Special article
Cardiovascular manifestations of systemic lupus erythematosus: Current perspective

https://doi.org/10.1016/0033-0620(85)90003-9Get rights and content

Abstract

Cardiovascular manifestations develop in the majority of SLE patients at some time during the course of their illness, the most common being acute fibrinous pericarditis and pericardial effusion. Echocardiography has demonstrated an increased incidence of pericardial effusion, even in those who have minimal symptoms. Chronic adhesive pericarditis, pericardial tamponade, and constrictive pericarditis occur rarely. While myocarditis is commonly noted at autopsy, it is often silent clinically. Diagnosis during life can be confirmed only by endomyocardial biopsy. Electrocardiographic changes are often nonspecific. Endocarditis with superimposed nonbacterial verrucous vegetations (Libman-Sacks) is noted in more than 40% of hearts at autopsy, but is rarely diagnosed during life. Valve dysfunctions, such as aortic stenosis, aortic insufficiency, mitral stenosis, and mitral insufficiency, occasionally manifest during life and rarely may necessitate surgery.

Atrial and ventricular arrhythmias, first degree AV block, and acquired CHB occur in association with pericarditis, myocarditis, vasculitis, and myocardial fibrosis, respectively. CCHB developing in newborns of mothers with SLE, particularly those who have an antibody to soluble tissue ribonuclear protein RO(SS-A), is increasingly being appreciated by both pediatric cardiologists and rheumatologists.

Recently, severe coronary atherosclerosis resulting in angina pectoris and/or myocardial infarction in young adults has been noted, particularly in those who had developed risk factors such as hypertension and hyperlipidemia while receiving prolonged corticosteroid therapy. Rarely, coronary arteritis may produce similar symptoms. Congestive heart failure of either single or multiple etiologies carries an ominous prognosis. It remains a cause of high morbidity and mortality unless recognized early and treated properly. Extracardiac vascular manifestations of SLE include telangiectasia, vasculitis, livedo reticularis, Raynaud's phenomena, and thrombophlebitis, all of which may occur either alone or in different combinations.

Evidence is now slowly accumulating that substantiates that immune complex deposition, complement activation and subsequent inflammatory reaction is responsible for the majority of the cardiovascular manifestations of SLE, for example, pericarditis, myocarditis, endocarditis, coronary arteritis, coronary atherosclerosis, and systemic and pulmonary vasculitis. For the pathogenesis of CCHB, a maternal antibody to the soluble tissue ribonuclear protein RO(SS-A), which crosses the placenta during the early stage of gestation and either interferes with the development or afflicts a degenerating process in the developing conduction system, is being incriminated.

Corticosteroid therapy remains the cornerstone for the management of pericarditis, myocarditis, vasculitis, and coronary arteritis. However, nonsteroidal inflammatory agents, antiarrhythmic drugs, antihypertensive agents, vasodilator drugs, intensive endocarditis prophylaxis, and rarely, intracardiac pacing when indicated also play an important role in the overall management of cardiovascular manifestations of SLE.

Renal failure, lupus cerebritis, and infections are still the three most common causes of death in patients with SLE. However, recently increased cardiac mortality as a result of hypertension, congestive heart failure, coronary atherosclerosis, and thrombosis, the latter particularly in young women, has been recognized. Mortality of infants with CCHB born to mothers with SLE remains significant despite intracardiac pacing.

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