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Vol. 2. Núm. S3.
Esclerosis sistémica
Páginas S31-S36 (Noviembre 2006)
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Vol. 2. Núm. S3.
Esclerosis sistémica
Páginas S31-S36 (Noviembre 2006)
Esclerosis sistémica
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Afección cardíaca en la esclerosis sistémica
Cardiac involvement in systemic sclerosis
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Luis H. Silveira-Torre
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luis_hsil@yahoo.com

Correspondencia: Dr. L.H. Silveira-Torre. Departamento de Bioquímica. Instituto Nacional de Cardiología Ignacio Chávez. Juan Badiano, 1. Colonia Sección XVI. 14080 México DF. México.
Departamento de Bioquímica. Instituto Nacional de Cardiología Ignacio Chávez. México DF. México
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La esclerosis sistémica (ES) puede afectar al pericardio, el miocardio, las arterias coronarias, el sistema de conducción y las válvulas cardíacas. La presencia de síntomas y signos es de mal pronóstico. Las manifestaciones incluyen disnea, palpitaciones, dolor precordial, síncope y datos de insuficiencia cardíaca derecha.

La prevalencia de la enfermedad pericárdica con manifestaciones clínicas varía entre el 5 y el 16%; con ecocardiografía, es del 5,4-41% y en estudios de necropsia, es del 33-77,5%. La afección miocárdica característica de la ES es la fibrosis en parches. El hallazgo patológico típico es la “necrosis en banda de contracción”. Las consecuencias importantes de la fibrosis incluyen hipertrofia ventricular izquierda, así como disfunción tanto sistólica como diastólica en ambos ventrículos. La detección en fases tempranas de estas alteraciones es muy importante, sobre todo de la disfunción diastólica, que ocurre mucho antes que la sistólica, por lo que podría predecir el desarrollo de daño cardíaco importante. Se ha descrito la asociación de miositis esquelética y cardíaca. La miopatía esquelética aumenta el riesgo de insuficiencia cardíaca congestiva, arritmias sintomáticas sostenidas y muerte súbita de origen cardíaco. Los vasos pequeños miocárdicos están afectados en la ES; las arterias coronarias epicárdicas no se afectan. Se presentan arritmias, principalmente extrasístoles auriculares y ventriculares, así como alteraciones de la conducción. La afección de las válvulas cardíacas es mínima, y la mitral es la que se afecta con mayor frecuencia. Otras alteraciones que se ha comunicado en relación con la ES son la rigidez de las arterias periféricas de gran calibre y la afección secundaria del corazón por hipertensión arterial pulmonar y sistémica. La afección cardíaca es causa importante de morbimortalidad en la ES.

Palabras clave:
Corazón
Esclerosis sistémica
Pericardio
Miocardio
Válvulas cardíacas
Cardiopatía isquémica

Systemic sclerosis (SS) can involve the pericardium, myocardium, conduction system, and cardiac valves. The presence of overt clinical signs of cardiac disease is a poor prognostic sign. Clinical manifestations include dyspnea, palpitations, chest pain, syncope, and symptoms of right heart failure.

Prevalence of clinically symptomatic pericardial disease is 5-16%. However, ecocardiographic prevalence is 5.4- 41% and at autopsy is 33-77.5%. Patchy fibrosis is the characteristic myocardial finding in SS. Contraction band necrosis is the typical pathological finding. Important complications of fibrosis include left ventricular hypertrophy, as well as systolic and diastolic dysfunction of both ventricles. Early detection of these abnormalities is very important, mainly of the diastolic dysfunction, since it occurs before the systolic dysfunction and can predict important cardiac damage. Association of skeletal myositis with myocardial disease has been described. Patients with skeletal myositis are more likely to develop congestive heart failure, sustained symptomatic arrythmias, and cardiac sudden death. Coronary arteries are normal in systemic sclerosis, but there is no endomyocardial vessel involvement. There is an increased prevalence of arrhytmias, mainly premature atrial and ventricular contractions, as well as conduction system disease. Cardiac valvular involvement is minor in systemic sclerosis; mitral valve is the most frequently affected. Other abnormalities described in this disease include peripheral large vessels stiffness and secondary cardiac involvement due to pulmonary and systemic arterial hypertension. Cardiac involvement confers a high morbi-mortality risk in systemic sclerosis.

Key words:
Heart
Systemic sclerosis
Pericardium
Myocardium
Cardiac valves
Ischemic cardiopathy
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Bibliografía
[1.]
S. Oram, W. Stokes.
The heart in slceroderma.
Br Heart J, 23 (1961), pp. 243-259
[2.]
A. Deswal, W.P. Follansbee.
Cardiac involvement in scleroderma.
Rheum Dis Clin North Am, 22 (1996), pp. 841-860
[3.]
P.J. Clements, P.A. Lachenbruch, D.E. Furst, H.E. Paulus, M.G. Sterz.
Cardiac score. A semiquantitative measure of cardiac involvement that improves prediction of prognosis in systemic sclerosis.
Arthritis Rheum, 34 (1991), pp. 1371-1380
[4.]
F.M. Wigley, L.K. Hummers.
Clinical features of systemic sclerosis.
Rheumatology, 3.ª ed., pp. 1463-1479
[5.]
P.J. Clements, D.E. Furst, W. Cabeen, D. Tashkin, H.E. Paulus, N. Roberts.
The relationship of arrhytmias and conduction disturbances to other manifestations of cardiopulmonary disease in progressive systemic sclerosis (PSS).
Am J Med, 71 (1981), pp. 38-46
[6.]
C. Ferri, L. Bernini, M.G. Bongiorni, D. Levorato, G. Viegi, P. Bravi, et al.
Noninvasive evaluation of cardiac dysrhythmias, and their relationship with multisystemic symptoms, in progressive systemic sclerosis patients.
Arthritis Rheum, 28 (1985), pp. 259-266
[7.]
B.H. Bulkley, P.G. Klacsmann, G.M. Hutchins.
Angina pectoris, myocardial infarction, and sudden cardiac death with normal coronary arteries: a clinicopathologic study of nine patients with progressive systemic sclerosis.
Am Heart J, 95 (1978), pp. 563-569
[8.]
A.E. Thompson, J.E. Pope.
A study of the frequency of pericardial and pleural effusions in scleroderma.
Br J Rheumatol, 37 (1998), pp. 1320-1323
[9.]
G.S. Uhl, G.M. Koppes.
Pericardial tamponade in systemic sclerosis (scleroderma).
Br Heart J, 42 (1979), pp. 345-348
[10.]
M. Satoh, M. Tokuhira, N. Hama, M. Hirakata, M. Kuwana, M. Akizuki, et al.
Massive pericardial effusion in scleroderma: a review of five cases.
Br J Rheumatol, 34 (1995), pp. 564-567
[11.]
H. Bouraui, B. Trimeche, A. Mahdhaoui, S. Ernez-Hajri, G. Jeridi, H. Ammar.
Cardiac tamponade revealing systemic scleroderma.
Rev Med Suisse Romande, 123 (2003), pp. 713-714
[12.]
R.M. Gowda, I.A. Khan, T.J. Sacchi, B.C. Vasavada.
Scleroderma pericardial disease presented with a large pericardial effusion -a case report.
Angiology, 52 (2001), pp. 59-62
[13.]
G. Peronato, P. De Sandre, M. Podswiadek, S. Arfiero, L. Bozzola.
Cardiac tamponade preceding skin involvement in progressive systemic sclerosis.
Reumatismo, 54 (2002), pp. 257-260
[14.]
R.M. Gokula, A.K. Gupta, S.E. Shirley, K. Coard, P.S. Ramphal.
Scleroderma with cardiac tamponade, hyperthyroidism and incidental papillary thyroid carcinoma.
West Indian Med J, 51 (2002), pp. 188-190
[15.]
F. Allali, M. Alami, N. Doghmi, A. Mohatane, M. Benomar, N. Hajjaj-Hassounni.
Scleroderma complicated with tamponade during pregnancy.
Joint Bone Spine, 72 (2005), pp. 341-343
[16.]
R.J. Byers, D.A.S. Marshall, A.J. Freemont.
Pericardial involvement in systemic sclerosis.
Ann Rheum Dis, 56 (1997), pp. 393-394
[17.]
W.A. D’Angelo, J.F. Fries, A.T. Masi, L.E. Shulman.
Pathologic observations in systemic sclerosis (scleroderma). A study of fifty-eight autopsy cases and fifty-eight matched controls.
Am J Med, 46 (1969), pp. 428-440
[18.]
W.P. Follansbee, T.R. Miller, E.I. Curtiss, J.E. Orie, R.L. Bernestein, J.M. Kiernan, et al.
A controlled clinicopathologic study of myocardial fibrosis in systemic sclerosis (scleroderma).
J Rheumatol, 17 (1990), pp. 656-662
[19.]
B.H. Bulkley, R.L. Ridolfi, W.R. Salyer, G.M. Hutchins.
Myocardial lesions of progressive systemic sclerosis. A cause of cardiac dysfunction.
Circulation, 53 (1976), pp. 483-490
[20.]
G.P. Rodnan, R.L. Myerowitz, G.O. Justh.
Morphologic changes in the digital arteries of patients with progressive systemic sclerosis (scleroderma) and Raynaud phenomenon.
Medicine (Baltimore), 59 (1980), pp. 393-408
[21.]
B. Janevski.
Arteries of the hand in patients with scleroderma.
Diag Imaging Clin Med, 55 (1986), pp. 262-265
[22.]
J.S. Gottdiener, H.M. Moutsopoulos, J.L. Decker.
Echocardiographic identification of cardiac abnormality in scleroderma and related disorders.
Am J Med, 66 (1979), pp. 391-398
[23.]
R.J. Siegel, B. O’Connor, I. Mena, J.M. Criley.
Left ventricular function at rest and during Raynaud’s phenomenon in patients with scleroderma.
Am Heart J, 108 (1984), pp. 1469-1476
[24.]
I. Hegedus, L. Czirjak.
Left ventricular wall motion abnormalities in 80 patients with systemic sclerosis.
Clin Rheumatol, 14 (1995), pp. 161-164
[25.]
W.P. Follansbee, E.I. Curtiss, T.A. Medsger Jr, V.D. Steen, B.F. Uretsky, G.R. Owens, et al.
Physiologic abnormalities of cardiac function in progressive systemic sclerosis with diffuse scleroderma.
N Eng J Med, 310 (1984), pp. 142-148
[26.]
G. Valentini, D.F. Vitale, A. Giunta, S. Maione, G. Gerundo, M. Arnese, et al.
Diastolic abnormalities in systemic sclerosis: evidence for associated defective cardiac functional reserve.
Ann Rheum Dis, 55 (1996), pp. 455-460
[27.]
G.P. Armstrong, G.A. Whalley, R.N. Doughty, G.D. Gamble, S.M. Flett, P.L. Tan, et al.
Left ventricular function in scleroderma.
Br J Rheumatol, 35 (1996), pp. 983-988
[28.]
G. Aguglia, A. Sgreccia, M.L. Bernardo, E. Carmenini, M. Giusti De Marle, A. Reali, et al.
Left ventricular diastolic function in systemic sclerosis.
J Rheumatol, 28 (2001), pp. 1563-1567
[29.]
K. Nakajima, J. Taki, M. Kawano, T. Higuchi, S. Sato, J. Nishijima, et al.
Diastolic dysfunction in patients with systemic sclerosis detected by gated myocardial perfusion SPECT: an early sign of cardiac involvement.
J Nucl Med, 42 (2001), pp. 183-188
[30.]
S. Maione, G. Cuomo, A. Giunta, L. Tanturri de Horatio, G. La Montagna, F. Manguso, et al.
Echocardiographic alterations in systemic sclerosis: A longitudinal study.
Semin Arthritis Rheum, 34 (2005), pp. 721-727
[31.]
C. Meune, Y. Allanore, J.Y. Devaux, O. Dessault, D. Duboc, S. Weber, et al.
High prevalence of right ventricular systolic dysfunction in early systemic sclerosis.
J Rheumatol, 31 (2004), pp. 1941-1945
[32.]
A. Giunta, E. Tirri, S. Maione, S. Canganiello, A. Mele, A. De Luca, et al.
Right ventricular diastolic abnormalities in systemic sclerosis. Relation to left ventricular involvement and pulmonary hypertension.
Ann Rheum Dis, 59 (2000), pp. 94-98
[33.]
P. Lindquist, K. Caidahl, G. Neuman-Andersen, C. Ozolins, S. Rantapää-Dahlquist, A. Waldenström, et al.
Disturbed right ventricular diastolic function in patients with systemic sclerosis: A Doppler tissue imaging study.
Chest, 128 (2005), pp. 755-763
[34.]
S.G. West, P.J. Killian, D.J. Lawless.
Association of myositis and myocarditis in progressive systemic sclerosis.
Arthritis Rheum, 24 (1981), pp. 662-667
[35.]
S. Carette, J. Turcotte, G. Matho.
Severe myositis and myocarditis in progressive systemic sclerosis.
J Rheumatol, 12 (1985), pp. 997-999
[36.]
W.P. Follansbee, T.R. Zerbe, T.A. Medsger Jr.
Cardiac and skeletal muscle disease in systemic sclerosis (scleroderma): a righ risk association.
Am Heart J, 125 (1993), pp. 194-203
[37.]
A. Long, G. Duffy, B. Bresnihan.
Reversible myocardial perfusion defects during cold challenge in scleroderma.
Br J Rheumatol, 25 (1986), pp. 158-161
[38.]
R. Gustafsson, F. Mannting, E. Kazzam, A. Waldenstrom, R. Hallgren.
Cold-induced reversible myocardial ischaemia in systemic sclerosis.
Lancet, 2 (1989), pp. 475-479
[39.]
E.L. Alexander, G.S. Firestein, J.L. Weiss, R.R. Heuser, G. Leitl, H.N. Wagner Jr, et al.
Reversible cold-induced abnormalities in myocardial perfusion and function in systemic sclerosis.
Ann Intern Med, 105 (1986), pp. 661-668
[40.]
W.W. Ellis, A.N. Baer, R.M. Robertson, T. Pincus, M.W. Kronenberg.
Left ventricular dysfunction induced by cold exposure in patients with systemic sclerosis.
Am J Med, 80 (1986), pp. 385-392
[41.]
H.T. Colfer, S.K. Das, L. Dabich, O.S. Randall, B. Pitt.
Effect of cold stress on coronary sinus blood flow in patients with scleroderma.
J Assoc Acad Minor Phys, 4 (1993), pp. 62-65
[42.]
A. Kahan, J.Y. Devaux, B. Amor, C.J. Menkes, S. Weber, A. Venot, et al.
The effect of captopril on thallium 201 myocardial perfusion in systemic sclerosis.
Clin Pharmacol Ther, 47 (1990), pp. 483-489
[43.]
A. Kahan, A. Nitenberg, J. Foult, B. Amor, C.J. Menkes, J.Y. Devaux, et al.
Decreased coronary reserve in primary scleroderma myocardial disease.
Arthritis Rheum, 28 (1985), pp. 637-646
[44.]
R. Montisci, A. Vacca, P. Garau, P. Colonna, M. Ruscazio, G. Passiu, et al.
Detection of early impairment of coronary flow reserve in patients with systemic sclerosis.
Ann Rheum Dis, 62 (2003), pp. 890-893
[45.]
A. Vacca, P. Siotto, A. Cauli, R. Montisci, P. Garau, V. Ibba, et al.
Absence of epicardial coronary stenosis in patients with systemic sclerosis with severe impairment of coronary flow reserve.
Ann Rheum Dis, 65 (2006), pp. 274-275
[46.]
S. Morelli, A. Sgreccia, P. De Marzio, C. Perrone, L. Ferrante, A.M. Gurgo, et al.
Noninvasive assessment of myocardial involvement in patients with systemic sclerosis: role of signal averaged electrocardiography.
J Rheumatol, 24 (1997), pp. 2358-2363
[47.]
M. Paradiso, M. Di Franco, A. Musca, S. Basili, V. Riccieri, V. Paoletti, et al.
Ventricular late potentials in systemic sclerosis: relationship with skin involvement.
J Rheumatol, 29 (2002), pp. 1388-1392
[48.]
V. Steen.
The heart in systemic sclerosis.
Curr Rheumatol Rep, 6 (2004), pp. 137-140
[49.]
A.C. Rankin, S. Osswald, B.A. McGovern, J.N. Ruskin, H. Garan.
Mechanism of sustained monomorphic ventricular tachycardia in systemic sclerosis.
Am J Cardiol, 83 (1999), pp. 633-636
[50.]
D. Lacroix, F. Brigadeau, C. Marquie, D. Klug.
Electroanatomic mapping and ablation of ventricular tachycardia associated with systemic sclerosis.
Europace, 6 (2004), pp. 336-342
[51.]
R.L. Ridolfi, B.H. Bulkley, G.M. Hutchins.
The cardiac conduction system in progressive systemic sclerosis: clinical and pathologic features of 35 patients.
Am J Med, 61 (1976), pp. 361-366
[52.]
N.K. Roberts, W.R. Cabeen.
Atrioventricular nodal function in progressive systemic sclerosis.
Semin Arthritis Rheum, 13 (1983), pp. 174-181
[53.]
M.A. Sackner, E.R. Heinz, A.J. Steinberg.
The heart in scleroderma.
Am J Cardiol, 17 (1966), pp. 542-559
[54.]
E. Kinney, W. Reeves, R. Zellis.
The echocardiogram in slceroderma endocarditis of the mitral valve.
Arch Intern Med, 139 (1979), pp. 1179-1180
[55.]
S.M. Comens, M.A. Alpert, G.C. Sharp, T.A. Pressly, D.L. Kelly, S.E. Hazelwood, et al.
Frequency of mitral valve prolapse in systemic lupus erythematosus, progressive systemic sclerosis, and mixed connective tissue disease.
Am J Cardiol, 63 (1989), pp. 369-370
[56.]
M. Penmetcha, S.W. Rosenbush, C.A. Harris.
Cardiac valvular disease and systemic lupus erythematosus/scleroderma overlap associated with antiphospholipid antibodies.
J Rheumatol, 23 (1996), pp. 2171-2174
[57.]
M.H. Taylor, J.A. McFadden, M.B. Bolster, R.M. Silver.
Ulnar artery involvement in systemic sclerosis (scleroderma).
J Rheumatol, 29 (2002), pp. 102-106
[58.]
J. Constans, P. Gosse, J.L. Pellegrin, P. Ansorborlo, B. Leng, J. Clementy, et al.
Alteration of arterial distensibility in systemic sclerosis.
J Intern Med, 241 (1997), pp. 115-118
[59.]
M. Ho, D. Veale, C. Eastmond, G. Nuki, J. Belch.
Macrovascular disease and systemic sclerosis.
Ann Rheum Dis, 59 (2000), pp. 39-43
[60.]
K.S. Cheng, A. Tiwari, A. Boutin, C.P. Denton, C.M. Black, R. Morris, et al.
Carotid and femoral arterial wall mechanics in slceroderma.
Rheumatology, 42 (2003), pp. 1299-1305
[61.]
I. Moyssakis, E. Gialafos, V. Vassiliou, E. Taktikou, C. Katsiari, D.P. Papadopoulos, et al.
Aortic stiffness in systemic sclerosis is increased independently of the extent of skin involvement.
Rheumatology, 44 (2005), pp. 251-254
[62.]
J.P.A. Ioannidis, P.G. Vlachoyiannopoulos, A.-B. Haidich, T.A. Medsger Jr, M. Lucas, C.J. Michet, et al.
Mortality in systemic sclerosis: an international meta-analysis of individual patient data.
Copyright © 2006. Elsevier España S.L. Barcelona
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