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Study population recruitment, selection, evaluations, and treatment. https://doi.org/10.1371/journal.pone.0247052.g001
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Background
Libman-Sacks endocarditis in patients with systemic lupus erythematosus (SLE) is commonly complicated with embolic cerebrovascular disease (CVD) or valve dysfunction for which high-risk valve surgery is frequently performed. However, the role of medical therapy alone for Libman-Sacks endocarditis and associated acute CVD remains undefine...
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Context 1
... assess the effect of medical therapy on Libman-Sacks endocarditis and its associated embolic CVD, 17 patients with both conditions underwent repeat clinical, laboratory, cardiac and cerebrovascular evaluations after a median of 6 months (interquartile range, 2.1-9.6) of clinically indicated and patients' provider guided anti-inflammatory and anti-thrombotic therapy. These 17 patients [age 36 ± 12 years (range, 18-57), 14 (82%) women, 53% Hispanic, with body mass index of 27.12 ± 7.5 Kg/m 2 , age at onset of SLE 29.31 ± 12.08, and SLE duration of 7.53 ± 6.10 years] constitute this study population and their selection is summarized in Fig 1. All initial and follow-up studies were coded, de-identified, randomly intermixed, and interpreted by experienced observers blinded to subjects' clinical and imaging data, baseline findings, and each other's readings. ...
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Citations
... Inflammatory heart diseases are particularly debilitating, refractory to treatment (especially long-term), and often fatal [44,65]. Disorders in this category include such conditions as endocarditis, in which the heart's inner layer, the endocardium, becomes inflamed, with resulting damage to the heart valves [66]. Likewise, cardiomegaly, a disorder characterized by the hypertrophic enlargement of the organ, with resulting loss of function, and myocarditis, a disease process in which monocytes and lymphocytes or eosinophiles infiltrate the heart muscle, are consequences of a failure to regulate inflammatory signaling processes, with consequent tissue damage [67][68][69][70][71][72]. ...
This report describes major pathomechanisms of disease in which the dysregulation of host inflammatory processes is a major factor, with cardiovascular disease (CVD) as a primary model, and reviews strategies for countermeasures based on synergistic interaction between various agents, including drugs and generally regarded as safe (GRAS) natural medical material (NMM), such as Ginkgo biloba, spice phytochemicals, and fruit seed flavonoids. The 15 well-defined CVD classes are explored with particular emphasis on the extent to which oxidative stressors and associated ischemia-reperfusion tissue injury contribute to major symptoms. The four major categories of pharmaceutical agents used for the prevention of and therapy for CVD: statins, beta blockers (β-blockers), blood thinners (anticoagulants), and aspirin, are presented along with their adverse effects. Analyses of major cellular and molecular features of drug- and NMM-mediated cardioprotective processes are provided in the context of their development for human clinical application. Future directions of the evolving research described here will be particularly focused on the characterization and manipulation of calcium- and calcineurin-mediated cascades of signaling from cell surface receptors on cardiovascular and immune cells to the nucleus, with the emergence of both protective and pathological epigenetic features that may be modulated by synergistically-acting combinations of drugs and phytochemicals in which phytochemicals interact with cells to promote signaling that reduces the effective dosage and thus (often) toxicity of drugs.
... Discussion LSE is rare in children. Patients with antiphospholipid, antiro/SS-A, and anti-la/SS-B antibody-positive SLE are more likely to have valve involvement than those without (3,4). Patients with LSE are typically asymptomatic unless the lesions progress to more severe valvular dysfunction or embolic events, which may be related to highly variable SLE activity (alternating between exacerbations and remissions) and standard anti-inflammatory and/or anti-thrombotic therapy (1). ...
... However, a case report involving a pregnant woman revealed that following hormone treatment (80 mg/day), the patient experienced a decrease in vegetations and an improvement in ejection fraction (19). Anti-inflammatory and anti-thrombotic therapies have been shown to reduce the activity of SLE, resolve or significantly improve Libman-Sacks vegetation and valve regurgitation, and cause a pathological shift from an active to a mixed or healed type of Libman-Sacks vegetation, which lowers the risk of embolization and may prevent the requirement for high-risk valve surgery (3). According to the 2015 European Society of Cardiology guidelines for the management of infective endocarditis, anticoagulation therapy should be pursued in patients with NBTE if there are no contraindications (20). ...
... This suggests that the management and therapeutic strategies for children with LSE and antiphospholipid antibody positivity differ from those in adult patients (21). Furthermore, several adult case studies have previously demonstrated that LSE can be resolved or improved by the administration of immunosuppressive medications in conjunction with antithrombotic and hydroxychloroquine therapy (3,(22)(23)(24)(25)(26). However, the specific time range during which LSE vegetation can disappear after early active treatment, which requires future multicenter clinical validation, has not yet been documented in the literature. ...
Libman-Sacks endocarditis (LSE) is a cardiac condition characterized by the growth of verrucous vegetation. Although relatively rare in children, LSE is nevertheless a known cardiac manifestation of autoimmune diseases, including systemic lupus erythematosus (SLE). The mitral valve is the most commonly affected region, followed by the aortic valve, while the tricuspid and pulmonary valves are rarely affected. The management of established Libman-Sacks vegetation poses significant challenges, often necessitating surgical interventions, although surgery is not the primary treatment modality. Herein, we present the case of a 14-year-old Chinese female patient whose initial lupus manifestation included LSE, among other symptoms and signs that provided insights into the final diagnosis of SLE. After early comprehensive pharmacological treatment, tricuspid regurgitation and vegetation disappeared within 28 days without necessitating cardiac surgery, indicating that the resolution of LSE vegetation in this patient was achieved through a combination of immunosuppressive and anticoagulant therapy. These findings suggest the potential of this treatment approach as a viable model for the management of LSE in young patients.
... While the treatment of lupus is well defined, that of Libman-Sacks endocarditis is unclear, and relies mainly on anti-inflammatory therapy [9]. Corticosteroids have anti-inflammatory and antithrombotic aims, but may accelerate fibrosis and hence valve dysfunction, making them controversial. ...
... Immunosuppressants and hydroxychloroquine aim to reduce SLE activity. However, there is no consensus on the efficacy of these therapies on valve damage [8,9]. Treatment of heart failure, if present, should also be initiated. ...
Libman-Sacks endocarditis (LSE) is a characteristic but rare feature of systemic lupus erythematosus (SLE), which can be found in association with antiphospholipid syndrome (APS), exposing the patient to an increased embolism risk, particularly the occurrence of ischemic stroke.
We present a case involving a 64-year-old man who was admitted for ischemic stroke accompanied by a fever. Cardiac investigation revealed mitral vegetations along with severe mitral regurgitation. The diagnosis of Libman-Sacks endocarditis associated with SLE and APS was made after a laboratory work-up showing negative blood cultures and positive antibodies. This case underlines the importance of early diagnosis for better management of this pathology.
... Zapalenie wsierdzia Libmana-Sacksa w SLE w większości przypadków przebiega bezobjawowo. Jest częstym powikłaniem CVDs w toczniu rumieniowatym układowym i może być przyczyną wtórnej choroby zakrzepowo-zatorowej. W wegetacjach obecnych na zastawkach serca odkładają się immunoglobuliny IgG, IgM i IgA, przeciwciała przeciwko kardiolipinie i składniki dopełniacza C1q, C3 i C4 [26,27]. ...
Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease of the connective tissue occurring mainly in the female population. It is associated with a much higher cardiovascular risk compared to the general population, and the incidence is estimated at about 10%. SLE is associated with a 15 times higher risk of atherosclerosis and a 4 times higher risk of myocardial infarction than in the general population. Mortality due to cardiovascular complications peaks 4 to 20 years after diagnosis. Accelerated atherosclerosis is associated with increased expression of pro-inflammatory cytokines, prothrombotic mediators, oxidative stress products and abnormal vascular tone. It should be noted that the diagnosis of SLE itself is associated with the presence of premature subclinical atherosclerosis. The aim of this study was to review the latest reports on the pathogenesis and cardiovascular complications in systemic lupus erythematosus.
Keywords: Systemic lupus erythematosus, Atherosclerosis, Cardiovascular disease, Endothelial dysfunction
... 7,8 It has been suggested that cardiovascular and cerebrovascular diseases associated with LSE are usually resolved or significantly improved by traditional antiinflammatory and anti-thrombotic therapy. 9 In the present case, the ICU doctors used heparin anticoagulation, methylprednisolone, hydroxychloroquine sulfate, and cyclosporine to regulate and suppress the SLE activity. After 4 days of hospitalization, the patient's hematologic, liver, and kidney function returned to normal. ...
This case report describes a 47-year-old woman with systemic lupus erythematosus (SLE) complicated by Libman–Sacks endocarditis (LSE) who developed multiple organ dysfunction after mitral valve replacement surgery. The patient presented with a 5-day history of cough, sputum, and fever. Transthoracic echocardiography showed significant vegetations on the mitral valve. Biopsy was performed, and the pathological diagnosis was SLE complicated by LSE. After the mitral valve replacement surgery, the patient developed clinical manifestations of hepatic and renal dysfunction, cardiopulmonary failure, oliguria, and shock. The clinical symptoms significantly improved after administration of mechanical ventilation, continuous renal replacement therapy, plasma exchange, anti-inflammatory and anti-infection treatments, immunomodulatory and immunosuppressive therapies, and low-molecular-weight heparin anticoagulation. Multiple organ dysfunction after mitral valve replacement in patients with SLE complicated by LSE has rarely been reported. This report discusses the clinical manifestations, pathogenesis, and treatment of this severe complication. We hope the sharing of our experience in this case will provide a clinical basis for the treatment of severe multiple organ dysfunction after mitral valve replacement in patients with SLE complicated by LSE.
... Diagnosing and treating the underlying condition is crucial, although it remains unclear whether these steps contribute to preventing NBTE relapse. For example, conflicting data have been reported on the association between SLE disease activity and the risk of Libman-Sacks endocarditis [35,123]. Additionally, the role of immunosuppressive treatment in preventing the formation, progression, and complications of thrombotic endocardial vegetations in patients with autoimmune diseases requires further investigation. ...
Nonbacterial thrombotic endocarditis (NBTE) is a form of endocarditis that occurs in patients with predisposing conditions, including malignancies, autoimmune diseases (particularly antiphospholipid antibody syndrome, which accounts for the majority of lupus-associated cases), and coagulation disturbances for which the correlation with classical determinants is unclear. The condition is commonly referred to as “marantic”, “verrucous”, or Libman–Sacks endocarditis, although these are not synonymous, representing clinical–pathological nuances. The clinical presentation of NBTE involves embolic events, while local valvular complications, generally regurgitation, are typically less frequent and milder compared to infective forms of endocarditis. In the past, the diagnosis of NBTE relied on post mortem examinations, while at present, the diagnosis is primarily based on echocardiography, with the priority of excluding infective endocarditis through comprehensive microbiological and serological tests. As in other forms of endocarditis, besides pathology, transesophageal echocardiography remains the diagnostic standard, while other imaging techniques hold promise as adjunctive tools for early diagnosis and differentiation from infective vegetations. These include cardiac MRI and 18FDG-PET/CT, which already represents a major diagnostic criterion of infective endocarditis in specific settings. We will herein provide a comprehensive review of the current knowledge on the clinics and therapeutics of NBTE, with a specific focus on the diagnostic tools.
... 14 A recent study highlighted the role of anti-inflammatory and antithrombotic therapy for obviating surgery in patients with LSE and cerebrovascular disease. 15 Our patient had risk factors for IE; she did fulfil the modified Dukes criteria for IE and was treated as a case of culture-negative endocarditis. ...
Background
Cardiovascular involvement is frequent in systemic lupus erythematosus (SLE). Valvular abnormalities are increasingly being recognized with the advent of echocardiography.
Case summary
We present a case of a 46-year-old lady who presented to the emergency department with upper limb ischaemia. On examination, she had poor dentition and a short systolic murmur on auscultation. A blood workup revealed a diagnosis of SLE. Further investigations showed vegetations on the mitral valve. Initially, an infective endocarditis (IE) diagnosis was made, which was treated with antibiotics. High-dose steroids and immunosuppressants were initiated due to her clinical deterioration and biopsy-proven lupus nephritis. She improved clinically before being discharged home.
Discussion
It can be difficult to distinguish between IE and Libman–Sacks endocarditis (LSE), especially in the setting of risk factors for both. Antibiotics and immunosuppressants might be started simultaneously in these cases. A multidisciplinary team is required to manage challenging cases of culture-negative endocarditis. Procalcitonin may have a role in differentiating bacterial endocarditis and LSE.
... However, the presence of Libman-Sacks vegetations is associated with varying degrees of valvular dysfunction, increased risk for stroke and transient ischemic attacks, and increased mortality (1,2). Early detection and initiation of medical therapy may resolve LSE and prevent valvular deterioration (3). In previous studies, left-sided valvular Libman-Sacks vegetations were more frequently detected than right sided vegetations; reported cases of bilateral involvement is very rare (1). ...
... Treatment mainly involves immunosuppressive therapy for underlying lupus and systemic anticoagulation (11). Surgery in LSE is associated with high mortality and should be reserved only for patients having severe valvular dysfunction, very large vegetations (greater than 2 centimeters), or recurrent thromboembolism despite therapeutic anticoagulation, after weighing the benefits and risks of surgery (3,11). LSE is associated with cerebrovascular embolism, focal brain lesions, and neuropsychiatric involvement (1,2). ...
... Conversely, patients who exhibit deep WMH on brain MRI scans have increased risk for ischemic stroke (12). Early anti-inflammatory and antithrombotic therapy might resolve vegetations-induced valvular dysfunction, improve cerebral perfusion, and avoid the need for surgery (3). ...
Libman-Sacks endocarditis accounts for 6-11 percent of systemic lupus erythematosus patients and is associated with varying degrees of valvular dysfunction, increased risk for stroke and transient ischemic attacks, and increased mortality. In previous studies, left-sided valvular Libman-Sacks vegetations were more frequently detected than right sided vegetations; reported cases of bilateral involvement is very rare. A comprehensive clinical assessment and the multimodality imaging is of utmost importance in the management of systemic lupus erythematosus. In this case report, we describe a 31-year-old female patient with uncontrolled systemic lupus erythematosus initially presented with gastrointestinal symptoms but eventually had a vegetation-like structure on the posterior leaflet of the mitral valve which was revealed during routine echocardiography. Two-dimensional/three-dimensional transthoracic and transesophageal echocardiography, cardiac magnetic resonance, and cardiac computed tomography further characterized the mitral valve vegetation and revealed an additional vegetation of the pulmonary valve. Echocardiography remains the cornerstone for the detection of Libman-Sacks vegetations. Cardiac MRI and cardiac CT are useful in characterizing lesion size and effects and may prove particularly helpful in the assessment of right-sided or multivalvular endocarditis. The presence of focal brain lesions on brain MRI prompted antithrombotic therapy.
... Available observational data are mixed, with most studies finding no association between combined immunosuppressive and antithrombotic/ anticoagulant therapy and vegetation remission [86][87][88]. One recent study in patients with systemic lupus erythematosus describes improvement in valvular lesions following treatment initiation [89]. In clinical practice, management involves anticoagulation and targeted management of the underlying disorder [82]. ...
Purpose of Review
Embolic stroke of undetermined source is a challenging clinical entity. While less common than atrial fibrillation and endocarditis, many noninfective heart valve lesions have been associated with stroke and may be considered as culprits for cerebral infarcts when other more common causes are excluded. This review discusses the epidemiology, pathophysiology, and management of noninfective valvular diseases that are commonly associated with stroke.
Recent Findings
Calcific debris from degenerating aortic and mitral valves may embolize to the cerebral vasculature causing small- or large-vessel ischemia. Thrombus which may be adherent to calcified valvular structures or left-sided cardiac tumors may also embolize resulting in stroke. Tumors themselves, most commonly myxomas and papillary fibroelastomas, may fragment and travel to the cerebral vasculature.
Summary
Despite this broad differential, many types of valve diseases are highly comorbid with atrial fibrillation and vascular atheromatous disease. Thus, a high index of suspicion for more common causes of stroke is needed, especially given that treatment for valvular lesions typically involves cardiac surgery whereas secondary prevention of stroke due to occult atrial fibrillation is readily accomplished with anticoagulation.
... In a recent longitudinal and cross-sectional study, patients with SLE and Libman-Sacks endocarditis complicated by embolic cerebrovascular disease were treated with conventional anti-inflammatory and antithrombotic therapy for six months; on the follow-up TEE, 76% of the patients experienced resolved or improved valvular vegetation or regurgitation. 22 In another six-year longitudinal and cross-sectional study, 16% of patients had new or recurrent stroke, 14% had cognitive disability, and 9% died. 16 ...
