Libman-Sacks endocarditis

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Libman-Sacks endocarditis
ICD-10 I39, M32.1
ICD-9 710.0
DiseasesDB 29254
eMedicine med/1295 
MeSH D008180

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

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

Overview

Libman-Sacks endocarditis (LSE) is a form of nonbacterial thrombotic endocarditis 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

  • The pathology is the same as nonbacterial thrombotic endocarditis except focal necrosis (hematoxylin bodies) can be found only in Libman-Sacks endocarditis.

Epidemiology and Demographics

Natural History, Complications and Prognosis

Complications

Prognosis

History and symptoms

Physical Examination

Laboratory findings

Laboratory investigations in Libman-Sacks Endocarditis Laboratory test findings
Blood culture
  • It is important to rule out coexisting infective endocarditis by taking blood cultures.
SLE investigations
  • Investigations for SLE are positive including:
    • Antinuclear antibodies
    • Anti-dsDNA antibodies
    • Anti-Smith antibodies
    • Anti-RNP
    • Antiphospholipid antibodies
    • Anticardiolipin antibodies (associated with increased risk of cardiac abnormalities)
    • Anti-Ro/SSA
    • Anti-La/SSB
    • False-positive serology in the form of VDRL is also common in SLE
CBC
  • CBC may show:
    • Neutrophilia
    • Anemia

Imaging findings

Imaging tests in Libman-Sacks Endocarditis Imagining Findings
Echocardiography
  • Echocardiography should be employed, although not all lesions will be detected.
  • Results with transoesophageal echo are superior but it is an invasive procedure.
  • As it is commonly complicated with embolic cerebrovascular disease. Thus, accurate detection of Libman-Sacks vegetations may lead to early therapy and prevention of their associated complications. Although two-dimensional transesophageal echocardiography (2D-TEE) has high diagnostic value for detection of Libman-Sacks vegetations, three-dimensional TEE (3D-TEE) may allow improved detection, characterization, and clinical correlations of Libman-Sacks vegetations. 3D-TEE provides clinically relevant additive information that complements 2D-TEE for the detection, characterization, and association with cerebrovascular disease of Libman-Sacks endocarditis.[25]
Chest X-Ray
  • CXR may show cardiomegaly and pulmonary congestion if the disease is severe.
  • Calcification of lesions is uncommon.

Other Diagnostic studies

Cardiac Catheterization

  • If valvular disease seems severe then cardiac catheterization may be required with a view to valve replacement.

Treatment

  • There is no specific treatment for Libman-Sacks endocarditis.
  • Steroids and immunosuppressive agents are useful in the treatment of the underlying disease but there is some controversy about their role in the pathogenesis of vegetations:
    • Compared with reports of postmortems on patients before the advent of steroids, those that have been treated have smaller and fewer lesions, mostly on one valve and usually confined to the left side.
    • Hypertension was 5 times as common and congestive heart failure was 8 times as common as in the days before corticosteroids. The steroids may be directly responsible for hypertension and heart failure. This paper was from 1975 and it is possible that better control of hypertension and heart failure may offset these problems today.
    • Expert opinion seems to conclude that steroids are detrimental to Libman-Sacks endocarditis although some praise them. The situation is far from certain.
  • Advice for procedures creating a risk of infective endocarditis can be found in the separate record Prevention of Endocarditis.
  • If there are systemic emboli then anticoagulation with warfarin is beneficial but the possible role of aspirin has not been adequately investigated. If there is evidence of 1 cerebrovascular event, anticoagulation is advised.
  • In serious valve disease it may be necessary to replace valves. Mechanical valves may be more susceptible to thromboemboli but it is uncertain if bioprosthetic valves are at risk of the disease. The operative mortality of mitral valve replacement in this condition may be as high as 25%.

Differential Diagnosis

Libman-Sacks endocarditis should be differentiated from other diseases presenting with fever, chest pain and anorexia. The differentials include the following:[26][27][28][29][30][31][32][33][34][35][36][37][38][39][40][41][42][43][44][45]

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) - - - - - -

References

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