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 |Favorite Table|Download (.pdf) TABLE 100–1. Common Clinical and Cardiovascular Manifestations of Systemic Autoimmune Diseases
|Disease ||Sex Distribution ||Clinical Manifestations ||Cardiovascular Manifestations |
|Rheumatoid arthritis ||F > M ||Inflammatory polyarthritis, rheumatoid nodules, RF, anti-CCP ||Pericarditis, coronary artery disease, cardiomyopathy, congestive heart failure |
|Systemic lupus erythematosus ||F > M ||Malar rash, arthritis, photosensitivity, serositis, nephritis, +ANA; may have concomitant APS ||Pericarditis, Libman-Sacks endocarditis, coronary artery disease, hypertension |
|Inflammatory myopathies ||F > M ||Proximal muscle weakness, DM with Gottron papules, shawl sign, mechanics hands ||Pericarditis, conduction system abnormalities, congestive heart failure, and myocarditis |
|Systemic sclerosis ||F > M ||Limited form is referred to as CREST. Diffuse form involves proximal skin and visceral organs ||Pulmonary hypertension, pericarditis, cardiomyopathy, conduction system disease |
|Seronegative spondyloarthropathy ||M > F ||Spinal or sacroiliac involvement, enthesitis, absence of rheumatoid factor, and a high incidence of HLA-B27 ||Aortitis, conduction system disease |
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, Loeys-Dietz, Ehlers-Danlos, and 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.
CARDIOVASCULAR MANIFESTATIONS OF SYSTEMIC RHEUMATIC DISEASES