ReviewMyocarditis in systemic lupus erythematosus
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.
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