Clinical research study
Libman-Sacks Endocarditis in Systemic Lupus Erythematosus: Prevalence, Associations, and Evolution

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Abstract

Purpose

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.

Methods

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.

Results

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.

Conclusions

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.

Section snippets

Patients and Methods

In this study, 342 consecutive patients with systemic lupus erythematosus (297 females and 45 males) who had a regular follow-up at the Department of Pathophysiology (the largest referral center for autoimmune diseases in Greece, including approximately 650 patients with systemic lupus erythematosus), were prospectively evaluated by Doppler echocardiography. Complete echocardiographic examination was performed by 2 cardiologists who were blinded to the clinical and laboratory findings of the

Echocardiography

The patients underwent an initial and a follow-up echocardiographic examination with pulsed, continuous, and color Doppler using a Hewlett Packard Sonos 1000 ultrasound system with a 2.5-MHz transducer (Hewlett Packard, Palo Alto, Calif). Libman-Sacks vegetations appeared as valve masses of varying size (≥2 mm in diameter) and shape with irregular borders and echodensity, firmly attached to the valve surface and exhibiting no independent motion. They involved either the valve or the subvalvular

Laboratory Examinations

Antinuclear antibodies were detected by indirect immunofluorescence. Anti-DNA antibodies were assayed by enzyme-linked immunosorbent assay, antibodies to extractable nuclear antigens were measured by counter immunoelectrophoresis, and complement concentrations were measured by immunodiffusion.13 Anticardiolipin antibodies (immunoglobulin-G and M) were measured by enzyme-linked immunosorbent assay using cardiolipin (cardiolipin 1649; Sigma, St Louis, Mo; 5 mg/mL in ethanol) as antigen on

Statistical Analysis

Statistical analysis was performed using programs available in the Statistical Package for the Social Sciences (SPSS Inc, Chicago, Ill). The Student t test was used to assess differences in continuous variables between the studied groups, and the chi-square test was used for categoric variables. Correlation coefficients were estimated, and univariate and multivariate logistic or linear regression analyses were performed to evaluate whether clinical and laboratory characteristics of patients

Valvular Abnormalities at Baseline

Thirty-eight (11%) of 342 patients with systemic lupus erythematosus had Libman-Sacks vegetations (Figure 1, Figure 2). Libman-Sacks vegetations were associated with valvular thickening in 14 (38%) of 38 cases. Twenty-four (63%) of 38 patients with Libman-Sacks vegetations had mitral valve involvement, resulting in mitral regurgitation in all of them (mild in 18, moderate in 4, and severe in 2 cases). Mitral stenosis in association with regurgitation was found in 9 patients (mild in 6 and

Clinical and Laboratory Associations

The comparisons of demographic, clinical, and laboratory characteristics between the patients with and without Libman-Sacks vegetations are presented in Table 1. Patients with systemic lupus erythematosus and Libman-Sacks vegetations had longer disease duration and higher disease activity characterized by higher frequency of pericarditis, lupus nephritis, and hemolytic anemia. A significant correlation also was found between Libman-Sacks endocarditis and arterial or venous thromboses,

Follow-Up Echocardiography

During a mean follow-up period of 46.24 ± 17.13 months, 252 of 342 patients with systemic lupus erythematosus were reevaluated. The valvular lesions in the 38 patients with Libman-Sacks endocarditis had the following changes: 5 patients with mild mitral regurgitation at the beginning showed deterioration, 4 patients developed moderate regurgitation, and 1 patient developed severe regurgitation (Table 2). In the majority of the patients, mitral stenosis remained unchanged and the mean pressure

Discussion

There are 5 major findings in this study:

  • Libman-Sacks vegetations can be found in approximately 1 of 10 patients with systemic lupus erythematosus;

  • The most frequently involved valve is the mitral valve followed by the aortic valve, whereas regurgitation represents the predominant functional lesion;

  • Libman-Sacks lesions are associated with lupus duration, disease activity, anticardiolipin antibodies, and antiphospholipid syndrome manifestations;

  • Progression of valve lesions, especially of aortic

Conclusion

Our results showed that Libman-Sacks vegetations are not uncommon in patients with systemic lupus erythematosus, particularly in those with longer disease duration, higher lupus activity, positive anticardiolipin antibodies, and secondary antiphospholipid syndrome manifestations. The predominant lesion is mitral regurgitation while a progression of valvular lesions occurs during a long-term follow-up period. Thrombotic events, mainly stroke and transient ischemic attacks, tend to develop in

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    1

    V. Vassilliou and M. G. Tektonidou contributed equally to the project.

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