Systemic Lupus Erythematosus and the Heart

KEY FEATURES

ESSENTIALS OF DIAGNOSIS

• Musculoskeletal and mucocutaneous manifestations of systemic lupus erythematosus

• Acute pericarditis/myocarditis with antinuclear antibodies detected in the pericardial fluid

• Libman-Sacks vegetations and atrioventricular valve regurgitation

• Intra- and extracardiac thrombosis and cardioembolism

• Premature atherosclerosis

GENERAL CONSIDERATIONS

• Systemic lupus erythematosus (SLE) is a multisystem chronic or recurrent inflammatory disease that mainly involves the musculoskeletal and mucocutaneous systems

• SLE is seen mainly in women (10:1) and is more common in those of African origin (3:1); it is rare, occurring in 4 to 250 per 100,000 people (varies with age, sex, and race of population)

• SLE-related cardiovascular disease is the third most common cause of death from SLE after infectious and renal disease

• Clinically important cardiovascular manifestations:

  • – Valvulopathy

  • – Pericarditis

  • – Premature coronary artery disease

  • – Vascular thrombosis and embolism

• Valvular disease is characterized by Libman-Sacks vegetations and leaflet thickening and regurgitation

  • – The incidence of valve disease increases with age, duration of SLE, moderate to high levels of antiphospholipid antibodies and treatment with corticosteroids

  • – Aortic and mitral valve disease predominate

CLINICAL PRESENTATION

SYMPTOMS AND SIGNS

• Valvular disease and myocarditis are usually mild and often asymptomatic; severe disease can lead to dyspnea and fatigue

• Pericarditis often presents with SLE flare-ups and is manifested by typical chest pain and dyspnea

• Tamponade is uncommon, and constriction is rare

• Vascular thrombosis can present as deep venous thrombosis with pulmonary emboli

• Acute arterial thrombosis can present as acute myocardial infarction or transient ischemic attack/stroke

PHYSICAL EXAM FINDINGS

• Murmurs of mitral or aortic regurgitation are found in a minority of those with valve disease

• Fever, tachycardia, tachypnea, and a pericardial friction rub are often seen in those with acute pericarditis

• Signs of tamponade can occur:

  • – Elevated jugular pressure

  • – Pulsus paradoxus

• Those with myocarditis may have cardiomegaly, gallop sounds, pulmonary rales, and edema

• Often findings vary according to which blood vessels are involved with thrombosis or emboli

DIFFERENTIAL DIAGNOSIS

• Other causes of acute pericarditis or myocarditis

• Other causes of valvular heart disease

• Other causes of vascular thrombosis

• Other connective tissue diseases

DIAGNOSTIC EVALUATION

LABORATORY TESTS

• Blood tests: low white blood cell count, elevated antiphospholipid antibodies, and a low C-reactive protein suggest an acute SLE flare-up

• Pericardial fluid is usually exudative and positive for antinuclear antibodies and exhibits low complement levels

ELECTROCARDIOGRAPHY

• Chamber enlargement is seen with chronic, more severe valve disease or hypertension associated with renal disease

• Typical ST changes of pericarditis can be seen

• Nonspecific ST-T–wave changes are seen with myocarditis

• ST-T–wave changes of ischemic heart disease can be seen

IMAGING STUDIES

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