Cardiac Manifestations of Systemic Lupus Erythematous: An Overview of the Incidence, Risk Factors, Diagnostic Criteria, Pathophysiology and Treatment Options

Abstract

Systemic lupus erythematosus (SLE) is a complex connective tissue disease that can potentially affect every organ of the human body. In some cases, SLE may present with diverse cardiac manifestations including pericarditis, myocarditis, valvular disease, atherosclerosis, thrombosis, and arrhythmias. Heart disease in SLE is associated with increased morbidity and mortality. It is unclear whether traditional treatments for coronary artery disease significantly impact mortality in this population. Current therapeutic agents for SLE include glucocorticoids, hydroxychloroquine, mycophenolate mofetil, azathioprine, methotrexate, cyclophosphamide, and B cell-directed therapies. This article will provide a comprehensive review and update on this important disease state.

Coronary Artery Disease in Systemic Lupus Erythematosus: What Do the Facts Say?

“…Endothelial cells exposed to oxLDL form monocyte chemotactic protein-1 (MCP-1) and other chemokines that stimulate the differentiation of macrophages to foam cells. Foam cells in smooth muscle form fibrosis and atherosclerotic plaques [ 5 ]. There is a decrease in the enzyme nitric oxide synthase (NOS) due to endothelial dysfunction, causing a reduction in the production of nitric oxide [ 2 ].…”

“…Th-1 cells produce interferon-gamma (IFN-γ) and inhibit the growth of endothelial cells and smooth muscle in excess, leading to instability of the atherosclerotic plaque. There is also excessive inflammation due to IL-17 produced by Th-17 cells [ 2 , 5 ]. In SLE, CD4+ cells migrate to the arterial wall with proatherogenic activity, and their increase is related to the development of coronary and carotid atherosclerosis [ 5 ].…”

“…There is also excessive inflammation due to IL-17 produced by Th-17 cells [ 2 , 5 ]. In SLE, CD4+ cells migrate to the arterial wall with proatherogenic activity, and their increase is related to the development of coronary and carotid atherosclerosis [ 5 ].…”

“…In asymptomatic coronary disease, invariant natural killer T (iNKT) cells have been shown to produce an atheroprotective effect on the plaque by differentiating macrophages toward an anti-inflammatory phenotype. On the contrary, in symptomatic coronary disease, iNKT cells do not have the atheroprotective effect, and proinflammatory monocytes increase [ 5 ].…”

“…It has been seen that premenopausal women with SLE have up to a 50-fold higher risk of AMI when compared to healthy women of the same age, with the first year of diagnosis being the most frequent presentation [ 1 ]. Most acute coronary syndrome (ACS) cases in patients with SLE are due to atherosclerosis, which in post-mortem studies has been found in 50% of patients [ 5 ]. Less common causes include thrombosis, vasculitis, and spontaneous coronary artery dissection.…”