Professional Reference articles are designed for health professionals to use. They are written by UK doctors and based on research evidence, UK and European Guidelines. You may find one of our health articles more useful.
Treatment of almost all medical conditions has been affected by the COVID-19 pandemic. NICE has issued rapid update guidelines in relation to many of these. This guidance is changing frequently. Please visit https://www.nice.org.uk/covid-19 to see if there is temporary guidance issued by NICE in relation to the management of this condition, which may vary from the information given below.
Synonyms: verrucous endocarditis
What is Libman-Sacks endocarditis?
Libman-Sacks endocarditis (LSE) is a rare cardiac manifestation of systemic lupus erythematosus (SLE).
Libman-Sacks endocarditis causes sterilised vegetations that mostly affects the mitral valve and possibly the aortic valve. The left heart is much more commonly affected. It may present with symptoms of heart failure due to valvular regurgitation and thromboembolic events.
Libman-Sacks endocarditis is typically situated in the tip, middle, or the base of the posterior mitral leaflets and this can be confused with infective endocarditis vegetation.
Libman-Sacks endocarditis is often missed at echocardiography.
Although the condition is classically associated with SLE, it can also occur in antiphospholipid syndrome (APS).
How common is Libman-Sacks endocarditis? (Epidemiology)
- With a prevalence of between 0.9% and 1.6%, Libman-Sacks endocarditis is an uncommon disease that is typically discovered postmortem.
- One study found that 61% of SLE patients who underwent transoesophageal echocardiography had valvular abnormalities.
- Pericarditis, myocarditis, Libman-Sacks endocarditis, pulmonary arterial hypertension, conduction dysfunction, and coronary artery disease are thought to be present in more than 50% of SLE patients.
- Most patients with Libman-Sacks endocarditis are asymptomatic.
- If valves are severely affected there may be features of the valve disease. Mitral valve disease is more common than aortic valve disease. Regurgitation is more frequent than stenosis and involvement of the tricuspid or pulmonary valves is unusual.
- Systemic embolism may occur with effects depending upon the destination of the emboli but brain and kidney are likely victims. Emboli can cause blockage of the peripheral circulation.
- The vegetations of Libman-Sacks endocarditis are sterile but secondary infective endocarditis can occur.
- There may or may not be typical features of SLE with the characteristic butterfly rash, fever and arthritis or features of APS, including recurrent miscarriage.
- Physical signs reflect the pathology and can be found in the various articles on mitral valve disease or aortic valve disease, infective endocarditis and, if the valve disease is severe, congestive heart failure.
- There may also be left ventricular hypertrophy, causing displacement of the apex beat.
- Echocardiography: not all lesions will be detected. Results with transoesophageal echo are superior but it is an invasive procedure.
- Blood culture is important to exclude infective endocarditis (IE), which may coexist.
- Investigations for SLE, including antiphospholipid antibodies and other autoantibodies. False positive serology in the form of VDRL is common in SLE and anticardiolipin antibodies increase the risk of cardiac abnormalities.
- FBC may show raised neutrophils and some anaemia.
- CXR may show cardiomegaly and pulmonary congestion if disease is severe. Calcification of lesions is uncommon.
- If valvular disease seems severe then cardiac catheterisation may be required with a view to valve replacement.
Libman-Sacks endocarditis treatment and management
- 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.
- Advice for procedures creating risk of infective endocarditis can be found in the separate record Prevention of Endocarditis.
- If there are systemic emboli then anticoagulation is beneficial but the possible role of aspirin has not been adequately investigated.
- If there is evidence of one cerebrovascular event, anticoagulation is advised.
- In serious valve disease it may be necessary to replace valves.
- Systemic emboli may occur but they are probably not very common.
- The risk is much higher with mitral stenosis and subsequent atrial fibrillation.
- When strokes occur it is difficult to know if they were due to systemic emboli or the underlying pathology of SLE or APS.
- Valvular disease can lead to heart failure.
- Maternal SLE with anti-Ro/SS-A (Sjögren's syndrome antigen A) autoantibodies is associated with congenital heart block in the baby in about 1 or 2%. It is usually complete but can be 1st or 2nd degree. The rate of recurrence is around 16%. Fluorinated steroids that do not cross the placenta may be beneficial.
The prognosis will depend on the extent and severity, as well as the other manifestations of SLE.
Further reading and references
Aringer M, Johnson SR; Classifying and diagnosing systemic lupus erythematosus in the 21st century. Rheumatology (Oxford). 2020 Dec 559(Suppl5):v4-v11. doi: 10.1093/rheumatology/keaa379.
Al Riyami H, Joshi N, Al Senaidi K, et al; All Endocarditis Is Not Infective: Libman-Sacks Endocarditis. Cureus. 2022 Jul 314(7):e26526. doi: 10.7759/cureus.26526. eCollection 2022 Jul.
Asher Syed M, Khokhar MU, Akbar F, et al; Libman-Sacks Endocarditis With Triple Valvular Involvement. Cureus. 2023 Apr 1715(4):e37734. doi: 10.7759/cureus.37734. eCollection 2023 Apr.
Aziz F, Baciewicz FA Jr; Lambl's excrescences: review and recommendations. Tex Heart Inst J. 200734(3):366-8.
Costedoat-Chalumeau N, Georgin-Lavialle S, Amoura Z, et al; Anti-SSA/Ro and anti-SSB/La antibody-mediated congenital heart block. Lupus. 200514(9):660-4.