We evaluated the prevalence and progression of Libman-Sacks endocarditis in patients with systemic lupus erythematosus and any association between this valvulopathy and their clinical and laboratory characteristics.

Doppler echocardiography was performed in 342 consecutive patients with systemic lupus erythematosus (297 females and 45 males). The clinical and laboratory data were recorded. Patients were reevaluated after a follow-up period of 4 years.

Libman-Sacks endocarditis was found in 38 patients (11%). In 24 of 38 patients, mitral valve involvement was found, resulting in regurgitation in all (mild in 18, moderate in 4, and severe in 2), whereas stenosis co-occurred with regurgitation in 9 patients (mild in 6 and moderate in 3). Thirteen (34%) of 38 patients had aortic valve involvement; 11 had regurgitation (mild) and 8 had stenosis (mild), coexistent with regurgitation in 6 of them. One patient had mild tricuspid regurgitation. A significant association was found between Libman-Sacks endocarditis and disease duration and activity, thromboses, stroke, thrombocytopenia, anticardiolipin antibodies, and antiphospholipid syndrome. During the follow-up period, 252 of 342 patients were reevaluated echocardiographically. Among the 38 patients with Libman-Sacks vegetations, 5 with mild mitral regurgitation at the beginning developed moderate (n=4) and severe mitral regurgitation (n=1), 2 patients with mitral stenosis (mild in 1 and moderate in 1) developed severe mitral regurgitation, and 2 patients with mild aortic regurgitation developed moderate and severe mitral regurgitation, whereas a significant deterioration of aortic stenosis was found. Two patients who were candidates for surgery died. Among the 213 patients without vegetations at the beginning, 8 developed new Libman-Sacks lesions.

Libman-Sacks vegetations can be found in approximately 1 of 10 patients with systemic lupus erythematosus, and they are associated with lupus duration, disease activity, anticardiolipin antibodies, and antiphospholipid syndrome manifestations. A progression of valve lesions may occur during long-term follow-up.