Establishing the Diagnosis of Libman–Sacks Endocarditis in Systemic Lupus Erythematosus

Section of General Internal Medicine, Tulane University School of Medicine, New Orleans, LA 70112, USA.
Journal of General Internal Medicine (Impact Factor: 3.42). 07/2008; 23(6):883-6. DOI: 10.1007/s11606-008-0627-8
Source: PubMed


A 43-year-old female with systemic lupus erythematosus (SLE) was admitted with fever and shortness of breath 1 month after aortic valve replacement. A diagnostic workup including chemistries, complete blood count, blood cultures, chest x-ray, and 2-D echocardiogram was performed to determine the etiology of her symptoms and differentiate between acute bacterial endocarditis and Libman-Sacks endocarditis.
By utilizing Duke's criteria, antiphospholipid antibodies, and serial echocardiography, we were able to make a diagnosis of Libman-Sacks endocarditis. The patient was successfully treated for Libman-Sacks endocarditis and recovered uneventfully.
This case highlights the challenges of making the correct diagnosis when 2 disease processes present with similar findings.

    • "Libman-Sacks endocarditis is the most common cardiac manifestation in patients with SLE and primary antiphospholipid syndrome. It is characterized by vegetations associated with the valves, valve thickening or fibrosis, valve regurgitation and rarely valve stenosis.[45] It is most commonly clinically associated with cardioembolism (predominantly to the brain) and it is difficult to diagnose.[4] "
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    ABSTRACT: Background:Systemic lupus erythematosus (SLE) is characterized by the presence of anti-nuclear antibodies (ANAs) in the serum of patients. These antibodies may cross over into the brain resulting in the development of neuropsychiatric symptoms and result in abnormal pathology in other organs such as the heart and kidneys.Objective:The objective of this study was to determine if SLE pathology could be detected in the hearts and brains of rats injected with positive human ANA serum.Materials and Methods:Lewis rats (n = 31) were selected for this study due to documented research already performed with this strain in the investigation of serum sickness, encephalitis and autoimmune related carditis. Rats were injected once a week with either ANA positive or negative control serum or saline. Hearts were examined for initial signs of heart disease including the presence of lipid deposits, vegetation, increased ventricular thickness and a change in heart weight. Brains were examined for the presence of human antibody and necrotic lesions. Animals were observed for outward signs of neuropathy as well. Blood samples were taken in order to determine final circulating concentrations of IgG and monitor histamine levels.Results:Animals injected with ANA were significantly higher for lipid deposits in the heart and an increased ventricular thickness was noted. One animal even displayed Libman-Sacks endocarditis. Brains were positive for the presence of human IgG and diffuse internal lesions occurred in 80% of the ANA positive serum injected animals examined. Blood histamine levels were not significantly different, but actually lower than controls by the end of the experiment.Conclusion:Since human antibodies were detected in the brain, further studies will have to identify which antibody cross reactions are occurring within the brain, examine cell infiltration as well as characterize the antibodies associated with more destructive consequences such as lesion formation.
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    • "The patient was treated with corticosteroids, despite numerous side-effects and controversies associated with its use with marked clinical improvement. Corticosteroids prevent SLE relapse (Bootsma et al., 1995) and patient tends to live longer (Menard, 2008). "
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    ABSTRACT: Systemic lupus erythematosus is relatively common medical disorder with female predominance. This disorder can affect any organ system. Cardiac involvement is variable which can include pericardium, myocardium and endocardium. The endocardial involvement commonly affects mitral and aortic valves. This report discusses lupus endocarditis in young man with atypical presentation.
    Full-text · Article · Jul 2010
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    • "The modified Duke criteria can be useful in helping differentiate between true infective endocarditis and LS endocarditis [28]. Helpful laboratory markers in distinguishing infective endocarditis from LS endocarditis are the white blood cell count (elevated in infective endocarditis and often decreased in LS endocarditis), C-reactive protein levels (elevated in infective endocarditis and relatively low in LS endocarditis), aPL levels (normal in infective endocarditis and moderate to high in LS endocarditis), and (repeated) blood cultures (positive in infective endocarditis and negative in LS endocarditis) [21,29]. Echocardiographically, LS vegetations appear as valve masses of varying size and shape with irregular borders and echodensity, they are firmly attached to the valve surface and exhibit no independent motion [8]. "
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    ABSTRACT: Libman-Sacks endocarditis of the mitral valve was first described by Libman and Sacks in 1924. Currently, the sterile verrucous vegetative lesions seen in Libman-Sacks endocarditis are regarded as a cardiac manifestation of both systemic lupus erythematosus (SLE) and the antiphospholipid syndrome (APS). Although typically mild and asymptomatic, complications of Libman-Sacks endocarditis may include superimposed bacterial endocarditis, thromboembolic events, and severe valvular regurgitation and/or stenosis requiring surgery. In this study we report two cases of mitral valve repair and two cases of mitral valve replacement for mitral regurgitation (MR) caused by Libman-Sacks endocarditis. In addition, we provide a systematic review of the English literature on mitral valve surgery for MR caused by Libman-Sacks endocarditis. This report shows that mitral valve repair is feasible and effective in young patients with relatively stable SLE and/or APS and only localized mitral valve abnormalities caused by Libman-Sacks endocarditis. Both clinical and echocardiographic follow-up after repair show excellent mid- and long-term results.
    Full-text · Article · Mar 2010 · Journal of Cardiothoracic Surgery
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