Libman-Sacks Endocarditis Symptoms, Treatment, Surgery, Mortality, Causes, and Reasons

Libman-Sacks Endocarditis (LSE)

1. Definition

Libman-Sacks Endocarditis (LSE) is a type of non-infectious endocarditiscaused by the autoimmune disease lupus(Systemic Lupus Erythematosus, SLE). This disease causes non-infectious inflammatory lesions in the endocardium of the heart, primarily affecting the heart valves. LSE is a complication that can occur in lupus patients, especially as part of lupus carditis. This inflammation, resulting from a systemic immune response, creates non-infectious lesions in the endocardium and valves, potentially impacting heart function.

2. Characteristics

A key characteristic differentiating Libman-Sacks endocarditis from other types of endocarditis is its non-infectiousnature. This disease arises from an autoimmune responseand is characterized by the presence of non-infectious lesionson the heart valves. The main characteristics are as follows:

• Inflammation due to autoimmune disease: LSE is primarily associated with lupus(SLE). This inflammatory lesion occurring in lupus patients is caused by an overactive immune response, not an infection, affecting the heart's inner lining.
• Heart valve damage: LSE mainly affects the heart valves, particularly the mitral valveand aortic valve. Scarringor fibrosiscan occur on the valves, potentially impacting heart function.
• Non-infectious nodules: Pathologically, Libman-Sacks nodulesappear, forming non-infectious lesions on the heart valves, endocardium, or heart wall. These nodules are made up of non-infectious inflammatory tissueand affect valve function over time.
• Systemic autoimmune response: LSE occurs as part of a systemic autoimmune disease(SLE), which involves the immune system attacking normal cells, causing inflammation. Lupus can affect various organs including the skin, joints, kidneys, blood vessels, and heart.

3. Cause

Libman-Sacks endocarditis is closely related to **lupus (SLE)**, and the autoimmune responseoccurring in lupus patients is the cause. Lupus is a chronic autoimmune disease where the immune system attacks the body's own normal cells and tissues, leading to the formation of immune complexesand subsequent inflammation in various organs. In lupus patients, LSE results from this autoimmune responseaffecting the endocardium and causing inflammatory lesions on the valves.

• Lupus: Lupus is the most significant cause of Libman-Sacks endocarditis. It is one of the cardiac diseases that frequently occurs in lupus patients, with inflammatory lesions appearing on the heart valves due to the autoimmune response.
• Immune complexes: Immune complexes(antibody-antigen complex) formed in lupus can deposit in the endocardium, causing inflammation. This inflammation is primarily non-infectious, unrelated to infectious agents or bacteria, and is caused by the autoimmune response.
• Genetic factors: Since lupus itself is a disease caused by genetic and environmental factors, genetic predisposition plays a significant role in Libman-Sacks endocarditis.

4. Symptoms

Symptoms of Libman-Sacks endocarditis are mainly related to the symptoms of lupus itself. When the heart is affected, symptoms of impaired heart functionor heart valve diseasemay appear. The main symptoms are as follows:

• Fatigue: This is one of the common symptoms in lupus patients and can also be caused by Libman-Sacks endocarditis. Fatigue is related to the systemic inflammatory response.
• Heart murmur: When inflammation occurs in the heart valves, a heart murmurmay be heard. This indicates dysfunction of the heart valves and can be detected through auscultation.
• Shortness of breath: If the function of the heart valves is impaired, heart failuremay occur, leading to symptoms such as shortness of breath.
• Edema: If heart function is impaired, problems with blood circulation can cause leg edemaor generalized edema.
• Chest pain: Inflammation in the heart valves or endocardium can cause chest pain. This pain is caused by inflammation and can be persistent.
• Fever: Mild fever may occur due to the inflammatory response. This is a common symptom in Libman-Sacks endocarditis.
• Autoimmune symptoms: Since lupus is the cause, symptoms associated with lupus such as skin rash, joint pain, and impaired kidney functionmay also be present.

5. Treatment and Surgical Methods

Treatment for Libman-Sacks endocarditis is mainly related to the management of lupusand aims to alleviate symptoms and protect heart function. Treatment methods are divided into medication and surgery.

5.1. Medication

• Steroid treatment: Since Libman-Sacks endocarditis is caused by inflammation due to an autoimmune response, steroidssuch as Prednisone, can be used to suppress inflammation. Steroids play a crucial role in reducing inflammation and regulating the immune response.
• Immunosuppressants: Immunosuppressantssuch as azathioprine(Azathioprine) and methotrexate(Methotrexate) can be used to suppress the excessive immune response in lupus patients. This helps to inhibit the progression of Libman-Sacks endocarditis.
• Anticoagulants: Since Libman-Sacks endocarditis can cause blood clots on the heart valves, the use of anticoagulants(e.g., warfarin) may be necessary. This helps prevent blood clot formation and is effective in preventing complications such as stroke or pulmonary embolism.
• Other medications: NSAIDs(non-steroidal anti-inflammatory drugs) or antiplatelet agentsmay be used to manage symptoms related to rheumatic diseases.

5.2. Surgical Treatment

• Heart valve surgery: If the heart valves are severely damaged and their function is impaired to the point of causing heart failure, heart valve replacementor valve repairsurgery may be necessary. This surgery plays a crucial role in maintaining heart function.
• Thrombus removal: If a blood clot forms in Libman-Sacks endocarditis, surgery to remove it may be necessary. This is because a blood clot spreading to the brain or lungs can cause fatal complications.

6. Prognosis and Management

The prognosis of Libman-Sacks endocarditis is closely related to the management of lupus. If lupus is well managed, the progression of Libman-Sacks endocarditis can be slowed. However, the prognosis may vary depending on the extent of heart valve damage and heart function impairment.

6.1. Prognosis

• Good prognosis with early detection and treatment: If Libman-Sacks endocarditis is detected early and treated appropriately, heart function can be maintained. The appropriate use of steroids and immunosuppressants can suppress inflammation and prevent valve damage.