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 (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

  • Thrombotic endocarditis develops due to endothelial damage and subsequent exposure of the subendothelial connective tissue to circulating platelets
  • The factors involved in pathogenesis can be divided into initiating NBTE and subsequent development of vegetation
  • Factors implicated in initiation are: (a) immune complexes, (b) hypoxia, (c) hypercoagulability and (d) carcinomatosis
    • Immune complexes: Libman-Sacks endocarditis is the prototype
    • Hypoxia: studied by Nakanishi et al. in a rodent model
    • Hypercoagubility: Trousseau first noted the association between thrombosis and malignancy
      • Histological evidence of disseminated intravascular coagulopathy (DIC) has been found in 50% patients with NBTE
    • Carcinomatosis: mucin producing adenocarcinoma from the gut, lung and ovary and acute promyelocytic leukemia are commonly associated with NBTE
  • The pathology is the same as nonbacterial thrombotic endocarditis except focal necrosis (hematoxylin bodies) can be found only in Libman-Sacks endocarditis.

Gross pathology

NBTE vegetations are typically small, friable, white or tan masses, < 1 cm in diameter, broad based and irregular, usually along lines of valve closure on leaflets which may be normal or previously damaged Vary from tiny lesions to large and exuberant masses Based on morphology, Allen and Sirota proposed a macroscopic classification of NBTE: Type 1: Small, < 3 mm univerrucal, firmly attached to the valve Type 2: Large, > 3mm univerrucal, adherent to the valve Type 3: Small, 1 - 3mm multiverrucal, friable

Microscopic Pathology

NBTE consists of degenerating platelets interwoven with strands of fibrin and forming a bland, featureless eosinophilic mass except for a few trapped leucocytes Three stages have been described in the evolution of NBTE vegetations: (eMedicine: Libman-Sacks Endocarditis Workup [Accessed 28 February 2018]) Active verrucae: Consist of clumps of fibrin on and within the valvular leaflet tissue which is focally necrotic, with plasma cells and lymphocytes Combined active and healed lesions: Contain vascularized, fibrous tissue adjacent to fibrinous and necrotic areas Healed lesions: Consist of dense, vascularized, fibrous tissue

Epidemiology and Demographics

Risk Factors

  • Advanced stage malignancy: solid organ or hematological
  • Chronic diseases: tuberculosis, uremia, AIDS
  • Connective tissue disorders with hypercoaguable state: SLE patients with APLA positive
  • Trauma from indwelling pulmonary catheter or central venous catheter, snake bite, late effect of radiation therapy

Natural History, Complications and Prognosis

Complications

Prognosis

Diagnosis

  • Requires a high degree of clinical suspicion in a patient treated for infective endocarditis (IE) and not clinically improving
  • Mckay and Wahler proposed a triad for diagnosis of NBTE:
    • Presence of a disease process known to be associated with NBTE
    • Presence of heart murmur and
    • Evidence of multiple systemic emboli

History and symptoms

Patients can present with:

  • Cardiac failure
    • Secondary to valvular dysfunction (most commonly mitral regurgitation), leading to dyspnea, orthopnea, paroxysmal nocturnal dyspnea, peripheral edema, lethargy
  • Cerebrovascular embolism
    • Focal weakness or numbness, visual loss, dysphasia, dysarthria, dysphagia, memory loss
  • Systemic thromboembolism
    • Pain, coldness and numbness of the peripheries, or acute abdominal syndromes with pain and vomiting
  • Secondary infective endocarditis
    • Fever, weight loss, night sweats, lethargy, chest pain

Physical Examination

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
Chest X-Ray

Other Diagnostic studies

Cardiac Catheterization

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%.

Treatment is difficult - correction of the underlying cause is of paramount importance In patients with potentially curable cancer, coagulopathy should be corrected and, if there is no contraindication, these patients should be anticoagulated with heparin There are no guidelines for surgical intervention in patients with NBTE - decision is based upon individual case

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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