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 endocardium including the valve leaflets, myocardium, coronary arteries, 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, echocardiography studies, and postmortem series. Furthermore, their pathogenesis and natural history are still incompletely understood and their therapy is not yet standardized. Increased awareness and better understanding of the cardiovascular diseases associated with connective tissue diseases may lead to earlier recognition and treatment with consequent decreased morbidity and mortality. Finally, and of importance, patients with connective tissue diseases and associated heart disease should be consulted and managed by a heart team (including a cardiologist with imaging expertise, an interventional cardiologist, and in selected cases a cardiothoracic surgeon) and rheumatologists given the potential morbidity and mortality of their heart disease and the complexity and potential benefits, but also potential harm of their pharmacotherapy and cardiac interventions.
SYSTEMIC LUPUS ERYTHEMATOSUS
ESSENTIALS OF DIAGNOSIS
Musculoskeletal and mucocutaneous manifestations of systemic lupus erythematosus (SLE).
Libman-Sacks vegetations, atrioventricular (AV) valve regurgitation, intra- and extra-cardiac thrombosis, premature atherosclerosis, pericarditis, myocarditis, and SLE.
Cardioembolism and SLE.
Acute pericarditis with antinuclear antibodies detected in the pericardial fluid.
Systemic lupus erythematosus (SLE) 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. SLE 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.
SLE affects the cardiovascular system and is associated with two- to threefold higher incidence of major cardiovascular events than matched controls without SLE. Cardiovascular disease is the third most important cause of death in SLE patients (after infectious and renal diseases) during the earlier course of disease, but later, cardiovascular disease is a predominant cause of their death. The most significant SLE-associated heart diseases are valvular heart disease, arterial or venous thrombosis and systemic thromboembolism, premature coronary artery disease (CAD), and pericarditis. Myocarditis or cardiomyopathy and cardiac arrhythmias or conduction disturbances are less common. Independent predictors of their cardiovascular disease include duration, age of onset, and activity and severity of SLE, presence of antiphospholipid antibodies, and corticosteroid therapy.