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Libman Sacks endocarditis

Summary

  • Nonbacterial thrombotic endocarditis associated with systemic lupus erythematosus (SLE)
  • Characterised by sterile vegetations on heart valves, predominantly mitral and aortic
  • Diagnosis based on clinical presentation, echocardiography, and exclusion of infective endocarditis

Pathophysiology

  • Immune complex deposition and complement activation on valve surfaces
  • Endothelial injury and platelet aggregation leading to vegetation formation
  • Valvular dysfunction due to thickening, fibrosis, and scarring
  • Associated with antiphospholipid antibodies in many cases

Demographics

  • Predominantly affects young to middle-aged adults with SLE
  • More common in females (reflecting SLE demographics)
  • Prevalence in SLE patients: 30-50% (based on echocardiographic studies)
  • Higher prevalence in patients with active SLE and longer disease duration

Diagnosis

  • Often asymptomatic; may present with heart murmurs or embolic phenomena
  • Echocardiography (transthoracic or transesophageal) is the primary diagnostic tool
  • Blood cultures to exclude infective endocarditis
  • Antiphospholipid antibody testing
  • SLE disease activity assessment

Imaging

  • Echocardiography:
    • Vegetations: typically small (<10mm), irregular, and sessile
    • Most common locations: mitral valve (anterior leaflet) and aortic valve
    • Valvular thickening and regurgitation may be present
  • Cardiac MRI:
    • Can detect vegetations and assess valvular function
    • Useful for evaluating associated myocardial involvement
  • CT angiography:
    • May detect larger vegetations and valvular calcifications
    • Useful for evaluating embolic complications

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  • 60-year-old patient with antiphospholipid syndrome presented with left sided weakness.
  • MRI showed acute infarcts in multiple arterial territories.
  • Transoesophageal echocardiogram (TOE) showed tricuspid and aortic vegetations witih regurgitation.

Treatment

  • Management of underlying SLE with immunosuppressive therapy
  • Anticoagulation in patients with antiphospholipid antibodies or thromboembolic events
  • Valve repair or replacement for severe valvular dysfunction
  • Regular echocardiographic follow-up to monitor progression
  • Antibiotic prophylaxis for invasive procedures (controversial)

Differential diagnosis

Differential diagnosis Differentiating feature
Infective endocarditis with septic emboli Multiple ring-enhancing micro-abscesses on MRI; haemorrhagic transformation; cortical and deep grey infarcts
Atrial fibrillation-related cardioembolic stroke Multiple cortical infarcts in different vascular territories; no valvular vegetations on echocardiography
Antiphospholipid syndrome Multiple cortical and deep infarcts; venous sinus thrombosis; can coexist with Libman-Sacks in SLE
Atrial myxoma embolism Mobile intracardiac mass visible on echocardiography; multiple embolic cortical infarcts
Non-bacterial thrombotic (marantic) endocarditis Sterile vegetations on echocardiography; multiple embolic cortical infarcts in different territories; indistinguishable from Libman-Sacks on brain MRI
Paradoxical embolism via PFO Single vascular territory cortical infarct; patent foramen ovale on bubble study echocardiography
Cerebral vasculitis (CNS lupus) Multifocal white matter lesions and infarcts; vessel wall enhancement on high-resolution MRI; can coexist in SLE