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This chapter discusses the cardiovascular manifestations of various rheumatological conditions, including autoimmune disorders (see accompanying Hurst’s Central Illustration) and inherited connective tissue diseases. The effects of the autoimmune disorders on the cardiovascular system may result from local or systemic mechanisms. Pericarditis, valvular disease, coronary artery disease and accelerated atherosclerosis, cardiomyopathy, and heart failure are common cardiovascular manifestations of the autoimmune rheumatological disorders, which include rheumatoid arthritis, adult-onset Still disease, systemic lupus erythematosus, antiphospholipid syndrome, dermatomyositis and polymyositis, systemic sclerosis, seronegative spondyloarthropathies, and systemic vasculitides. Notably, 40% of deaths in rheumatoid arthritis are attributable to cardiovascular disease and pericarditis occurs in up to a quarter of patients with adult-onset Still disease. However, cardiovascular involvement is particularly common in patients with systemic lupus erythematosus, with up to 70% of patients showing signs of cardiovascular disease. The inherited connective tissue diseases (including Marfan syndrome, Loeys-Dietz syndrome, Ehlers-Danos syndrome, familial thoracic aortic aneurysms and dissections, pseudoxanthoma elasticum, and osteogenesis imperfecta) are generally associated with vascular aneurysms, particularly of the aorta, and aortic dissections; some of these conditions are also associated with valvular disease.

eFig 100-01

Hurst's Central Illustration: Cardiovascular manifestations of Various Autoimmune Rheumatological Conditions.


Rheumatologic conditions such as systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), and the vasculitides that affect multiple organ systems may also impact the cardiovascular system. These pathologic processes fall in the category of autoimmune diseases, which are initiated by a complex interplay between genetic factors and environmental stimuli. They are presumed to be driven by self-reactive T and B lymphocytes, which in tandem with a network of endogenous and exogenous signals, activate the immune system, producing tissue inflammation and damage (Table 100–1). The effects of autoimmunity on the cardiovascular system may be the result of local or systemic mechanisms. For example, locally aberrant immunity may selectively target the pericardium, myocardium, or conduction system in systemic sclerosis. On the other hand, in patients with SLE or systemic vasculitis, circulating immune complexes may deposit in blood vessels, where they evoke an inflammatory response, which in turn occludes the vessel lumen and causes ischemic manifestations distal to the site of critically limited blood flow. A hypercoagulable state from antiphospholipid antibody syndrome can lead to thrombotic occlusion, producing myocardial infarction, stroke, or ischemic damage of the visceral organs. Thus, diverse pathways of a dysregulated immune system may converge to directly or indirectly damage the heart and vasculature.

TABLE 100–1.Common Clinical and Cardiovascular Manifestations of Systemic Autoimmune Diseases

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