Atypical warty endocarditis (Liebmann-Sachs endocarditis). What is endocarditis The causative agents of infective endocarditis include
Previously, postmortem examination described vegetation similar to bunches of grapes or warts on the ventricular surface of the posterior mitral leaflet, often with adhesion of the mitral leaflet and chordae to the mural endocardium.
Vegetations, as a rule, consist of accumulations of immune complexes and mononuclear cells. The use of steroid therapy for systemic lupus erythematosus significantly changed the spectrum of valvular lesions.
If atypical warty endocarditis of Liebman-Sachs was previously detected in children only during postmortem examination, now, thanks to echocardiography, it is diagnosed in vivo, but relatively rarely (in 13% of patients), as well as its outcome (in 4% of patients) in severe valve insufficiency ...
Liebman-Sachs endocarditis can be combined with primary or secondary antiphospholipid syndrome. However, the role of antiphospho-lipid antibodies in the pathogenesis of endocarditis remains controversial.
In addition to the valve endocardium, the parietal endocardium is also affected in patients with SLE (5%). In recent years, the nature of endocarditis has changed. Against the background of early and active therapy, the manifestations of endocarditis are rarely pronounced and undergo a rapid reverse development against the background of modern complex therapy... According to A.A. Baranova, L.K. Bazhenova (2002), the formation of valvular defects is not typical for SLE in children.
Diagnostics
Clinically, Liebman-Sachs endocarditis is difficult to suspect. Mitral valvulitis can be detected, which does not cause hemodynamic disturbances and does not create conditions for the occurrence of organic noise. Sometimes mitral valvulitis is combined with lesions of the aortic valves or tricuspid valve. In typical cases, auscultation reveals a distinct systolic murmur or a combination of systolic murmur with diastolic murmur. However, a systolic murmur at the apex or at other points occurs in 2/3 of patients with systemic lupus erythematosus and is often associated with muscle insufficiency of the mitral valve or with concomitant fever and anemia.
Transthoracic echocardiography has low sensitivity (63%) and specificity (58%) in the diagnosis of Liebman-Sachs endocarditis compared with transesophageal echocardiography. Three-dimensional echocardiography is highly sensitive in the diagnosis of Liebman-Sachs endocarditis.
The combination of Liebman-Sachs endocarditis and infective endocarditis in children is very rare, but possible at an older age (\u003e 18 years), mainly in women. Authors made following conclusions: 1) with Libman-Sachs endocarditis, IE mask is possible; 2) in about 10% of patients with Liebman-Sachs endocarditis, there is a risk of developing secondary IE; 3) it is advisable to prevent IE with hemodynamic expressed forms endocarditis Liebman-Sachs.
Treatment
Treatment of the underlying disease (SLE) is usually effective and prevents the formation of Liebman-Sachs endocarditis.
Forecast
In children and adolescents, the prognosis of the disease is relatively favorable.
There may be symptoms of deterioration of the state of the myocardium, rarely a pericardial rubbing noise may occur.
It must be borne in mind that myocarditis and pericarditis can occur as a result of systemic lupus erythematosus and without the occurrence of endocarditis. This makes recognizing endocarditis challenging and can often only be detected by a pathologist.
During the diagnosis of endocarditis, correct interpretation of heart murmurs is important. In 2/3 of those examined with a diagnosis of systemic lupus erythematosus, there is a systolic murmur at the apex or at other points, and it can be associated with insufficiency of the mitral valve muscle, and sometimes with anemia and fever. These factors can also cause diastolic murmur, which is much less common in lupus.
It is especially difficult to diagnose in cases of early onset of symptoms of endocardial damage, when the diagnosis of the underlying disease, systemic lupus erythematosus, has not yet been made. Usually in this case, the presence of rheumatism is assumed, rheumatoid arthritis, protracted septic endocarditis, due to the presence of fever and lymphadenopathy.
To recognize systemic lupus erythematosus in the early stages of the development of the disease, it must be remembered that the first symptoms of the disease are often arthralgia, which is often recurrent in the future.
In treatment, the main attention is paid to suppressing the activity of the process with the help of a rational combination of cytostatics and glucocorticoids.
Liebman-Sachs disease
Systemic lupus erythematosus (Liebman-Sachs disease) is a systemic disease with severe autoimmunization, which has an acute or chronic course and is characterized by a predominant lesion of the skin, blood vessels and kidneys.
Systemic lupus erythematosus (SLE) occurs at a rate of 1 in 2500 healthy people... Young women (90%) at the age of 20-30 are sick, but the disease also occurs in children and older women.
Etiology. The cause of SLE is unknown. At the same time, a lot of data have accumulated that indicate a deep sex of the immunocompetent system under the influence of a viral infection (presence of virus-like inclusions in the endothelium, lymphocytes and platelets; persistence of viral infection in the body, determined using antiviral antibodies; frequent presence of measles viruses, parainfluenza , rubella, etc. A contributing factor in the onset of SLE is a hereditary factor.It is known that in patients with SLE antigens HLA-DR2, HLA-DR3 are most often determined, the disease develops in identical twins, in patients and their relatives the function of the immunocompetent system is reduced. development of SLE are a number drugs (hydrazine, D-penicillamine), vaccination for various infections, ultraviolet radiation, pregnancy, etc.
Pathogenesis. It has been proven that in SLE patients there is a sharp decrease in the function of the immunocompetent system, leading to a perversion of its function and the formation of multiple organ autoantibodies. The main sex concerns the processes of regulation of immunological tolerance by reducing T-cell control - Autoantibodies and effector cells are formed to the components of the cell nucleus (DNA, RNA, histones, various nucleoproteins, etc. in total there are more than 30 components). Toxic immune complexes and effector cells circulating in the blood affect the microvasculature, in which hypersensitivity reactions of a non-slow type occur predominantly, and multiple organ damage occurs.
Pathological anatomy. The morphological nature of changes in SLE is very diverse. Fibrinoid changes in the walls of the vessels of the microvasculature prevail; nuclear pathology, manifested by vacuolization of nuclei, karyorrhexis, and the formation of so-called hematoxylin bodies; characterized by interstitial inflammation, vasculitis (microcirculatory bed), polyserositis. Lupus cells (phagocytosis by neutrophilic leukocytes and macrophages of the cell nucleus) and antinuclear, or lupus, factor (antinuclear antibodies) are typical for SLE. All these changes are combined in various relationships in each specific observation, determining the characteristic clinical and morphological picture of the disease.
The skin, kidneys and blood vessels are most severely affected in SLE.
On the skin of the face, a red "butterfly" is noted, which is morphologically represented by proliferative-destructive vasculitis in the dermis, edema of the papillary layer, and focal perivascular lymphohistiocytic infiltration. Immunohistochemically detected deposits of immune complexes in the walls of blood vessels and on the basement membrane of the epithelium. All these changes are regarded as subacute dermatitis.
Lupus glomerulonephritis occurs in the kidneys. The characteristic signs of SLE with it are "wire loops", foci of fibrinoid necrosis, hematoxylin bodies, hyaline thrombi. Morphologically the following types of glomerulonephritis are distinguished: mesangial (mesangioproliferative, mesangiocapillary) focal proliferative, diffuse proliferative, membranous nephropathy. As a result of glomerulonephritis, kidney wrinkling may occur. Currently, kidney damage is the leading cause of death in SLE patients.
Vessels of various calibers undergo significant changes, especially the vessels of the microvasculature - arteriolitis, capillaritis, and venulitis appear. In large vessels, due to a change in vasa vasorum, elastofibrosis and elastolysis develop. Vasculitis causes secondary changes in organs in the form of dystrophy of parenchymal elements, foci of necrosis.
In the heart of some SLE patients, abacterial warty endocarditis (Liebman-Sachs endocarditis) is observed, a characteristic feature of which is the presence of hematoxylin cells in the foci of necrosis.
In the immune system (bone marrow, the lymph nodes, spleen), the phenomena of plasmatization, hyperplasia of lymphoid tissue are found; the development of periarterial "bulbous" sclerosis is characteristic in the spleen.
Complications of SLE are mainly due to lupus nephritis - the development renal failure... Sometimes due to intensive corticosteroid treatment and cytostatic drugs purulent and septic processes, "steroid" tuberculosis may occur.
Aseptic thromboendocarditis (Liebman-Sachs endocarditis)
A characteristic feature of infective endocarditis is the formation of vegetation on the valves or parietal endocardium. Endocarditis usually develops as a result of bacterial colonization of initially sterile vegetation, consisting of platelets and fibrin.
Sterile vegetation (aseptic thromboendocarditis) is formed at the sites of endothelial injury due to foreign body in the cavity of the heart or turbulent blood flow (for example, with deformation of the valves), on scars and in severe non-cardiac diseases (Marantic endocarditis).
There may be symptoms of deterioration of the state of the myocardium, rarely a pericardial rubbing noise may occur.
It must be borne in mind that myocarditis and pericarditis can occur as a result of systemic lupus erythematosus and without the occurrence of endocarditis. This makes recognizing endocarditis challenging and can often only be detected by a pathologist.
During the diagnosis of endocarditis, correct interpretation of heart murmurs is important. In 2/3 of those examined with a diagnosis of systemic lupus erythematosus, there is a systolic murmur at the apex or at other points, and it can be associated with insufficiency of the mitral valve muscle, and sometimes with anemia and fever. These factors can also cause diastolic murmur, which is much less common in lupus.
It is especially difficult to diagnose in cases of early onset of symptoms of endocardial damage, when the diagnosis of the underlying disease, systemic lupus erythematosus, has not yet been made. Usually, in this case, they suggest the presence of rheumatism, rheumatoid arthritis, prolonged septic endocarditis, due to the presence of fever and lymphadenopathy.
To recognize systemic lupus erythematosus in the early stages of the development of the disease, it must be remembered that the first symptoms of the disease are often arthralgia, which is often recurrent in the future.
In treatment, the main attention is paid to suppressing the activity of the process with the help of a rational combination of cytostatics and glucocorticoids.
Liebman-Sachs disease
Systemic lupus erythematosus (Liebman-Sachs disease) is a systemic disease with severe autoimmunization, which has an acute or chronic course and is characterized by a predominant lesion of the skin, blood vessels and kidneys.
Systemic lupus erythematosus (SLE) occurs with a frequency of 1 in 2,500 healthy people. Young women (90%) at the age of 20-30 are sick, but the disease also occurs in children and older women.
Etiology. The cause of SLE is unknown. At the same time, a lot of data have accumulated that indicate a deep sex of the immunocompetent system under the influence of a viral infection (presence of virus-like inclusions in the endothelium, lymphocytes and platelets; persistence of viral infection in the body, determined using antiviral antibodies; frequent presence of measles viruses, parainfluenza , rubella, etc. A contributing factor in the onset of SLE is a hereditary factor.It is known that in patients with SLE antigens HLA-DR2, HLA-DR3 are most often determined, the disease develops in identical twins, in patients and their relatives the function of the immunocompetent system is reduced. development of SLE are a number of drugs (hydrazine, D-penicillamine), vaccination for various infections, ultraviolet radiation, pregnancy, etc.
Pathogenesis. It has been proven that in SLE patients there is a sharp decrease in the function of the immunocompetent system, leading to a perversion of its function and the formation of multiple organ autoantibodies. The main sex concerns the processes of regulation of immunological tolerance by reducing T-cell control - Autoantibodies and effector cells are formed to the components of the cell nucleus (DNA, RNA, histones, various nucleoproteins, etc. in total there are more than 30 components). Toxic immune complexes and effector cells circulating in the blood affect the microvasculature, in which hypersensitivity reactions of a non-slow type occur predominantly, and multiple organ damage occurs.
Pathological anatomy. The morphological nature of changes in SLE is very diverse. Fibrinoid changes in the walls of the vessels of the microvasculature prevail; nuclear pathology, manifested by vacuolization of nuclei, karyorrhexis, and the formation of so-called hematoxylin bodies; characterized by interstitial inflammation, vasculitis (microcirculatory bed), polyserositis. Lupus cells (phagocytosis by neutrophilic leukocytes and macrophages of the cell nucleus) and antinuclear, or lupus, factor (antinuclear antibodies) are typical for SLE. All these changes are combined in various relationships in each specific observation, determining the characteristic clinical and morphological picture of the disease.
The skin, kidneys and blood vessels are most severely affected in SLE.
On the skin of the face, a red "butterfly" is noted, which is morphologically represented by proliferative-destructive vasculitis in the dermis, edema of the papillary layer, and focal perivascular lymphohistiocytic infiltration. Immunohistochemically detected deposits of immune complexes in the walls of blood vessels and on the basement membrane of the epithelium. All these changes are regarded as subacute dermatitis.
Lupus glomerulonephritis occurs in the kidneys. The characteristic signs of SLE with it are "wire loops", foci of fibrinoid necrosis, hematoxylin bodies, hyaline thrombi. Morphologically the following types of glomerulonephritis are distinguished: mesangial (mesangioproliferative, mesangiocapillary) focal proliferative, diffuse proliferative, membranous nephropathy. As a result of glomerulonephritis, kidney wrinkling may occur. Currently, kidney damage is the leading cause of death in SLE patients.
Vessels of various calibers undergo significant changes, especially the vessels of the microvasculature - arteriolitis, capillaritis, and venulitis appear. In large vessels, due to a change in vasa vasorum, elastofibrosis and elastolysis develop. Vasculitis causes secondary changes in organs in the form of dystrophy of parenchymal elements, foci of necrosis.
In the heart of some SLE patients, abacterial warty endocarditis (Liebman-Sachs endocarditis) is observed, a characteristic feature of which is the presence of hematoxylin cells in the foci of necrosis.
In the immunocompetent system (bone marrow, lymph nodes, spleen), the phenomena of plasmatization, hyperplasia of lymphoid tissue are found; the development of periarterial "bulbous" sclerosis is characteristic in the spleen.
Complications of SLE are mainly due to lupus nephritis - the development of renal failure. Sometimes, in connection with intensive treatment with corticosteroids and cytostatic drugs, purulent and septic processes, "steroid" tuberculosis may occur.
Aseptic thromboendocarditis (Liebman-Sachs endocarditis)
A characteristic feature of infective endocarditis is the formation of vegetation on the valves or parietal endocardium. Endocarditis usually develops as a result of bacterial colonization of initially sterile vegetation, consisting of platelets and fibrin.
Sterile vegetation (aseptic thromboendocarditis) is formed at the sites of endothelial injury due to a foreign body in the heart cavity or turbulent blood flow (for example, with deformation of the valves), on scars and in severe non-cardiac diseases (marantic endocarditis).
Liebman-Sachs endocarditis is characterized by endocardial damage with systemic lupus erythematosus and antiphospholipid syndrome. This is a kind of pathology that is most often found in a person after his death. It has several names: lupus, warty, mirantic, thrombotic endocarditis.
General characteristics of the disease
With Liebman-Sachs endocarditis, the mitral and aortic valves of the heart are affected, but in some cases inflammatory processes arise in the remaining valves, as well as the endocardial surfaces of the ventricles. The valves are mainly involved, as a result of which insufficiency and stenosis develops in the valves. Refers to autoimmune pathology.
The disease has been known since the beginning of the last century (1924), named after the people who first described it. It affects the predominantly female half of humanity, which lives in the southern regions of Africa. It is extremely rare in men.
The peculiarity of systemic lupus erythematosus is the wrong reaction immune system for viruses present. Instead of attacking them, immune cells damage their own body. The disease is characterized by multiple lesions, less often - a single one, since bacteria quickly spread throughout the body. With a single lesion, vegetation is observed in the form of islands, which are localized on the parietal endocardium or on the valves. Initially, foci are found only at the edges of the valve cusps, after which they move to two surfaces, and then to the atria and ventricles. It can also be located in the pockets of the ventricles and other parts of the heart.
As the disease progresses, small ulcerations are formed, deformation of the heart departments is not observed. There are also no hemodynamic disturbances, blood flow turbulence and embolic rupture.
In medicine, it is customary to diagnose Liebman-Sachs endocarditis only in cases where there is significant damage to the valves and parietal endocarditis. With minimal growths, the diagnosis is usually not established.
There are two main types of disease:
- Acute endocarditis characterized by thinning of the valve cusps, in which blood vessels absent. There is a diffuse histiolymphocytic infiltration, against the background of which necrosis fibers develop.
- When recurrent endocarditis the valve flaps are thickened. There are capillary neoplasms, destruction of the endothelium over necrosis, and mixed thrombi.
Liebman-Sachs endocarditis can only be detected in two ways - by echocardiography and post-mortem dissection.
Causes of occurrence
Before today the exact causes of Liebman-Sachs endocarditis have not been identified, but there are factors that contribute to this. In general, the disease develops against the background of the penetration of special viruses into the body, which begin to be activated only after the body develops antibodies to the proteins of its own tissues. That is, the body usually produces antibodies against viruses, but in this case, these are antibodies against its own body. It is these specific proteins that provoke inflammatory processes and swelling.
Based on research, a relationship was found at the genetic level. Therefore, this type of endocarditis can be inherited. But there are also such triggering factors:
- frequent stressful situations, emotional experiences, anxieties, fears;
- childbirth and abortion;
- tobacco smoking and abuse alcoholic beverages;
- impact chemical substances;
- the consequences of vaccination of certain species;
- influence medications;
- the presence of adenoviruses in the body;
- epstein-Barr disease;
- exposure to ultraviolet radiation in high doses.
The clinical picture of Liebman-Sachs endocarditis
If we talk about clinical picture endocarditis of lupus erythematosus, it is blurred and practically does not appear. Even if you inspect cardiovascular system, no signs are found. The presence of pathology can only be judged by the following symptoms:
- frequent miscarriages;
- blood clots in arteries and veins;
- skin rashes in the cheekbones;
- the presence of pathologies such as arthritis;
- alopecia;
- increased sweat separation;
- the presence of thrombocytopenia;
- pathological abnormalities in the valve mechanism;
- heart failure.
There is also an indirect symptomatology - protracted illness with the manifestation of fever, changes in auscultatory indicators, the development of diastolic murmurs.
Diagnostics
Lupus endocarditis is difficult to diagnose. Usually, the examination reveals valvulitis in the mitral valves. Due to the absence of hemodynamics, organic noises do not arise. Namely, they could indicate pathological disorders such as lupus erythematosus. If there is auscultation, then a combination of diastolic murmurs with systolic murmurs can be noticed. But these indicators are not special for Liebman-Sachs endocarditis, because the same indicators occur with ordinary lupus erythematosus, fever, muscle insufficiency in the mitral valves and even anemia.
Three main methods are used to identify pathology:
- Transesophageal echocardiography is highly sensitive. The heart is examined using ultrasound equipment. An endoscope equipped with a transducer and ultrasound is inserted into the esophagus. The patient must swallow it, after which all the necessary information is displayed on the monitor. The procedure is not very pleasant, as in many cases it causes a gag reflex. Local or general anesthesia can be used to prevent the patient from experiencing pain. Breathing is additionally monitored, arterial pressure and ripple. The duration of the procedure is 10-15 minutes.
- Echocardiogram in three-dimensional projection is also considered to be the most informative. It is performed in the same way as conventional echocardiography, with the difference that the results are displayed in three-dimensional image.
- Transthoracic echocardiography is considered not very effective, therefore, it is practically not used.
Treatment
Therapy for Liebman-Sachs endocarditis is aimed at suppressing the progression of pathology. For this, cytostatic and glucocorticosteroid drugs are used. The dosage and duration of the course of treatment depends on the severity of the disease, the severity of other pathological disorders and the individual indicators of a particular organism.
Antibacterial joins this therapy. The doctor prescribes specific antibiotics that are used for infective endocarditis. In especially advanced cases, organ prosthetics are performed.
Forecast
Due to the presence of complications such as heart failure, thromboembolism, etc., which are included in the combined incidence of Liebman-Sachs endocarditis, a lethal prognosis is possible in 20-22 cases out of 100. If the disease is detected at early stages of development, the mortality rate decreases.
Unfortunately, the symptoms of Liebman-Sachs endocarditis will not help to detect violations in a timely manner. Therefore, the only thing that experts recommend is a routine heart examination at least once a year. This is the only way to timely identify this dangerous disease.
Lupus endocarditis - endocardial damage in systemic lupus erythematosus - is very peculiar; during his lifetime, he is still rarely diagnosed.
Lupus endocarditis it is more correct to consider only as one of the manifestations, as in rheumatism, and partly in protracted septic endocarditis, although this latter disease has long been considered as an isolated lesion of the endocardium.
Only in 1924, Libman and Sacks described a kind of atypical warty endocarditis, different from rheumatic and septic, as the main manifestation of heart damage in 4 patients who suffered, which was more definitely established later. Later, such a lesion of the endocardium was called Liebman-Sachs endocarditis.
Later, lupus endocarditis was described by Baehr et al. (1931, 1935) and Gross (1932). In 1940, Gross gave a summary analysis of the characteristics of lupus endocarditis. Lupus warty endocarditis is undoubtedly characterized by multiple valvular lesions and widespread lesions of the parietal endocardium. More often the mitral valve is affected, often in combination with aortic, pulmonary, tricuspid; much less often, the lesion is single - in the form of vegetation, spreading islets along the parietal endocardium or isolated on the valves pulmonary artery and tricuspid.
The lesions are located on the free edge of the valves with spread on both surfaces and to the adjacent endocardium of the atria and ventricle. Gross drew attention to the characteristic localization of vegetations that accumulate on the underside of the valve, at the junction with the ventricular endocardium (pocket lesion); further from the valves, the lesion takes on the character of flat plaques.
Lupus endocarditis never leads to disfigurement of the valves, chords, trabeculae; only superficial ulcerations are possible, which do not contribute to the development of sharp hemodynamic disturbances, vortex of blood flow, separation of emboli.
The incidence of endocarditis is very different according to individual authors. This may depend, in part, on the inclusion in the incidence of endocarditis of minimal valve enlargements or even thrombotic overlays, possibly terminal and lacking specificity for the disease. Most of the authors registers as a lesion of the Liebman-Sachs type only significant lesions of the valves, with the presence of parietal endocarditis as a mandatory sign.
Baggenstoss (1952) found typical warty lupus endocarditis (namely, valvulitis with tissue necrosis and exudative reaction, usually stronger than in rheumatism) in 40% of cases, Harvey - in ⅓ sectional cases (mainly mitral valve endocarditis).
Lupus endocarditis rarely gives clear intravital auscultatory and other manifestations, therefore, its reliable diagnosis is possible only on the sectional table. As Armas-Cruz et al (1958) write, such endocarditis actually exists only on the anatomical table rather than in the clinic.
Systolic murmur for recognition of the Liebman-Sachs syndrome has only a relative value, which is obvious from the observations of a number of authors. Harvey of 7 patients with apical systolic murmur only in 4 found endocarditis, and, conversely, of 6 patients with lupus endocarditis, only 2 had systolic murmur. Similar comparisons are given by Griffith and Vural (1951), who out of 7 patients with systolic murmur found mitral endocarditis on the section only in 2, while out of 6 patients with proven endocarditis only 2 had apical systolic murmur during life.
Previously, endocarditis with systemic lupus erythematosus was observed more often; now it develops less frequently, obviously being delayed by modern more active therapy. The distribution and localization of endocarditis also changed in the sense that the defeat of many valves, especially the pulmonary, tricuspid, and aortic, began to be observed less often. Now they often find only the mitral valve lesion and, to some extent, the parietal lesion, which, one might say, somewhat smoothes the difference between lupus and rheumatic endocarditis. However, in advanced and late hormone-treated cases, one can now observe multiple lesions, for example, three valves simultaneously.
Phonocardiogram of a patient with acute systemic lupus erythematosus. Insufficiency of the mitral valve. Lupus endocarditis can be recognized rather by indirect signs: a long-term active disease with high fever and damage to other parts of the heart with a relatively low severity of other visceritis. Of course, sound phenomena, especially changing diastolic noises, retain a certain value. A thorough dynamic clinical and phonocardiographic study with capturing the variability of auscultatory data may help in solving this problem.
Lupus endocarditis only rarely gives rise to bacterial endocarditis type endocarditis lenta... Thus, in all 4 cases of Libman and Sacks, the blood culture was negative. Klemperer et al. (1941) discovered prolonged septic endocarditis in 4 cases, E.M. Tareev. in their early observations - only in 1 case.
Apparently, a small violation of hemodynamics does not contribute to the infection of the affected valves. Patients with systemic lupus erythematosus, moreover, already in early dates diseases, even not yet correctly recognized, usually receive intensive antibiotic treatment due to fever and other manifestations of the disease. This, presumably, prevents the streptococcal infection... Moreover, with modern treatment steroid hormones more reliably prevent the development of endocarditis in general.
Data from E.M. Tareeva confirm the complexity of intravital diagnosis of lupus endocarditis.
So, in an early series of observations, Libman-Sachs endocarditis, E.M. Tareev. and his colleagues observed in 22 out of 50 patients, including damage to the mitral valve during life was established in 10 patients (5 even had signs of stenosis), damage to the mitral valve and aortic valves - in 2 more patients; in the rest, endocarditis was detected only at autopsy. Among the diagnosed lesions of the mitral valve, in 7 cases, autopsy revealed changes in the form of warty endocarditis or focal sclerosis of the mitral and other valves, as well as the parietal endocardium. Among 10 patients in whom lupus endocarditis was not established during life, 6 people had clinical signs, clearly underestimated by clinicians, and 4 patients showed no symptoms of valvular lesion, however, the section showed warty endocarditis of the aortic valves (in one) and focal sclerosis of various valves (in the other three).
Phonocardiogram of a patient with acute systemic lupus erythematosus. Endocarditis of Liebman-Sachs.According to a later series of observations, endocardial damage was recognized in 29 out of 85 patients. In 17 of them, a careful targeted clinical and instrumental examination revealed current (active) endocarditis, predominantly of the mitral valve, with possible formation of its insufficiency in 5 (see figure); for the remaining 12, one could think about eliminating the current process without the formation of a defect (see figure). In 6 patients of this group, an intense, but very dynamic independent murmur was also found, localized on the pulmonary artery, possibly due to the defeat of all valves.
Let us give the following observation as an example.
A 21-year-old patient presented with a hemorrhagic rash on the face, fever, arthralgia, abdominal pain, and increasing weakness. The rash later resembled erysipelas and has spread to the trunk, limbs and oral mucosa with the formation of necrosis. The lungs developed confluent pneumonia, kidney infarction, progressed. There was an increase in the left border of the heart, a systolic murmur at the apex, an accent of the second tone on the pulmonary artery, tachycardia - up to 130 beats per minute, blood pressure 100/50 mm Hg. On the electrocardiogram - the right type, sinus tachycardia, unsharp changes in the myocardium. The patient died 3 months after the onset of the disease.
An autopsy revealed verrucous endocarditis of the mitral, tricuspid and pulmonary artery valves, focal parietal endocarditis of the left ventricle, and moderate sclerosis of the mitral valve.
Sclerosis of the mitral valve and its hyalinosis with areas of fibrinoid swelling were histologically revealed; large round cell infiltrates under the endocardium; gentle perivascular myocardial sclerosis, connective tissue in places with symptoms of fibrinoid swelling, dull swelling of muscle fibers.