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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

  • Chest x-ray: may show cardiomegaly from valve disease or pericardial effusion

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