Libman-Sacks endocarditis

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Libman-Sacks endocarditis Microchapters

Overview

Historical Perspective

Pathophysiology

Epidemiology and Demographics

Risk Factors

Natural History, Complications and Prognosis

Diagnosis

History and Symptoms

Physical Examination

Laboratory Findings

Imaging Findings

Other Diagnostic Studies

Treatment

Differential Diagnosis

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1];Associate Editor(s)-in-Chief: Sara Mohsin, M.B.B.S.[2]

Synonyms and Keywords: : Nonbacterial thrombotic endocarditis (NBTE), Marantic endocarditis, Verrucous endocarditis

Overview

Libman-Sacks endocarditis (LSE) is a form of nonbacterial thrombotic endocarditis (NBTE) that is considered to be the most common cardiac manifestation seen in patients with systemic lupus erythematosus. LSE is a term used for sterile and verrucous vegetations around the heart valves mostly affecting the mitral and aortic heart valves but other valves may also be involved. Valvular involvement in LSE may lead to valvular regurgitation, aortic insufficiency, thromboembolic cerebrovascular events, and increased risk of infective endocarditis. It is also usually associated with the other autoimmune diseases such as antiphospholipid syndrome (APS) and some malignancies. Secondary APS has a higher rate of cardiac involvement as compared to primary APS, mostly due to the autoimmune causes related to the SLE. LSE can be complicated by embolic cerebrovascular disease, superimposed infective endocarditis, and peripheral arterial embolism. It is also associated with increased mortality, hence, early recognition of LSE and appropriate treatment are of significant importance in preventing any further complications.

Historical Perspective

Pathophysiology

Pathology

Factors responsible for the initiation of Libman-Sacks endocarditis
Initiation factor Description
Immune complexes
Hypoxia
Hypercoagulability
Carcinomatosis Following carcinomas are commonly associated with NBTE and Libman-Sacks endocarditis:

Gross pathology

Allen and Sirota macroscopic classification of NBTE
Type of NBTE Features
Type 1
  • Small
  • < 3 mm
  • Univerrucal
  • Firmly attached to the valve
Type 2
Type 3
  • Small
  • 1 - 3 mm
  • Multiverrucal
  • Friable
Libman Sacks Endocarditis. The presence of vegetations predisposes patients to bacterial endocarditis. Source: Brigden et al,1960.

Microscopic Pathology

Stages of evolution of vegetations in Libman-Sacks endocarditis
Stage Description
Stage 1 (active verrucae)
Stage 2 (Combined active and healed lesions)
Stage 3 (Healed lesions)
Pathology slide of mitral valve vegetation. Lots of necrosis: 10 cm circumference vegetation. Mitral valve tissue shows focal necrosis. No bacterial or fungal organisms were present. Source: Ghulam Murtaza. et al, Department of Internal Medicine, Advocate Christ Medical Center, Oak Lawn, IL, USA
High power pathology slide of the liver showing lots of steatosis, congestion, and necrosis. Source: Ghulam Murtaza. et al, Department of Internal Medicine, Advocate Christ Medical Center, Oak Lawn, IL, USA

Epidemiology and Demographics

Risk Factors

Risk factors for the development of Libman-Sacks endocarditis
Risk factor Details
Advanced stage malignancy Advanced stage malignancies such as:
Chronic diseases Chronic diseases such as:
Connective tissue disorders with hypercoagulable state
Trauma Trauma due to:

Natural History, Complications and Prognosis

Complications

Prognosis

Diagnosis

Mckay and Wahler triad for diagnosis of NBTE
1:
2:
3:

History and Symptoms

Common manifestations of patients with Libman-Sacks endocarditis
Manifestation Description
Heart failure Heart failure can occur secondary to the valvular dysfunction (most commonly mitral regurgitation), leading to the following signs and symptoms:
Cerebrovascular embolism[29] Cerebrovascular embolism can present as any of the following:
Systemic thromboembolism Systemic thromboembolism can cause any of the following:
Secondary infective endocarditis Secondary infective endocarditis can present as:

Physical Examination

Physical examination findings in a patient of Libman-Sacks endocarditis
Pathology Physical examination finding
Left ventricular hypertrophy[30][31][32][33][34][35] LVH can present as any of the following:
Congestive heart failure Physical examination findings of CHF include:
Infective endocarditis (IE)[36][37][38][39] IE can present as:
Mitral valve disease[40]
Aortic valve disease
Tricuspid valve disease

Laboratory Findings

Laboratory Investigations in Libman-Sacks Endocarditis Laboratory test findings
Blood culture
SLE investigations

(Immunological assays)

CBC
Studies to rule out DIC
Polymerase chain reaction (PCR)


Imaging Findings

Imaging tests in Libman-Sacks Endocarditis Imagining Findings
Echocardiography[41][26][42]
2D transesophageal ultrasound. Image on the left shows a thickened mitral valve with a 1 cm vegetation that can be seen on the anterior mitral leaflet. Image on the right is a four-chamber color flow Doppler view showing biventricular dilatation, severe left ventricular dysfunction. For orientation purposes, left ventricle is the bottom right chamber. Video of both views is attached as a supplementary file. Source: Ghulam Murtaza. et al, Department of Internal Medicine, Advocate Christ Medical Center, Oak Lawn, IL, USA
Chest X-Ray
MRI[43]
MRI of the brain. Images on the top show increased signal on diffusion weighted imaging (DWI) throughout the bilateral frontal, parietal, and occipital lobes. Images on the bottom show a corresponding decreased signal intensity on apparent diffusion coefficient that is consistent with acute abnormal restricted diffusion. These findings suggest new/ongoing acute infarcts.Source: Ghulam Murtaza. et al, Department of Internal Medicine, Advocate Christ Medical Center, Oak Lawn, IL, USA
CT scan (Head)
CT of head without contrast showing extensive multifocal areas of hypoattentuation throughout the bilateral frontal, parietal, occipital, and right > left temporal lobes. No mass effect or midline shift or hemorrhage was seen.Source: Ghulam Murtaza. et al, Department of Internal Medicine, Advocate Christ Medical Center, Oak Lawn, IL, USA

Other Diagnostic Studies

Cardiac Catheterization

Treatment

Common treatment options for Libman-Sacks endocarditis depending on the underlying cause
Treatment option Details
Steroids and immunosuppressive agents They are useful in the treatment of underlying disease but they have a controversial role in the pathogenesis of vegetations:
Anticoagulants
Other medications
Valve replacement[7][44][11]

Differential Diagnosis

Libman-Sacks endocarditis should be differentiated from other diseases presenting with fever, chest pain and anorexia. The differentials include the following:[45][46][47][48][49][50][51][52][53][54][55][56][57][58][59][60][61][62][63][64]

Diseases Diagnostic tests Physical Examination Symptoms Past medical history Other Findings
CT scan and MRI EKG Chest X-ray Tachypnea Tachycardia Fever Chest Pain Hemoptysis Dyspnea on Exertion Wheezing Chest Tenderness Nasalopharyngeal Ulceration Carotid Bruit
Pulmonary embolism
  • On CT angiography:
    • Intra-luminal filling defect
  • On MRI:
    • Narrowing of involved vessel
    • No contrast seen distal to obstruction
    • Polo-mint sign (partial filling defect surrounded by contrast)
✔ (Low grade) ✔ (In case of massive PE) - - - -
Infective Endocarditis
  • Goldberg's criteria may aid in diagnosis of left ventricular dysfunction: (High specificity)
    • SV1 or SV2 + RV5 or RV6 ≥3.5 mV
    • Total QRS amplitude in each of the limb leads ≤0.8 mV
    • R/S ratio <1 in lead V4
- - - - - -
Non-Bacterial Thrombotic Endocarditis
  • ST elevation
  • PR depression
✔ (Low grade) ✔ (Relieved by sitting up and leaning forward) - - - - -
  • May be clinically classified into:
    • Acute (< 6 weeks)
    • Sub-acute (6 weeks - 6 months)
    • Chronic (> 6 months)
Libman Sack Endocarditis - - - -
Vasculitis

Homogeneous, circumferential vessel wall swelling

-
Fever of unknown origin (FUO) - - - - - -

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