Systemic Lupus Erythematosus

• Overview of systemic lupus erythematosus.
  by: Leonard Q.C. Siew - ls4181@bristol.ac.uk
  Department of Rheumatology, University of Bristol.
  
• Pathological specimen showing Libman-Sacks endocarditis of the mitral valve.
• Same image part of the University of Brimingham Clinical Immunology course.
• Patient handout on Systemic Lupus Erythematosus formatted for printing (from the NIH).
• Cardiac involvement written in Portuguese.
• Facial appearance: drawing from the year 1856.

Echocardiography and Systemic Lupus Erythematosus

Two dimensional echocardiography with doppler examination was performed in 54 patients with systemic lupus erythematosus (SLE). Nine (17%) had significant cardiac involvement (four left ventricular hypertrophy, one moderate pericardial effusion, one severe aortic regurgitation, and three ventricular systolic dysfunction). The authors further studied diastolic function in 45 patients who did not have a major abnormality in echo. SLE was graded as active in 16 patients (SLEDAI > 5) and inactive in 29 patients. Twenty age- and sex- matched subjects acted as controls. The data were compared using one way ANOVA test. Patients with active disease had significant diastolic dysfunction compared to inactive patients and controls as indicated by increased peak A (P < 0.01) and decreased E/A ratio (P < 0.01). There was no linear correlation between disease activity and diastolic dysfunction if SLEDAI was considered as a continuous variable (r=0.29 for E/A). Anticardiolipin antibodies (both IgG and IgM) were elevated in five patients (13 studied). One of them had severe mitral regurgitation, one had trace mitral and aortic regurgitation and one had diastolic dysfunction. The authors conclude that asymptomatic diastolic dysfunction is present in SLE patients.

Reference: Kalke S; Balakrishanan C; Mangat G; Mittal G; Kumar N; Joshi VR.
Lupus 1998;7(8):540-4

Prospective two dimensional and Doppler echocardiographic studies were performed in 41 patients to assess the incidence and spectrum of cardiac abnormalities. All patients included in the study fulfilled the 1982 revised criteria of the American Rheumatism Association for classification of SLE. There were 37 women and 4 men with average age of 38 years. Average duration of SLE was 6.5 years (range 6 months to 20 years). Nineteen patients (46.3%) with SLE had cardiac abnormalities. Valvular abnormalities were found in 14 patients (34.1%). Mitral valve abnormalities were the most common findings-in 7 patients (17.1%). There were 6 patients with aortic (14.6%), and 3 patients with tricuspid valve abnormalities (7.3%). One patient had morphological echocardiographic pattern suggesting noninfective verrucous vegetations affecting the tricuspid valve. Pericardial effusion was identified in 5 patients (12.2%). The authors found no correlation between the prevalence of cardiac abnormalities and duration, age and disease activity in SLE patients.

Reference: Straburzynska-Migaj E; Leszczynski P; Piszczek I; Cieslinski A; Mackiewicz S
Pol Merkuriusz Lek 1997 Aug;3(14):76-8

SLE affects most aspects of cardiac function, and recent studies have reported increasing cardiovascular morbidity and mortality. Pathologically, SLE is characterized by a pancarditis involving pericardium, myocardium, endocardium, and coronary arteries. In autopsy series, pericarditis has been found in 43% to 100% (mean 62%, Table I), and myocarditis was found in 8% to 78% (mean 40%, Table II), but both have been underdiagnosed clinically. Libman-Sacks lesions have been noted in 25% to 100% (mean 43%) and infective endocarditis in 1.1% to 4.9% of clinical and autopsy studies. Coronary disease may be due to arteritis, which should be treated with high-dose steroids, or it may be due to atherosclerosis, which is amenable to medical or surgical therapy. Valvular disease has been treated surgically, but with a combined surgical mortality as high as 25%. Aortic insufficiency and mitral regurgitation are the most common valvular problems, although aortic and mitral stenosis have also been reported. Hypertension has been noted in 14% to 69%, and heart failure in 5% to 44%. Evidence for a lupus cardiomyopathy, which may be subclinical, is reviewed. While steroids may ameliorate SLE pancarditis, they have also been associated with hypertension, LV hypertrophy, purulent and constrictive pericarditis, mitral regurgitation, and perhaps accelerated atherosclerosis. It remains to be seen if improved diagnosis and treatment of the cardiovascular manifestations of SLE can enhance survival.

Reference: Doherty NE; Siegel RJ
Am Heart J 1985 Dec;110(6):1257-65

Mitral valve vegetations in a patient with systemic lupus erythematosus.

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The contents and links on this page were last verified on August 13, 2001.