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The connective tissue diseases are immune-mediated inflammatory diseases, primarily of the musculoskeletal system; however, they frequently also involve the cardiovascular system. The most important of these diseases are systemic lupus erythematosus, rheumatoid arthritis, scleroderma, ankylosing spondylitis, polymyositis/dermatomyositis, and mixed connective tissue disease. They affect the valve leaflets, coronary arteries, pericardium, myocardium, conduction system, and great vessels with different rates of prevalence and degrees of severity. Although heart involvement in patients with connective tissue diseases contributes significantly to their morbidity and mortality rates, there is a large discrepancy between clinically recognized heart disease and postmortem series. Furthermore, the pathogenesis, natural history, and effects of therapy are incompletely understood. Increased awareness and better understanding of the cardiovascular disease associated with connective tissue diseases may lead to earlier recognition, treatment and, perhaps, increased longevity.

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Systemic Lupus Erythematosus

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Essentials of Diagnosis

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  • Musculoskeletal and mucocutaneous manifestations of systemic lupus erythematosus (SLE).
  • Libman-Sacks vegetations, atrioventricular (AV) valve regurgitation, myocarditis, vascular thrombotic disease, and SLE.
  • Cardioembolism and SLE.
  • Acute pericarditis with antinuclear antibodies detected in the pericardial fluid.

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General Considerations

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Systemic lupus erythematosus is a multisystem chronically recurrent inflammatory disease that affects the musculoskeletal, mucocutaneous, visceral, and central nervous systems. Symptoms include fatigue, myalgias, arthralgias or arthritis, photosensitivity, and serositis. The prevalence of SLE varies widely, from 4 to 250 cases per 100,000 persons. It is more frequent in a patient’s relatives than in the general population. Systemic lupus erythematosus is predominantly seen in females, with a female-to-male ratio of 10:1. The pathophysiology of the disease is related to the multiorgan deposition of circulating antigen-antibody complexes and activation of the complement system, leading to humoral- and cellular-mediated inflammation.

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Although SLE affects the cardiovascular system with varied frequency and degrees of severity, cardiovascular disease is the third most important cause of death in SLE patients (after infectious, renal, and central nervous system diseases). The most significant SLE-associated heart diseases are valvular heart disease, arterial or venous thrombosis and systemic thromboembolism, coronary artery disease (CAD), and pericarditis. Myocarditis or cardiomyopathy and cardiac arrhythmias or conduction disturbances are less common.

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The pathogenesis of SLE-associated cardiovascular disease is believed, as it is for the primary disease, that the immune complex deposition and complement activation lead to an acute, chronic, or recurrent inflammation of the valve leaflets, endocardium, vascular endothelium, pericardium, myocardium, or conduction system. The presence in these tissues of immune complexes, complement, antinuclear antibodies, lupus erythematosus cells, mononuclear inflammatory cells, necrosis, hematoxylin bodies, and deposits of fibrin and platelet thrombi support this theory. Many studies suggest that antiphospholipid antibodies (aPL) (IgA, IgG, or IgM anticardiolipin antibodies [aCL], lupus-anticoagulant [LA], or antibodies to plasma phospholipid-binding protein β2-glycoprotein I) cause cardiovascular injury. These antibodies, present in as many as half of SLE patients, are directed against negatively charged phospholipids present in the membrane of endothelial cells causing endothelial dysfunction, vascular injury, and ...

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