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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 89–1). 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 a condition known as 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 89–1. Common Clinical and Cardiovascular Manifestations of Systemic Autoimmune Diseases

This chapter aims to familiarize the cardiovascular specialist with the clinical features of rheumatologic diseases that affect the heart and blood vessels. These diseases derive from chronic inflammation and abnormal tissue repair. This chapter also covers the most common heritable diseases of connective tissue (eg, Marfan syndrome, Ehlers-Danlos pseudoxanthoma elasticum). Unlike autoimmune diseases, these rare disorders mostly result from mutations in specific genes encoding various components of connective tissue that maintain the structural integrity of the vasculature.

Rheumatoid Arthritis

With a worldwide prevalence of 0.5% to 1%,1 RA ranks as the most common of the systemic autoimmune diseases. RA is a ...

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