In 1924, American cardiologists Emanuel Libman and Benjamin Sacks at New York’s Mount Sinai Hospital first observed a form of endocarditis that was not caused by an infectious agent. They therefore initially referred to it as “atypical verrucous endocarditis.” In total, they observed and followed the disease in four different patients. Over the years, this form of endocarditis was then named after its two discoverers and is now known only as Libman-Sacks endocarditis. It occurs primarily in women 30 years of age and older in the course of systemic lupus erythematosus. Because its diagnosis is quite difficult, complications and, in the worst cases, even valvular or heart failure are relatively common.
What is Libman-Sacks endocarditis?
Libman-Sacks endocarditis, abbreviated LSE, represents a special form of endocarditis, which is inflammation of the endocardium of the heart that is not caused by infection. It usually occurs in the context of chronic systemic diseases, mainly lupus erythematosus, and is characterized by fibrotic changes in the heart valves, primarily the mitral valve and the aortic valve. In the worst cases, the disease can progress to heart failure and death.
Causes
The cause of Libman-Sacks endocarditis is a systemic disease called lupus erythematosus. It is a generalized autoimmune disease that can present with changes in all organs. The disease usually progresses in episodes and it mainly affects women from the age of 30, so that Libman-Sacks endocarditis is also found almost exclusively in the female sex. The main symptoms of systemic lupus are a butterfly erythema on the face and inflammation of the joints (arthritis), but the kidneys, lungs and heart are also frequently affected. Endocarditis occurs when the body produces autoantibodies and immune complexes that attack connective tissue. Triggers for such immune complex formation include UV radiation, viral infection, medications, and stress.
Symptoms, complaints, and signs
Libman-Sacks endocarditis is usually asymptomatic or even entirely asymptomatic. If symptoms occur, they are usually an expression of concomitant disease, such as valvular insufficiency. Large vegetations on the heart valves, especially the mitral and aortic valves, are typical. These are usually fibrotic or consist of proliferating endothelial cells, myocytes and mononuclear inflammatory cells. However, most vegetations are very small and have no hemodynamic significance. Only when they become very large can they also cause problems that are either mechanical in nature or hemodynamic in the form of emboli. The function of the chordae tendineae can be limited by the fact that they show severe thickening. Local inflammatory infiltrates are frequently found. It is not uncommon for pleurisy or pericarditis to occur in addition. Terminal valve insufficiency affects about ten percent of patients. At this stage, pathological heart murmurs can be detected on auscultation and signs of heart failure appear. These include general symptoms such as impaired exercise capacity, weakness and fatigue, but also dyspnea, cardiac arrhythmias and edema. The patient may become cyanotic and the neck veins may become prominent. If lupus erythematosus is known, special attention must be paid to the possible presence of endocarditis.
Diagnosis and course of the disease
The diagnosis of Libman-Sacks endocarditis is often difficult because signs of the disease cannot always be visualized. Therefore, it is first very important to obtain a good and detailed medical history. Here, special attention should be paid to existing previous diseases, which may already be indicative, as well as possible symptoms of a perhaps previously unrecognized lupus erythematosus. Typical laboratory changes that may occur are elevated transaminases, bilirubin, creatinine, and urea. In the course of apparative diagnostics, an ECG, a chest X-ray and an echocardiography should always be performed. Transesophageal echocardiography is clearly superior to transthoracic echocardiography.Furthermore, sonography to determine heart size, cardiac catheterization for cardiac output, and coronary angiography to assess blood flow may also be performed. If Libman-Sacks endocarditis remains undetected for a long time, complications such as pleurisy, pericarditis, valvular regurgitation, heart failure, and emboli may occur. The prognosis depends on several factors. Chief among these are the time of diagnosis, the patient’s age, other chronic diseases, the immune system, and (pre)damage to the heart.
Complications
The symptoms and complications of Libman-Sacks endocarditis vary widely. In many cases, the disease progresses completely without symptoms or with only very mild and minor symptoms, so that no direct treatment of this disease is necessary. However, those affected may suffer from valvular insufficiency and, in the worst case, die from it. It is not uncommon for hemorrhages or severe fever to occur. Furthermore, the patient suffers from reduced exercise tolerance and general weakness. Fatigue also sets in and the affected person no longer takes an active part in life. In addition, disturbances and complaints of the heart can also occur, which not infrequently lead to inner restlessness. The quality of life of the affected person is considerably reduced and limited by Libman-Sacks endocarditis. The patient’s concentration may also be reduced by Libman-Sacks endocarditis. It is not possible to treat Libman-Sacks endocarditis causally. Therefore, treatment of the disease takes place symptomatically, but does not lead to a positive course of the disease in every case. As a rule, patients are dependent on taking medication for the rest of their lives. Whether this results in a reduced life expectancy cannot be generally predicted.
When should you see a doctor?
The timing of when to see a doctor for Libman-Sacks endocarditis is often difficult to determine. In many cases, the disease runs asymptomatic for a long time, so incidental findings occur at this stage of the disease. In principle, the affected person should consult a physician as soon as he or she notices a feeling of illness, a general malaise or a diffuse feeling of physical changes. If changes in the appearance of the skin occur without any apparent reason, a visit to the doctor is advisable. In the case of the formation of poplars, discoloration or itching, a doctor should be consulted. A fatigue, lassitude or apathy are indications of irregularities that should be investigated. Disturbances of the heart rhythm, problems of restful sleep or irregularities of the circulation should be investigated and treated. In many cases, various examinations and tests are needed before a diagnosis of Libman-Sacks endocarditis can be made. The affected person should therefore consult a physician if the existing symptoms are constant or increase. If edema develops, if there is a decrease in performance, or if internal weakness develops, the affected person needs medical attention. If breathing is disturbed or there is shortness of breath, a doctor is needed. If the symptoms persist over a longer period of time, the heart may be severely stressed. A visit to the doctor should be initiated early to avoid serious complications.
Treatment and therapy
Optimal therapy for the treatment of Libman-Sacks endocarditis is not yet known. The primary approach is to treat the underlying disease with immunosuppressive drugs such as cortisone, although their benefit for LSE has not been clearly demonstrated. Surgical interventions are rather discouraged because they are associated with high complication rates. General measures include blood pressure reduction, physical rest, exercise therapy, respiratory exercises, and nicotine abstinence. Medications that are used when heart failure is already present are: Glucocorticoids, diuretics, ACE inhibitors, beta blockers, antiarrhythmics, and anticoagulants.
Outlook and prognosis
The earliest possible diagnosis is important for a good prognosis. Difficulties often arise at this point because the symptoms of the disease often occur in parallel with other diseases, making it difficult to clarify the exact cause.In addition, the symptoms are individually pronounced in patients and therefore often lead to very different results in the assessment of the health condition by the physician. If left untreated and unnoticed, the course of the disease is difficult and the prognosis is unfavorable. Consequential diseases develop, which can lead to the premature death of the affected person. The cardiac activity is considerably restricted, so that in the further course a failure of the organ activity can take place or life-threatening embolisms occur. In addition, a chronic course of the disease may become apparent. Patients who are of advanced age and have pre-existing conditions are more likely to suffer permanent impairment from Libman-Sacks endocarditis. The stress caused by the disease is often difficult for the organism to regulate. In fundamentally healthy individuals, early diagnosis and initiation of medical treatment, the prognosis is greatly improved. Nevertheless, long-term measures to support cardiac activity are necessary and regular checks of cardiac function must be carried out. Otherwise, there is a risk of complications as well as irreparable organ damage.
Prevention
Unfortunately, effective prevention for the occurrence of Libman-Sacks endocarditis does not exist. However, because it is associated with systemic lupus erythematosus, as mentioned above, care should be taken to avoid possible known relapse triggers as much as possible. This includes first and foremost avoiding intense sun exposure, i.e. UV radiation, but also stress or some medications. If SLE already exists, it is important to treat it according to guidelines and try to get it under control.
Follow-up
Libman-Sacks endocarditis is associated with various complications and symptoms, so it should always be treated by a physician. Most patients feel permanently tired and fatigued as a result of this disease, while also being unable to actively participate in daily life. Since the disease is generally
Since the disease is generally not completely curable, aftercare is primarily aimed at limiting the symptoms and improving the quality of life of those affected. At the same time, patients should inform themselves about any complications. Excessive physical exertion and stress should be avoided as far as possible. Because of the relatively high risk of complications, it is advisable to work out all measures in cooperation with a cardiologist. The heart condition also means a psychological burden that can be worked through in therapeutic counseling. Many of those affected suffer from depression or other psychological complaints, which can also have a negative effect on the symptoms of the disease. The further course of this disease depends strongly on the time of diagnosis, so that a general prognosis regarding the effectiveness of follow-up care is usually not possible. It is also possible that Libman-Sacks endocarditis may reduce the life expectancy of the affected person.
Here’s what you can do yourself
No causative treatment is available for Libman-Sacks endocarditis. Medical treatment focuses on relieving symptoms and protecting the patient with a pacemaker and other preventive measures. Self-help measures focus on regular visits to the doctor as well as talking to other affected individuals. In addition, attention must be paid to unusual symptoms and discomfort that may indicate worsening of the heart disease. Patients may engage in moderate exercise. However, all physical activities should be discussed with the responsible physician beforehand. The diet must be changed in collaboration with a nutritionist. Foods that could overload or even damage the heart must be avoided. These include foods with a high salt and sugar content, as well as coffee and alcohol. Relaxation exercises help with palpitations and stress. This can greatly reduce the risk of a heart attack.
