Abstract
The prognosis for patients with systemic sclerosis (SSc) has improved in the past three decades, with fewer patients succumbing to renal-crisis-related death. While pulmonary fibrosis and hypertension are currently the most frequent causes of death, there is evidence that cardiovascular disease will have an important role in the long-term prognosis of SSc in the future. Ischemia–reperfusion injury and endothelial dysfunction are cardinal features of SSc, and may predispose a patient to microvascular disease and atherosclerosis. In order to alleviate the cardiovascular burden in patients with SSc, it is important to detect endothelial dysfunction, microvascular flow disturbance and atherosclerosis. Noninvasive techniques that evaluate flow-mediated dilatation and arterial pulse waves (endothelial function measurements), microvascular blood flow (measurement of the microcirculation), carotid intima–media thickness and left ventricular hypertrophy (detection of atherosclerosis) are recommended. In addition, these measurements will facilitate trials of therapeutic strategies that, in addition to controlling conventional risk factors, prevent and treat cardiovascular disease in patients with SSc.
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References
Szekanecz, Z. & Koch, A. E. Vascular involvement in rheumatic diseases: 'vascular rheumatology'. Arthritis Res. Ther. 10, 224 (2008).
Karassa, F. B. & Ioannidis, J. P. Mortality in systemic sclerosis. Clin. Exp. Rheumatol. 26 (5 Suppl. 51), S85–S93 (2008).
Steen, V. D. & Medsger, T. A. Changes in causes of death in systemic sclerosis, 1972–2002. Ann. Rheum. Dis. 66, 940–944 (2007).
Ferri, C. et al. Systemic sclerosis: demographic, clinical and serological features and survival in 1,012 Italian patients. Medicine (Baltimore) 81, 139–153 (2002).
Hettema, M. E., Bootsma, H. & Kallenberg, C. G. M. Macrovascular disease and atherosclerosis in SSc. Rheumatology (Oxford) 47, 578–583 (2008).
Fukuda, D., Enomoto, S., Nagai, R. & Sata, M. Inhibition of renin–angiotensin system attenuates periadventitial inflammation and reduces atherosclerotic lesion formation. Biomed. Pharmacother. 63, 754–761 (2009).
Youssef, P., Brama, T., Englert, H. & Bertouch, J. Limited scleroderma is associated with increased prevalence of macrovascular disease. J. Rheumatol. 22, 469–472 (1995).
Ho, M., Veale, D., Eastmond, C., Nuki, G. & Belch, J. Macrovascular disease and systemic sclerosis. Ann. Rheum. Dis. 59, 39–43 (2000).
Sulli, A. et al. Blunted coronary flow reserve in systemic sclerosis. Rheumatology (Oxford) 43, 505–509 (2004).
Khurma, V. et al. A pilot study of subclinical coronary atherosclerosis in systemic sclerosis: coronary artery calcification in cases and controls. Arthritis Rheum. 59, 591–597 (2008).
Jacobsen, S., Halberg, P. & Ullman, S. Mortality and causes of death of 344 Danish patients with systemic sclerosis (scleroderma). Br. J. Rheumatol. 37, 750–755 (1993).
Ad-Dhaher, F. F., Pope, J. E. & Oiumet, J. M. Determinants of morbidity and mortality of systemic sclerosis in Canada. Semin. Arthritis Rheum. 39, 269–277 (2010).
Akram, M. R. et al. Angiographically proven coronary artery disease in scleroderma. Rheumatology (Oxford) 45, 1395–1398 (2006).
Follansbee, W. P. et al. Physiologic abnormalities of cardiac function in progressive systemic sclerosis with diffuse scleroderma. N. Engl. J. Med. 310, 142–148 (1984).
Follansbee, W. P. et al. A controlled clinicopathologic study of myocardial fibrosis in systemic sclerosis (scleroderma). J. Rheumatol. 17, 656–662 (1990).
Abraham, D. J., Krieq, T., Distler, J. & Distler, O. Overview of pathogenesis of systemic sclerosis. Rheumatology (Oxford) 48 (Suppl. 3), 3–7 (2009).
Le Brocq, M., Leslie, S. J., Milliken, P. & Megson, I. L. Endothelial dysfunction: from molecular mechanisms to measurement, clinical implications, and therapeutic opportunities. Antioxid. Redox Signal. 10, 1631–1674 (2008).
Derrett-Smith, E. C., Denton, C. P. & Sonnylal, S. Animal models of scleroderma: lessons from transgenic and knockout mice. Curr. Opin. Rheumatol. 21, 630–635 (2009).
Doria, A., Sherer, Y., Meroni, P. L. & Shoenfeld, Y. Inflammation and accelerated atherosclerosis: basic mechanisms. Rheum. Dis. Clin. N. Am. 31, 355–362 (2005).
Boin, F. et al. Independent association of anti-β2-glycoprotein I antibodies with macrovascular disease and mortality in scleroderma patients. Arthritis Rheum. 60, 2480–2489 (2009).
Celermajer, D. S., Sorensen, K. E., Bull, C., Robinson, J. & Deanfield, J. E. Endothelium-dependent dilation in the systemic arteries of asymptomatic subjects relates to coronary risk factors and their interaction. J. Am. Coll. Cardiol. 24, 1468–1474 (1994).
Bartoli, F. et al. Flow-mediated vasodilation and carotid intima–media thickness in systemic sclerosis. Ann. NY Acad. Sci. 1108, 283–290 (2007).
Szücs, G. et al. Endothelial dysfunction precedes atherosclerosis in systemic sclerosis—relevance for prevention of vascular complications. Rheumatology (Oxford) 46, 759–762 (2007).
Timár, O. et al. Increased arterial stiffness as the marker of vascular involvement in systemic sclerosis. J. Rheumatol. 35, 1329–1333 (2008).
Turner, J., Belch, J. J. & Khan, F. Current concepts in assessment of microvascular endothelial function using laser Doppler imaging and iontophoresis. Trends Cardiovasc. Med. 18, 109–116 (2008).
Murray, A. K. et al. Noninvasive imaging techniques in the assessment of scleroderma spectrum disorders. Arthritis Rheum. 61, 1103–1111 (2009).
de Groot, E. et al. Measurement of carotid intima–media thickness to assess progression and regression of atherosclerosis. Nat. Clin. Pract. Cardiovasc. Med. 5, 280–288 (2008).
de Groot, P. et al. Evaluation of cardiac abnormalities by Doppler echocardiography in a large nationwide multicentric cohort of patients with systemic sclerosis. Ann. Rheum. Dis. 67, 31–36 (2008).
Greenland, P. et al. ACCF/AHA 2007 clinical expert consensus document on coronary artery calcium scoring by computed tomography in global cardiovascular risk assessment and in evaluation of patients with chest pain: a report of the American College of Cardiology Foundation Clinical Expert Consensus Task Force (ACCF/AHA Writing Committee to Update the 2000 Expert Consensus Document on Electron Beam Computed Tomography) developed in collaboration with the Society of Atherosclerosis Imaging and Prevention and the Society of Cardiovascular Computed Tomography. J. Am. Coll. Cardiol. 49, 378–402 (2007).
Mok, M. Y. et al. Coronary atherosclerosis using computed tomography coronary angiography in patients with systemic sclerosis. Scand. J. Rheumatol. 38, 381–385 (2009).
Kobaysahi, H. et al. Cardiac magnetic resonance imaging with pharmacological stress perfusion and delayed enhancement in asymptomatic patients with systemic sclerosis. J. Rheumatol. 36, 106–112 (2009).
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Mok, M., Lau, C. The burden and measurement of cardiovascular disease in SSc. Nat Rev Rheumatol 6, 430–434 (2010). https://doi.org/10.1038/nrrheum.2010.65
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DOI: https://doi.org/10.1038/nrrheum.2010.65
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