Prevalence, Morphologic Types, and Evolution of Cardiac Valvular Disease in Systemic Lupus Erythematosus

• Published in: The New England journal of medicine Vol. 319; no. 13; pp. 817 - 823
• Main Authors: Galve, Enrique, Candell-Riera, J, Pigrau, Carlos, Permanyer-Miralda, G, Garcia-Del-Castillo, H, Soler-Soler, J
• Format: Journal Article
• Language: English
• Published: Boston, MA Massachusetts Medical Society 29.09.1988
• Subjects: Adolescent; Adult; Age; Age Factors; Aged; Biological and medical sciences; Echocardiography; Electrocardiography; Endocarditis; Endocarditis - etiology; Female; Follow-Up Studies; Heart Valve Diseases - classification; Heart Valve Diseases - epidemiology; Heart Valve Diseases - etiology; Humans; Internal medicine; Lupus; Lupus Erythematosus, Systemic - complications; Lupus Erythematosus, Systemic - drug therapy; Male; Medical sciences; Methylprednisolone - therapeutic use; Middle Aged; Patients; Prospective Studies; Rheumatic heart disease; Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis; Systemic lupus erythematosus; Time Factors; Ultrasonic imaging; Human; Connective tissue disease; Prognosis; Cardiac valvular disease; Cardiovascular disease; Frequency; Lupus erythematosus; Disseminated
• ISSN: 0028-4793; 1533-4406
• DOI: 10.1056/NEJM198809293191302

We performed echocardiography prospectively, 4.9±0.7 years apart (mean ±SD), in 74 patients with systemic lupus erythematosus. On the basis of the first study, the patients were distributed in four groups according to the type of valvular involvement: 7 patients had vegetations (Libman—Sacks endocarditis; group 1); 6 patients had rigid and thickened valves with stenosis, regurgitation, or both (group 2); 5 patients had miscellaneous forms of valvular involvement without valvular dysfunction (group 3), as did the 60 controls; and 56 patients had no valvular disease (group 4). The overall prevalence of clinically important valvular disease (groups 1 and 2) was 18 percent. Patients in group 1 were younger than those in group 2 (33.5± 16.7 vs. 47.8±17.6 years; P<0.05), had a shorter mean duration of lupus (4.8±2.2 vs. 10.7±6.4 years; P<0.001), and had received a smaller cumulative dose of steroids (21.5±13.1 vs. 79.5±63.4 g of methylprednisolone or its equivalent; P<0.05). During the five-year follow-up, one patient in group 1 and five in group 2 required valve surgery, no patient in group 3 had valvular dysfunction, and five patients in group 4 had mild valvular lesions. We conclude that clinically important valvular involvement in systemic lupus is relatively frequent and sometimes requires surgery. Echocardiography can identify a subset of lesions (valvular thickening and dysfunction), other than verrucous (Libman–Sacks) endocarditis, that are prone to hemodynamic deterioration. (N Engl J Med 1988;319:817–23.) SYSTEMIC lupus erythematosus is a vascular disorder of connective tissue, mediated by autoantibodies directed against cellular antigens, that results in multisystemic inflammatory damage. Cardiac lesions involving the endocardium, myocardium, pericardium, and coronary vessels have been commonly found in autopsy studies. 1 2 3 The most characteristic finding is endocardial involvement, as described by Libman and Sacks, 4 which is present in 13 to 50 percent of autopsy studies. 5 6 7 However, the prevalence in living patients has not been established, since cardiac involvement is usually clinically silent and few studies have used laboratory techniques for its detection. 8 9 10 11 Valvular damage in systemic lupus is usually considered to . . .