSc despite the lack of atherosclerotic CAD [17][18][19].…”

Section: Introductionmentioning

confidence: 99%

Diagnosis and mechanisms of cardiac involvement in patients with systemic sclerosis

Pintér

Komócsi

2012

IMAS

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Abstract:Introduction: Systemic sclerosis (SSc) is characterized by fibrosis and vascular abnormalities of skin and internal organs. Cardiac involvement includes coronary artery disease (CAD), pulmonary arterial hypertension (PAH) related right ventricular changes and microvascular disease (MVD). Aims: To characterize the distribution of cardiac alterations and to investigate the mechanisms of the microvascular impairment of SSc patients. Methods: Based on non-invasive investigations, patients were selected for right heart catheterization and intracoronary pressure-wire supplemented coronary angiography. Results: 17 SSc patients (selected from 120 cases) and 17 controls were enrolled. In the "suspected PAH" and the "suspected CAD" groups, PAH was found in 12/20 and 2/10 cases, and coronary artery stenosis in 9/20 and 6/10 cases, respectively. Patients with decreased coronary flow reserve (CFR) had accelerated flow velocity (p<0.05), but myocardial resistance index (IMR) in hyperemia did not differ from patients with normal CFR or from the controls (p=0.292 and p=0.308). The coronary flow velocity of SSc patients correlated to the IMR at baseline (r=0.56, p=0.019). Conclusions: PAH, CAD and MVD show an overlap in symptomatic SSc. The maintained vasodilatation response indicates the paucity of irreversible functional damage of the coronary arterioles. The reduced CFR, the decreased basal IMR and higher velocity pinpoint to possible compensatory vasodilatory mechanisms.

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Physiologic Abnormalities of Cardiac Function in Progressive Systemic Sclerosis with Diffuse Scleroderma

Cited by 222 publications

References 29 publications

Comprehensive noninvasive assessment of cardiac involvement in limited systemic sclerosis

Comprehensive noninvasive assessment of cardiac involvement in limited systemic sclerosis

Systemic sclerosis is an independent risk factor for increased coronary artery calcium deposition

Diagnosis and mechanisms of cardiac involvement in patients with systemic sclerosis

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