Review
Myocarditis in systemic lupus erythematosus

https://doi.org/10.1016/S0002-9343(02)01223-8Get rights and content

Abstract

Although clinical manifestations of myocarditis in systemic lupus erythematosus are uncommon, noninvasive cardiac testing may detect subclinical cases. The pathogenesis of myocarditis in systemic lupus erythematosus has been ascribed to many factors, including autoimmunity, medications, and coexisting diseases. Lupus myocarditis merits urgent clinical attention because of the likely progression to arrhythmias, conduction disturbances and heart block, dilated cardiomyopathy, and heart failure. Endomyocardial biopsy can be used to identify the underlying inflammatory histopathology. Usual therapy includes high-dose corticosteroids, in addition to standard cardiac medications.

Section snippets

Prevalence

In clinical studies, myocarditis has been identified, on average, in about 9% (123 of 1326) of patients with systemic lupus erythematosus 4, 5, 6, 7, 8, 9, 10, 11, 12. Postmortem studies from the 1950s and 1960s found an average prevalence of myocarditis in 57% (72 of 126) of patients, indicating that subclinical myocardial involvement occurs commonly in patients who die of systemic lupus erythematosus 6, 13, 14, 15, 16, 17. Following the introduction of corticosteroid therapy into the

Clinical features

Most myocarditis in systemic lupus erythematosus is asymptomatic but may manifest with fever, dyspnea, palpitations, and nonexertional chest pain (Table). Additional signs include jugular venous distension, resting tachycardia that is disproportionate to the patient’s temperature, gallop rhythms, new cardiac murmurs, cardiomegaly, and peripheral edema. Nonspecific ST-T wave changes, conduction abnormalities, frequent premature complexes, and supraventricular and ventricular tachycardia may be

Pathology

The frequency of myocardial pathology that is directly related to systemic lupus erythematosus or another autoimmune process is not known 1, 39. Other factors, such as accelerated atherosclerotic coronary artery disease, coronary vasculitis, valvulopathy, pulmonary hypertension, renal insufficiency, anemia, and calciphylaxis, as well as adverse effects of medications such as steroids and chloroquine, might cause or contribute to the underlying myocardial pathology 2, 39, 40, 41, 42.

Diagnosis

The diagnosis of myocarditis depends largely on clinical suspicion rather than definitive diagnostic tests (53). Unexplained fever, dyspnea, palpitations, tachycardia not due to fever, ventricular gallop rhythm, cardiomegaly, conduction disturbances, new murmurs, an abnormal ECG, or cardiac failure in the setting of systemic lupus erythematosus should suggest the possibility of myocarditis 6, 7, 9, 10, 31. A chest radiograph may disclose cardiomegaly; in this situation, exclusion of pericardial

Echocardiographic findings in lupus myocarditis

Echocardiography is useful for noninvasive localization of inflammation and assessment of its extent in patients with presumed myocarditis (53). Echocardiography does not establish the diagnosis of myocarditis, but it may provide evidence of global, regional, or segmental wall motion abnormalities, indicating myocardial inflammation 21, 22, 23, 26. In addition, ultrasonic tissue characterization may be useful in evaluating myocardial involvement (58). Other echocardiographic findings, although

Other noninvasive testing in lupus myocarditis

Perfusion defects occur in about half (56 of 103) of lupus patients with no known cardiac symptoms 30, 74, 75, 76. These include both fixed and reversible defects. If coronary angiography has excluded epicardial coronary artery disease, nuclear perfusion abnormalities suggest coronary spasm, myocardial fibrosis, or myocardial damage due to inflammation of small vessels and the neighboring myocardium. Single photon emission tomography may detect about twice as many perfusion abnormalities (in

Treatment

Acute lupus myocarditis requires urgent clinical attention. Current treatment strategies are based on clinical experience, rather than randomized trials. Myocarditis is usually treated with bed rest and corticosteroids 9, 31, 40. High-dose intravenous therapy (e.g., methylprednisone pulses of 1000 mg/d) is generally used, followed by high doses of an oral preparation (e.g., prednisone, 1 mg/kg/d) for 1 to 2 weeks. Clinical findings, including gallop rhythms, cardiomegaly, peripheral edema, and

Conclusion

Although myocarditis is not a common manifestation of systemic lupus erythematosus, the functional and prognostic implications of the diagnosis are important. Noninvasive diagnostic tests have improved the ability to detect these problems. Enhanced clinical scrutiny should improve diagnostic accuracy and facilitate implementation of effective treatments.

References (85)

  • R.W. Chang

    Cardiac manifestations of SLE

    Clin Rheum Dis

    (1982)
  • K.G. Moder et al.

    Cardiac involvement in systemic lupus erythematosus

    Mayo Clin Proc

    (1999)
  • B.F. Mandell

    Cardiovascular involvement in systemic lupus erythematosus

    Semin Arthritis Rheum

    (1987)
  • W.H. Leung et al.

    Association between antiphospholipid antibodies and cardiac abnormalities in patients with systemic lupus erythematosus

    Am J Med

    (1990)
  • A. del Rio et al.

    Myocardial involvement in systemic lupus erythematosus. A noninvasive study of left ventricular function

    Chest

    (1978)
  • I.G. Crozier et al.

    Cardiac involvement in systemic lupus erythematosus detected by echocardiography

    Am J Cardiol

    (1990)
  • W.H. Leung et al.

    Doppler echocardiographic evaluation of left ventricular diastolic function in patients with systemic lupus erythematosus

    Am Heart J

    (1990)
  • Z. Sasson et al.

    Impairment of left ventricular diastolic function in systemic lupus erythematosus

    Am J Cardiol

    (1992)
  • T.M. Winslow et al.

    The left ventricle in systemic lupus erythematosusinitial observations and a five-year follow-up in a university medical center population

    Am Heart J

    (1993)
  • B.E. Strauer et al.

    Lupus cardiomyopathycardiac mechanics, hemodynamics, and coronary blood flow in uncomplicated systemic lupus erythematosus

    Am Heart J

    (1976)
  • J.D. Hosenpud et al.

    Myocardial perfusion abnormalities in asymptomatic patients with systemic lupus erythematosus

    Am J Med

    (1984)
  • U. Kuhl et al.

    Antimyosin scintigraphy and immunohistologic analysis of endomyocardial biopsy in patients with clinically suspected myocarditis—evidence of myocardial cell damage and inflammation in the absence of histologic signs of myocarditis

    J Am Coll Cardiol

    (1998)
  • K. Murai et al.

    Alterations in myocardial systolic and diastolic function in patients with active systemic lupus erythematosus

    Am Heart J

    (1987)
  • F.P. Quismorio

    Cardiac abnormalities in systemic lupus erythematosus

  • M. Petri

    Systemic lupus erythematosus and the cardiovascular systemthe heart

  • E.M. Tan et al.

    The 1982 revised criteria for the classification of systemic lupus erythematosus

    Arthritis Rheum

    (1982)
  • W. Brigden et al.

    The heart in systemic lupus erythematosus

    Br Heart J

    (1960)
  • E.L. Dubois et al.

    Clinical manifestations of systemic lupus erythematosus. Computer analysis of 520 cases

    JAMA

    (1964)
  • D. Estes et al.

    The natural history of systemic lupus erythematosus by prospective analysis

    Medicine (Baltimore)

    (1971)
  • M.W. Ropes

    Systemic Lupus Erythematosus

    (1976)
  • D.G. Borenstein et al.

    The myocarditis of systemic lupus erythematosusassociation with myositis

    Ann Intern Med

    (1978)
  • E. Badui et al.

    Cardiovascular manifestations in systemic lupus erythematosus. Prospective study of 100 patients

    Angiology

    (1985)
  • H. Jonsson et al.

    Outcome in systemic lupus erythematosusa prospective study of patients from a defined population

    Medicine (Baltimore)

    (1989)
  • N.F. Rothfield

    Cardiac aspects

  • G.C. Griffith et al.

    Acute and subacute disseminated lupus erythematosusa correlation of clinical and postmortem findings in eighteen cases

    Circulation

    (1951)
  • A.M. Harvey et al.

    Systemic lupus erythematosusreview of the literature and clinical analysis of 138 cases

    Medicine

    (1954)
  • T.Q. Kong et al.

    Clinical diagnosis of cardiac involvement in systemic lupus erythematosusa correlation of clinical and autopsy findings in thirty patients

    Circulation

    (1962)
  • T.N. James et al.

    Pathology of the cardiac conduction system in systemic lupus erythematosus

    Ann Intern Med

    (1965)
  • P. Nihoyannopoulos et al.

    Cardiac abnormalities in systemic lupus erythematosus. Association with raised anticardiolipin antibodies

    Circulation

    (1990)
  • B. Cujec et al.

    Cardiac abnormalities in patients with systemic lupus erythematosus

    Can J Cardiol

    (1991)
  • R. Cervera et al.

    Cardiac disease in systemic lupus erythematosusprospective study of 70 patients

    Ann Rheum Dis

    (1992)
  • G. Sturfelt et al.

    Cardiovascular disease in systemic lupus erythematosus. A study of 75 patients from a defined population

    Medicine (Baltimore)

    (1992)
  • Cited by (149)

    • Myocarditis in the forensic setting

      2023, Cardiovascular Pathology
    • Autoimmune heart disease

      2022, Translational Autoimmunity: Autoimmune Diseases in Different Organs
    View all citing articles on Scopus
    View full text