Endocarditis libman sacksM32.1+I39.8

Libman- Sacks endocarditis was first described in 1924 by Emanuel Libman and Benjamin Sacks in New York in four patients (Ibrahim 2022).

Libman-Sacks endocarditis (LS endocarditis) is an atypical form of endocarditis in which sterile nodular changes occur in the - usually left-sided - heart valves (Erdmann 2006).

E. Libman- Sacks belongs - along with E. rheumatica and- Endomyocarditis eosinophilica - to the group of abacterial endocarditis (Herold 2022).

These are cardiac manifestations of systemic or malignant disease. Typically, sterile vegetations of the heart valves are found, which carry a significant risk for additional infective endocarditis (Ramiandrisoa 2019).

LS endocarditis is one of the rare diseases (Sadeghpour 2021) and occurs in 6-11% of patients with systemic lupus erythematosus, and up to 50% according to autopsy studies (Ibrahim 2022). The risk of cerebral embolism (a complication of E. Libman- sacks) is 33% higher for patients with SLE than in the healthy population (Litmathe 2019).

Together with antiphospholipid syndrome, this shows a prevalence of 33% (Ibrahim 2022).

In the context of malignancies, the prevalence is 1.25% (prevalence of the general population is 0.25%) (Ibrahim 2022).

The disease can occur at any age, but is found clustered between 40 - 80 years of age. There is no gender predilection (Ibrahim 2022).

In children and adolescents, E. Libman- Sacks sometimes manifests as the initial manifestation of systemic lupus erythematosus (Sharma 2012).

LS endocarditis is most commonly caused by systemic lupus erythematosus (SLE) (Herold 2022). There is a significant correlation between the occurrence of LS endocarditis and the severity or duration of SLE (Ibrahim 2022). If antiphospholipid antibodies are also detectable in SLE, there is a 3x higher prevalence of mitral valve vegetations and significant mitral regurgitation compared to healthy individuals (Sharma 2012). According to Yoo (2020), there is a significant prevalence of antiphospholipid antibodies in patients with SLE.

However, SL- endocarditis is also found in association with malignancies, particularly solid tumors such as adenocarcinoma of the pancreas, colon, ovary, lung, biliary tract, and prostate (Ibrahim 2022).

In addition, E. Libman- sacks may occur in the setting of antiphospholipid syndrome (APS) (Ibrahim 2022).

The development of E. Libman- sack is unclear to date. It initially appears to arise from endothelial injury in the context of a hypercoagulable state due to circulating cytokines or interleukins. This results in deposition of platelets, thrombi, and inflammatory molecules (Ibrahim 2022).

Verrucous, mostly left-sided valve abnormalities are found. The ventricular surface of the mitral valve leaflet is particularly commonly affected (Kasper 2015).

Multiple valves may also be affected, even the entire surface of the endocardium (Ramiandrisoa 2019).

Here, larger fibrin droplets, so-called verrucous valve anomalies (Kasper 2015) are most commonly found on the mitral, aortic, and pulmonary valves (Herold 2022).

These lesions may embolize or become infected but rarely cause hemodynamically significant valve regurgitation (Kasper 2015).

E. Libman- sacks is often accompanied by pleurisy and pericarditis (Herold 2022).

The disease is usually asymptomatic. The most common initial presentation is systemic thromboembolism (Sadeghpour 2021).

Patients with SLE commonly present with symptoms of the underlying disease, such as:

• Fever
• rash around the cheekbones
• discoid rash
• Pericarditis
• pleurisy
• arthritis
• diffuse glomerulonephritis
• pancytopenia (Ibrahim 2022)

If Libman's sack endocarditis is suspected, the usual laboratory tests should be performed, as well as hypercoagulation, lupus anticoagulants, determination of antiphospholipid antibodies, and, if necessary, a tumor search (Ibrahim 2022).

At least 3 sets of blood cultures should also be obtained.

A definite diagnosis can only be made by the detection of platelet thrombi (Ibrahim 2022).

Since the risk of infective endocarditis is relatively high, therefore it should be obligatorily clarified at the same time. For this purpose, 3 laboratory data are important:

- Leukocyte count (decreases during systemic lupus activity)

- CRP level (elevation indicates infective endocarditis )

- Blood cultures (Ramiandrisoa 2019)

Transthoracic echocardiography (TTE).

Primarily, TTE should be performed. If this is not informative, TEE is recommended (Ibrahim 2022), as this allows better visualization of mitral and aortic valve vegetations in particular (Roldan 2015).

Transesophageal echocardiography (TEE).

Both sensitivity and specificity of this method are high.

This shows:

- irregular borders

- heterogeneous echo density

- verrucous vegetations on valve(s) and endocardium

- regurgitation of the valves are possible (Ibrahim 2022)

- pericardial effusion (frequent accompanying symptom of active SLE [Flachskampf 2006]) 117

In this case, laboratory signs of inflammation are typically absent, and blood cultures are negative (Sadeghpour 2021).

In addition to the usual laboratory tests, the following should be investigated:

- specific activity signs of systemic lupus erythematosus such as.

- ANA

- Anti- dsDNS- Ak

- Anti- Sm - Ak

- Anti- Ro- Ak (SSA)

- Anti- C1q- Ak

- U1- RNP antibody

- Phospholipid antibodies (see "Systemic lupus erythematosus" for details)

- Antiphospholipid antibodies

- Blood cultures are to be taken several times

- Active verrucae consist of:

- Fibrin clots with focal necrosis

- lymphocytes

- plasma cells

- healed lesions consisting of:

- vascularized dense fibrous and scar tissue (Ibrahim 2022).

- Infective endocarditis (there is a significant risk for this [Ramiandrisoa 2019]).

- Tumors (Sadeghpour 2021)

- rheumatic valve disease

- atrial myxoma

- degenerative valvular disease

- vasculitis

- fibroelastoma (Ibrahim 2022)

- Lambl's excrescence:

This is understood to be filamentous fronds that appear as small thrombi at sites of valve obstruction and may embolize to distant organs (Aziz 2007).

- Cholesterol embolism syndrome (Ibrahim 2022)

The fibrinous lesions of the affected valve may lead to:

- embolism

- infection

- very rarely regurgitation of the valve (Kasper 2015)

- pleurisy

- pericarditis (Herold 2022)

- Stenosis of the affected valve (Ishizu 2019).

To date, there is no specific therapy for LS endocarditis (Steffel 2014).

In a study from 2021, Roldan et al. showed that combined anti-inflammatory and antithrombotic therapy can sometimes avoid the need for high-risk valve surgery (Sadeghpour 2021).

The common practice in the treatment of LS endocarditis is to administer glucocorticoids or immunosuppressive therapy. However, it has not yet been proven whether this treatment leads to an improvement in E. Libman- sacks (Kasper 2015).

- Immunosuppressants such as:

- Azathioprine (Imurek) 2.5 mg / kg bw / d

- or

- Cyclophosphamide (Endoxan) 2 mg / kg bw / d (Unger 2013).

Ishizu (2019) reported a positive outcome with decrease of mitral regurgitation from severe to mild (regurgitation volume 32 ml, effective reflux orifice area 0.17 cm 2) under early therapy with corticosteroids.

Anticoagulation should be considered as secondary prevention in patients with thromboembolic events that have already occurred, such as with warfarin and a target INR between 2 - 3 (Ibrahim 2022).

The underlying disease must also be treated accordingly (Steffel 2014).

The indications for surgery in the form of vegetation removal or valve replacement are the same as those for infective endocarditis (Ibrahim 2022):

- Vegetations > 10 mm (in this case, the risk of embolism increases up to 60%).

- emboli

- persistent valvular vitium

- hemodynamically relevant valvular vitium

- heart failure

- paravalvular abscess

- AV blockages (Herold 2022)

In contrast to infective endocarditis, however, preservation of the valve is possible here with surgical measures (Ibrahim 2022).

Further studies are needed to assess the prognosis. Based on clinical observations, the overall prognosis tends to be poor, with recurrent thromboembolic events, cognitive impairment, and death often occurring. However, further studies are needed for prognostic assessment (Ibrahim 2022).

Patients should be informed of the need for antibiotic prophylaxis for dental procedures, incisions, or other interventions (Ibrahim 2022).

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