What is lupus-like syndrome? Systemic lupus erythematosus

is a chronic autoimmune disease in which the body's immune system becomes overactive and attacks healthy tissue. Symptoms include inflammation, swelling and damage to joints, skin, kidneys, blood, heart and lungs.

Because of its complex nature, people sometimes refer to lupus as the “disease of 1000 faces.”

In the United States, people report 16,000 new cases of lupus each year, and up to 1.5 million people may be living with the disease, according toLupus Foundation of America.

Lupus affects women in particular, and it most often appears between the ages of 15 and 44. Lupus gained public attention in 2015 after singer Selena Gomez revealed that she developed lupus as a teenager and received treatment for the condition.

Lupus is not an infectious disease. It is not sexually transmitted. However, in in rare cases Women with lupus may give birth to children who develop lupus. This is called neonatal lupus.

Types of Lupus

There are various types lupus This article will focus primarily on systemic lupus erythematosus (SLE), but other types include discoid, drug-induced, and neonatal lupus.

Systemic lupus erythematosus

This is the most familiar type of lupus. SLE can range from mild to severe. SLE can affect any organ or organ system in the body. It can cause inflammation of the skin, joints, lungs, kidneys, blood, heart, or a combination of these. SLE usually goes through certain cycles. During remission, a person's symptoms go away. During an outbreak, the disease is active and symptoms appear.

Discoid lupus erythematosus

Discoid lupus erythematosus (DLE) affects only the skin. A rash appears on the face, neck and scalp. The damaged areas may become scaly and this can lead to scarring. The rash can last from a few days to several years, and it may recur.

Subacute cutaneous lupus erythematosus

Subacute cutaneous lupus erythematosus refers to skin lesions that appear on parts of the body that are exposed to the sun. The lesions do not cause scarring.

Drug-induced lupus

About 10% of people with SLE experience symptoms due to a reaction to certain prescription drugs. According toGenetics Home Reference, about 80 drugs can cause this condition.

These include some of the drugs that people use to treat hypertension. They also include some thyroid medications, antibiotics, antifungal agents and oral contraceptives.

Medicines that are commonly associated with this form of lupus:

• Hydralazine;
• Procainamide;
• Isoniazid.

Drug-induced lupus usually goes away after a person stops taking the drug.

Neonatal lupus

Most babies born to mothers with SLE are healthy. However, about 1% of women with lupus autoantibodies will have a child with neonatal lupus. Sjögren's syndrome is another autoimmune condition that is common with lupus. The main symptoms include dry eyes and dry mouth.

At birth, babies with neonatal lupus may have skin rashes, liver problems, and low blood pressure. About 10% of them suffer from anemia.

The lesions usually resolve within a few weeks. However, some babies have cardiac problems and the baby may need a pacemaker. This can be a life-threatening condition. Women with SLE or other related autoimmune diseases require constant medical supervision during pregnancy.

Causes of lupus

The immune system protects the body and fights antigens such as viruses, bacteria and germs. It does this by producing proteins called antibodies. White blood cells or B lymphocytes produce these antibodies. When a person has an autoimmune condition such as lupus, the immune system cannot distinguish between unwanted substances, antigens, and healthy tissue. As a result, the immune system directs antibodies to both healthy tissue and antigens. This causes swelling, pain and tissue damage. The most common type of autoantibody that develops in people with lupus is antinuclear antibody (ANA). These autoantibodies circulate in the blood, but some of the body's cells have walls that are permeable enough to allow some autoantibodies to pass through.

Autoantibodies can then attack the DNA in the nucleus of these cells. Several genetic factors likely influence the development of SLE.

Some genes in the body help the immune system function. In people with SLE, changes in these genes may interfere with function immune system. One possible theory involves cell death, a natural process that occurs when the body renews its cells, according toGenetics Home Reference.

Some scientists believe that due to genetic factors, the body does not get rid of dead cells. These dead cells that remain can release substances that cause the immune system to malfunction.

Risk factors: hormones, genes and environment

Lupus can develop in response to a number of factors. They may be hormonal, genetic, environmental, or a combination of these.

The U.S. National Institutes of Health notes that women are nine times more likely to develop lupus than men. Age: Symptoms and diagnosis often begin between the ages of 15 and 45. However, 20% of cases appear after age 50, according to Genetics Home Reference.

Since 9 out of 10 cases of lupus affect women, researchers are looking at a possible connection between estrogen and lupus. In a review published in 2016, scientists noted that estrogen can influence immune activity and induce lupus antibodies in mice that are susceptible to lupus. This may explain why autoimmune diseases affect women more often than men.

There is not yet enough evidence that estrogen causes lupus. If there is a link, estrogen-based treatments may regulate the severity of lupus. However, more research is needed before doctors can suggest it as a treatment.

Researchers have not proven that any specific genetic factor causes lupus, although it is more common in some families.

A person who has a first- or second-degree relative with lupus will have a higher risk of developing it.

Scientists have identified genes that may contribute to the development of lupus, but there is not enough evidence that they cause the disease.

Environment

Environmental agents, such as chemicals or viruses, can trigger lupus in people who are already genetically susceptible.

Possible environmental triggers include:

1. Smoking: The increase in incidence in recent decades may be due to higher levels of tobacco exposure.
2. Exposure to sunlight: Some believe this may be a trigger.
3. Drugs: About 10% of cases may be related to drugs, according to Genetics Home Reference.
4. Viral infections: This can cause symptoms in people who are prone to SLE.

Gut microbiota

Recently, scientists have been looking at the gut microbiota as a possible factor in the development of lupus. Scientists publishing research in applied and environmental microbiology in 2018 noted that specific changes in the gut microbiota are common in both humans and mice with lupus.

This requires more research in this area.

Are children at risk?

Lupus is rare in children under 15 years of age. Infants with neonatal lupus may have more high chance development of another autoimmune disease later in life.

Lupus symptoms

Lupus symptoms occur during flares. Between outbreaks, people usually experience periods of remission when there are few or no symptoms.

• fatigue;
• loss of appetite and weight loss;
• pain or swelling in joints and muscles;
• swelling in the legs or around the eyes;
• swelling of the glands and lymph nodes;
• skin rash due to bleeding under the skin;
• mouth ulcers;
• sensitivity to the sun;
• fever;
• headaches;
• chest pain when breathing deeply;
• hair loss;
• Raynaud's syndrome;
• arthritis.

Effect on other body systems

Lupus can also affect the following systems:

Kidneys: kidney inflammation (nephritis). 1 in 3 people with lupus have kidney problems. Lungs: Some people develop pleurisy, which causes chest pain, especially when breathing. Pneumonia may develop.

Central nervous system:Lupus can sometimes affect the brain and central nervous system. Symptoms include headaches, dizziness, depression, memory problems, vision problems, seizures, stroke or behavioral changes.

Vasculitis, or inflammation of the blood vessels, may occur. This may affect blood circulation.

Blood: Lupus can cause anemia, leukopenia (reduced number of white blood cells), or thrombocytopenia (decreased number of platelets in the blood, which help blood clot).

Heart: If inflammation affects the heart, it can lead to myocarditis and endocarditis. It can also affect the lining that surrounds the heart, causing pericarditis. Chest pain or other symptoms may occur. Endocarditis can damage the heart valves.

Other complications

Having lupus increases your risk of a number of health problems:

Infection: Infection becomes more likely because lupus also weakens the immune system. Common infections include urinary tract infections, respiratory infections, yeast infections, salmonella, herpes. Bone osteoporosis: This occurs when the blood supply to the bone is reduced. Eventually, the bone may break down. This most often affects the hip joint.

Pregnancy complications: Women with lupus have a higher risk of pregnancy loss. premature birth and preeclampsia, a condition that involves high blood pressure. To reduce the risk of these complications, doctors often recommend delaying pregnancy until lupus has been under control for at least 6 months.

Treatment and home remedies

There is currently no cure for lupus, but people can manage their symptoms and outbreaks with lifestyle changes and medications.

Goal of treatment:

• prevent or control outbreaks;
• reduce the risk of organ damage.

Medicines can help:

• reduce pain and swelling;
• regulate the activity of the immune system;
• balance hormones;
• reduce or prevent damage to joints and organs;
• manage blood pressure;
• reduce the risk of infection;
• control cholesterol.

The exact treatment will depend on how lupus affects a person. Without treatment, flare-ups can occur, which can have life-threatening consequences.

Alternative and home therapy

In addition to medications, the following may help relieve pain or reduce the risk of a flare-up:

• application of heat and cold;
• participating in relaxation or meditation activities, including yoga and tai chi;
• regular exercise whenever possible;
• avoid exposure to the sun;
• avoid stress.

Forecast

People with a history of lupus usually do not survive more than 5 years. Now, however, treatment can , according to the National Institutes of Health.

Effective therapy can also help manage lupus so a person can live an active, healthy life.

As scientists learn more about genetics, doctors hope they will one day be able to identify lupus more early stage. This will make it easier to prevent complications before they occur.

Scientists believe that .

LUPUS ERYTHEMATOSUS (lupus erythematodes, lupus erythematosus; syn.: erythema centrifugum, erythematosis) - a group concept that includes a number of nosological units, Ch. arr. systemic lupus erythematosus and discoid lupus erythematosus, as well as drug-induced lupus syndrome. Systemic and discoid K. v. have a number of common features. So, both systemic and discoid K. v. affects predominantly women; Both forms are characterized by erythematous rashes on the skin of the face, limbs, trunk and mucous membranes (enanthema), increased sensitivity to solar radiation (photosensitization); transition to discoid K. is possible. systemic (in 3-5% of patients); in some families there may be patients with discoid, systemic K. v. and other collagen diseases. At the same time, differences in the nature of erythematous rashes and especially systemic manifestations in systemic and discoid K. v., features of pathogenesis, in particular deep disturbances of immunogenesis in systemic K. v., allow most authors to consider them as separate nosole forms. This was reflected in the “Statistical Classification of Diseases and Causes of Death” (1969): discoid K. v. belongs to class XII “Diseases of the skin and subcutaneous tissue”, and systemic K. v. - to class XIII “Diseases musculoskeletal system And connective tissue».

Systemic lupus erythematosus

Systemic K.v. ( lupus erythematosus systemicus; syn.: acute lupus erythematosus, erythematous chroniosepsis, Libman-Sachs disease) - a chronic systemic inflammatory disease of connective tissue and blood vessels with pronounced autoimmune pathogenesis and, apparently, viral etiology; refers to diffuse connective tissue diseases - collagenosis (see Collagen diseases). Systemic K. v. is a disease of women of childbearing age (20-30 years), teenage girls often get sick. The ratio of women to men suffering from this disease, according to most statistics, is 8: 1 - 10: 1.

Story

Systemic K.v. described in 1872 by the Viennese dermatologist M. Kaposi as discoid K. v., characterized by fever, pleuropneumonia, rapid development of coma or stupor, and death. In 1923, Libman and Sacks (E. Libman and V. Sacks) described atypical verrucous endocarditis (Libman-Sacks endocarditis), polyserositis, pneumonia and erythematous rashes in the dorsum of the nose and zygomatic arches - the so-called. butterfly Modern doctrine of systemic K. v. associated with the names of Klemperer, Pollack and Baer (P. Klemperer, A.D. Pollack and G. Baehr), who in 1941 drew attention to diffuse collagen disease, describing the systemic damage to connective tissue in this disease and scleroderma. With the discovery of LE cells (Lupus erythematosus cells) by Hargraves, Richmond and Morton (M. M. Hargraves, H. Richmond, R. Morton) in 1948, and in 1949 by Y. R. Ha-serick, the lupus factor was attention is paid to autoimmune disorders.

In the domestic literature, the first wedge, description of “acute lupus erythematosus” belongs to G. I. Meshchersky (1911), and pathomorphology - I. V. Davydovsky (1929) and others. Systematic study of systemic K. v. in our country, started by E. M. Tareev, O. M. Vinogradova and others. In 1965, E. M. Tareev et al., in the monograph “Collagenoses,” after analyzing 150 observations, described systemic K. v. in all its diversity, raised the question of the curability of the disease and outlined ways for further study. Unconditional progress in the development of the doctrine of systemic medicine. due to highly effective treatment with corticosteroids and immunosuppressants.

Statistics

Population studies by Siegel et al. (1962-1965) showed that the incidence in the Manhattan region (New York) increased from 25 to 1 million people. in 1955 to 83 per 1 million in 1964. Dubois (E. L. Dubois, 1974) suggests that in the USA systemic K. century. 5,200 people fall ill every year, therefore, every 5 years a minimum of 25,000 patients accumulate. Leonhardt (T. Leonhardt) in 1955 showed that the prevalence of systemic K. v. in Malmo (Sweden) from 1955 to 1960 there was 29 per 1 million. Mortality in the USA, according to Cobb (1970), is 5.8 per 1 million population, higher among women aged 25-44 year. Mortality, according to materials from the Institute of Rheumatism of the USSR Academy of Medical Sciences, decreased from 90% in 1959-1960. up to 10% by 1975

Etiology

The etiology is not clear, however, the hypothesis about the role of hron, a persistent viral infection, was developed in connection with the discovery by electron microscopy in the affected organs (skin, kidneys, synovium) of tubuloreticular structures located in the cytoplasm of endothelial cells, as well as in lymphocytes and platelets of peripheral blood , which resembled the nucleoprotein of paramyxoviruses. With systemic K. v. Circulating antibodies to measles, rubella, parainfluenza and other RNA viruses from the group of paramyxoviruses were also detected in high titers. Lymphocytotoxic antibodies, which are markers of persistent viral infection, were detected in patients and their relatives, and, in addition, in the same groups and among medical staff working with patients, antibodies to double-stranded (viral) RNA were detected. In connection with the viral etiology of systemic K. v. phenomena such as hybridization of the measles virus genome with the DNA of cells of affected organs (spleen, kidneys), detection of oncornavirus type C antigens in fractions of the spleen, placenta and kidney are discussed. Hypothesis about the significance of hron, viral infection in systemic K. century. is also based on the study of the disease of New Zealand mice, in which the role of oncornavirus type C has been proven.

Intolerance to drugs, vaccines, photosensitivity, the formation of the menstrual cycle, pregnancy, childbirth, abortion, etc. are considered as factors provoking the disease or its exacerbation; they are important for prevention and timely diagnosis, since the connection between the onset or exacerbation of the disease with these factors is more typical for systemic K. v. than for other related diseases.

Pathological anatomy

Systemic K. v., being a representative of the group of collagen diseases, is characterized by a generalized spread of patol, a process that covers all organs and systems, which determines the clinical and anatomical polymorphism of the disease. Generalization is caused by the circulation in the blood of immune complexes that damage the vessels of the microvasculature, resulting in systemic progressive disorganization of connective tissue. Immunopathol. reactions are confirmed by increased function of the organs of immunogenesis, precipitation in the walls of blood vessels and in the affected tissues of immune complexes with the appearance of immunocompetent cells (see). Damage to microcirculation vessels is manifested by common vasculitis of a destructive or proliferative nature (see Vasculitis). In the endothelium of the capillaries, electron microscopy reveals peculiar tubular formations (Fig. 1), similar to the ribonucleoprotein of the paramyxovirus and, possibly, playing an etiol role.

The specificity of tissue reactions during systemic K. v. cause signs of pathology of cell nuclei: fibrinoid basophilia, karyorrhexis, hematoxylin bodies, LE cells, central chromatolysis. Fibrinoid basophilia is caused by the admixture of acidic nuclear decay products. Hematoxylin bodies, described in 1932 by L. Gross, are swollen nuclei of dead cells with lysed chromatin. LE cells, or lupus erythematosus cells, are mature neutrophils, the cytoplasm of which is almost entirely filled with the phagocytosed nucleus of a dead leukocyte. At the same time, the own core is pushed to the periphery. They can be found in the sinuses of lymph nodes, in imprint smears from inflammatory exudate, for example, from pneumonic foci (Fig. 2). Central chromatolysis is manifested by the washing out of chromatin from the center of cell nuclei with clearing of the latter.

Rice. 6. Microscopic specimen of a kidney for lupus glomerulonephritis with characteristic signs of systemic lupus erythematosus: 1 - focal fibrinoid: 2 - “wire loops”; 3 - hyaline thrombi; 4 - karyorrhexis.

The most characteristic changes in systemic K. century. noted in the kidneys, heart, spleen. Kidney damage is characterized by the development of lupus glomerulonephritis, microscopically manifested in two forms: 1) with characteristic signs of systemic K. v.; 2) without characteristic signs of systemic K. v. (V.V. Serov et al., 1974). Characteristic signs include fibrinoid in the glomerular capillaries, the phenomenon of “wire loops”, hyaline thrombi, karyorrhexis (tsvetn. Fig. 6). “Wire loops” are thickened, impregnated with plasma proteins and exposed due to desquamation of the endothelium, the basement membranes of glomerular capillaries, which are considered as a prestage of fibrinoid changes. They were described in 1935 by G. Baehr et al. Hyaline thrombi are located in the lumen of the glomerular capillaries and, based on their tinctorial properties, are regarded as intravascular fibrinoid. The second form is characterized by the development of membranous, membranous-proliferative or fibroplastic changes inherent in banal glomerulonephritis. Both forms are often found in combination.

The development of lupus glomerulonephritis is based on damage to the renal glomeruli by immune complexes. Immunofluorescence microscopy reveals luminescence of immunoglobulins (Fig. 3), complement, and fibrin in the glomeruli. Electron microscopy reveals equivalents of immune complexes in the form of deposits (Fig. 4). When the latter are localized on the subepithelial surface basement membrane There is damage to the processes of podocytes, the formation of spine-like outgrowths of the membrane, which is referred to as membranous transformation. In the clinic, nephrotic syndrome is often noted. The proliferative reaction, according to V.V. Serov et al. (1974), is associated with the proliferation of mesangial cells. As a result of lupus nephritis, secondary shrinkage of the kidneys develops.

Heart damage is characterized by the development of Libman-Sachs endocarditis (Fig. 5). Endocarditis affects the leaflets and chords of the valves, the parietal endocardium, usually does not lead to heart disease, but the development of insufficiency is possible mitral valve. Found in the myocardium fatty degeneration muscle cells (“tiger” heart), less often diffuse proliferative interstitial myocarditis - lupus carditis. The pericardium is most often affected.

The spleen is enlarged, microscopically a characteristic feature is found in it - “bulbous” sclerosis - a layered ring-shaped growth of collagen fibers in the form of a muff around sclerotic arteries and arterioles (Fig. 6). The follicles are atrophied, plasmatization and macrophage reaction are expressed in the red pulp. Plasmatization is also noted in enlarged lymph nodes, bone marrow, thymus gland.

It is possible to develop lupus pneumonitis, which occurs as interstitial pneumonia with vasculitis and cellular infiltration of interstitial tissue. Lung damage may be associated with a secondary infection.

Lupus can affect the liver. In this case, lymphoplasmacytic infiltration and degeneration of hepatocytes are observed in the portal tracts.

Vasculitis is associated with damage to the nervous system.

Visceral lesions are often combined with lesions of the musculoskeletal system and skin. With high disease activity in the skeletal muscles, the picture of acute focal myositis is determined. In the joints, a picture of acute synovitis may develop with a predominance of exudative reactions and usually without subsequent deforming processes.

Microscopic examination of the skin of affected and externally unaffected areas reveals vasculitis, often proliferative, in 70-80% of patients (tsvetn. Fig. 7). Immunofluorescence study reveals the glow of immunoglobulins on the basement membrane in the area of ​​the dermal-epidermal junction (Fig. 7).

Complications and manifestations of the disease leading to the death of patients (renal failure, focal confluent pneumonia, sepsis, anemia, vasculitis leading to cerebral and heart infarctions) have clear morphological signs. For morphol. the picture is left imprinted by corticosteroid therapy, the consequence of which is inhibition of the reaction of immunogenesis organs, adrenal atrophy, osteoporosis, areactive ulcers of gallstones. tract, signs of Itsenko-Cushing syndrome, sometimes an outbreak of tuberculosis, sepsis. Active treatment caused a medicinal pathomorphosis of the disease, characterized by a predominance of chronic forms of the disease over acute ones, an increase in the proportion of proliferative processes, sclerotic changes, a decrease in the frequency of karyorrhexis, hematoxylin bodies, and Libman-Sachs endocarditis.

Morphol, diagnosis of systemic K. v. is based on taking into account nuclear pathology, lupus glomerulonephritis, “bulbous” sclerosis in the spleen, positive immunofluorescence results, vasculitis, connective tissue disorganization, Libman-Sachs endocarditis. For intravital morphology, diagnosis, biopsy material of the kidneys, skin, and skeletal muscles is examined with the mandatory use of immunofluorescent methods.

Pathogenesis

With systemic K. v. the role of disorders of the humoral immunity with the development of organ-nonspecific autoimmune reactions is obvious, which is manifested by hyperfunction of B-lymphocytes and a wide range of circulating autoantibodies (see) - to whole cell nuclei and individual components of the nucleus (DNA, nucleoprotein), as well as lysosomes, mitochondria, cardiolipids ( false-positive Wasserman reaction), blood coagulation factors, leukocytes, platelets, erythrocytes, aggregated gamma globulin (see Rheumatoid factor), etc. These antibodies, being antibodies that witness the damage that has occurred, are capable of forming circulating immune complexes that are deposited on the basal membranes of the kidneys , skin, etc., cause their damage with the development of an inflammatory reaction. This is the immune-complex mechanism for the development of lupus nephritis, vasculitis, etc. The presence of a DNA complex - an antibody to this DNA and complement is proven by the isolation of antibodies to DNA from kidney tissue, and the immune complexes themselves are detected by immunofluorescence (see). High activity of systemic K. v. characterized by hypocomplementemia - a decrease in the content of whole complement (CH50) and its components, especially C3, which takes part in the antigen-antibody reaction, C4, CD1, C9, etc. (see Complement). Many facts have accumulated indicating that there is an imbalance in the humoral and cellular components of immunity; the latter manifests itself various reactions delayed type hypersensitivity, decreased T-lymphocyte content. The presence in certain families of systemic and discoid K. v., various autoimmune diseases, photosensitivity and drug intolerance, the detection of a wide range of circulating autoantibodies in members of these families allows us to think about the role of genetic predisposition in the development of the disease, but the specific mechanisms of this predisposition are not yet known.

Experimental models of systemic K. v. - a disease of New Zealand mice (NZB, NZW and their hybrids NZB/NZW F1) and dogs of special genetic lines (canine lupus) - confirm the above statements, since these models are certainly characterized by a genetic predisposition, an imbalance in humoral and cellular immunity and vertical transmission of oncornavirus C in New Zealand mice.

Clinical picture

The complaints of patients are varied, but most often they complain of pain in the joints, fever, loss of appetite, and sleep. As a rule, systemic K. v. begins subacutely with recurrent polyarthritis, reminiscent of rheumatic, fever, various skin rashes, malaise, weakness, weight loss. Less commonly observed is an acute onset with high fever, sharp pain and swelling of the joints, the “butterfly” symptom, polyserositis, nephritis, etc. In 1/3 of patients 5-10 years or more, one of the monosyndromes is observed - recurrent arthritis, polyserositis, Raynaud's syndrome , Verlhof, epileptiform, but later the disease acquires a relapsing course with the development of a characteristic polysyndromic pattern.

Lupus arthritis observed in almost all patients; it is manifested by migrating arthralgia (see), arthritis (see), transient painful flexion contractures. Mostly small joints of the hands, wrists, ankles, and less commonly are affected. large joints. In 10-15% of patients, fusiform deformation of the fingers and muscle atrophy on the back of the hands may develop. Articular syndrome is usually accompanied by myalgia, myositis, ossalgia and tendovaginitis. When rentgenol, the study reveals epiphyseal osteoporosis, mainly in the joints of the hands and wrists.

Rice. 1. "Butterfly" type of centrifugal erythema.

Rice. 2. “Butterfly” in the form of spots with sharp dense swelling.

Skin damage. The most typical syndrome is “butterfly” - erythematous rashes on the face in the area of ​​the dorsum of the nose (“butterfly body”) and zygomatic arches (“butterfly wings”). According to O. L. Ivanov, V. A. Nasonova (1970), the following variants of erythema are observed: 1) vascular (vasculitic) “butterfly” - unstable, pulsating, diffuse redness with a cyanotic tint in the middle zone of the face, increasing with exposure external factors(insolation, wind, cold, etc.) or excitement; 2) “butterfly” type of centrifugal erythema - persistent erythematous-edematous spots, sometimes with mild follicular hyperkeratosis (erythema centrifugum Biett; color Fig. 1); 3) “butterfly” in the form of bright pink spots with sharp dense swelling against the background of general swelling and redness of the face (erysipelas faciei perstans Kaposi; color Fig. 2); 4) “butterfly”, consisting of discoid-type elements with clear cicatricial atrophy. Erythematous changes are also localized on the earlobes, neck, forehead, scalp, red border of the lips, torso (usually in the upper chest in the form of a décolleté), limbs, and above the affected joints. Some patients experience polymorphic erythema, urticaria, purpura, nodules and other elements.

A peculiar analogue of the “butterfly” of the first and second types are vasculitis (capillaritis) - small erythematous spots with slight swelling, telangiectasia and mild atrophy on the terminal phalanges of the fingers and toes, less often on the palms and soles (color Fig. 3). Various trophic disorders - hair loss, deformation and brittleness of nails, ulcerative skin defects, bedsores, etc. create the characteristic appearance of a patient with systemic K. v.

Damage to mucous membranes manifests itself as enanthema on the hard palate, aphthous stomatitis, thrush, hemorrhages, lupus cheilitis.

Polyserositis- migrating bilateral pleurisy and pericarditis, less commonly peritonitis - is considered an integral component of the diagnostic triad, along with dermatitis and arthritis. The effusion is usually small and its composition resembles rheumatic effusion, but contains LE cells and antinuclear factors. Recurring, polyserositis (see) leads to the development of adhesions up to obliteration of the pericardial cavity, pleura, perisplenitis and perihepatitis. Wedge, manifestations of serositis are usual (pain, friction noise of the pericardium, pleura, peritoneum, etc.), but due to the small amount of exudates and the tendency to quickly disappear, clinicians easily view them, however, with X-ray examination, pleuropericardial adhesions or thickening of the costal bone are often revealed , interlobar, mediastinal pleura.

Lupus carditis very typical for systemic K. century; it is characterized by the simultaneous or sequential development of pericarditis (see), myocarditis (see) or atypical warty Libman-Sachs endocarditis on the mitral and other heart valves, as well as the parietal endocardium and large vessels. Endocarditis ends with marginal sclerosis of the valve, less often with mitral valve insufficiency with characteristic auscultatory symptoms.

Vascular damage with systemic K. century. characteristic of pathol. processes in organs. Nevertheless, it should be noted the possibility of developing Raynaud's syndrome (long before the typical picture of the disease), damage to both small and large arterial and venous trunks (endarteritis, phlebitis).

Lupus pneumonitis- a vascular-connective tissue process in the lungs, in an acute course it proceeds as vasculitis (“vascular pneumonia”), and in other variants of the course - in the form of basal pneumonitis (see) with a normal wedge, a picture of a parenchymal process, but characteristic rentgenol, symptoms ( the reticular structure of the enhanced pulmonary pattern, high position of the diaphragm and basal discoid atelectasis) gives the syndrome a large diagnostic value.

Lupus glomerulonephritis(lupus nephritis) - classic immunocomplex glomerulonephritis (see), observed in half of the patients during the generalization of the process according to the type of urinary syndrome, nephritic and nephrotic. Kidney biopsy followed by gistol and immunomorphol is of great diagnostic importance. research.

Damage to the neuropsychic sphere(neurolupus) - manifests itself at the onset of the disease asthenovegetative syndrome, and at the height of the disease one can observe a variety of symptoms and syndromes from the central and peripheral nervous systems, usually combined - meningoencephalitis, encephalopolyneuritis, encephalomyelitis or meningoencephalomyelitis with polyradiculoneuritis (the latter has diagnostic value).

In the acute form of the disease, affective disorders, delirious-oneiric and delirious types of stupefaction, and patterns of stupor varying in depth can be observed.

Affective disorders manifested by states of anxious depression, as well as manic-euphoric syndromes. Anxious depression is accompanied by pictures of verbal hallucinosis with condemnatory content, fragmentary ideas of attitude and nihilistic delusions (the latter is characterized by instability and lack of tendency to systematize). In manic-euphoric states, there is an elevated mood with a feeling of carelessness, self-satisfaction, and a complete lack of awareness of the disease. At times, some psychomotor agitation is observed, and persistent insomnia is characteristic; during short periods of sleep - vivid dreams, the content of which is often mixed in the patient’s mind with real events.

Delirious-oneiric states excessively volatile; either dream disorders with fantastic or ordinary themes, or abundant colorful, scene-like visual hallucinations come to the fore. Patients feel like observers of ongoing events or victims of violence. Excitement in these cases is of a confused and fussy nature, limited to the boundaries of the bed, and is often replaced by a state of immobility with muscle tension and a loud, monotonously prolonged cry.

Delirious states begin with the appearance of vivid nightmares during the period of falling asleep, followed by multiple, colorful, threatening visual hallucinations, accompanied by verbal hallucinations and a constant feeling of fear.

The intensity of mental disorders correlates with the severity of somatic manifestations, with a high degree of activity of the lupus process.

The described correlations of somatopsychic disorders make it possible to attribute psychoses to systemic K. v. to the group of exogenous organic brain lesions.

It must be borne in mind that with systemic K. v. disturbances in the emotional sphere can also develop in connection with hormonal therapy (steroid psychoses).

Damage to the reticuloendothelial system is expressed in polyadenia (enlargement of all groups of lymph nodes) - a very common and, apparently, early sign of generalization of the lupus process, as well as enlargement of the liver and spleen.

Flow

There are acute, subacute and chronic course of the disease. With an acute onset, patients can indicate the day of development of fever, acute polyarthritis, serositis, “butterfly”, and in the next 3-6 months. One can note pronounced polysyndromic behavior and lupus nephritis or meningoencephalomyelitis with polyradiculoneuritis. Untreated acute systemic K. v. previously led to death 1 to 2 years from the onset of the disease.

With a subacute onset, general asthenic syndromes or recurrent arthralgia, arthritis, and nonspecific skin lesions gradually develop. With each exacerbation in patol, the process involves more and more new organs and systems. A polysyndromic pattern develops, similar to that observed in the acute course of the disease, with a significant incidence of diffuse lupus nephritis and neurolupus.

When chronic, the course of the disease manifests itself for a long time as individual relapses of certain syndromes, and in the 5-10th year of the disease other organ manifestations (pneumonitis, nephritis, etc.) may develop with the development of characteristic polysyndromicity.

Variants of the onset and course of systemic K. century. have age-related patterns. The acute course is usually observed in children and adolescents, menopausal women and the elderly, subacute - mainly in women of childbearing age.

Complications

Among the complications of systemic K. v. the most common is a secondary infection (coccal, tuberculous, fungal, viral), associated with a violation of natural immunity, or with illness, or with inadequate treatment with corticosteroids, the use of immunosuppressants. With the progressive course of systemic K. v. and long-term treatment with corticosteroid drugs, especially in young people, miliary tuberculosis develops, therefore attention to tuberculosis infection with systemic K. v. must be constant for timely recognition and appropriate correction. Shingles (herpes zoster) develops in 10-15% of patients treated for a long time with large doses of corticosteroids and cytotoxic drugs.

Diagnosis

The diagnosis does not present great difficulties in patients with a typical “butterfly” of any type. However, this sign occurs in less than half of patients, and as an early sign - only in 15-20% of patients. Therefore, other symptoms, such as arthritis, nephritis, and their combinations, become of great diagnostic importance. The possibility of intravital biopsy of the joint and kidney makes it possible to more often recognize the lupus nature of arthritis or nephritis. Polysyndromy, detection of LE cells, high titer of antinuclear factors (ANF) or antibodies to native DNA (nDNA) are of diagnostic value. LE cells are found in 70% of patients with systemic K. v. and more. Single LE cells can also be observed in other diseases.

ANF ​​is an IgG directed against the patient's cell nuclei. Usually, to determine ANF, the Immunofluorescence method is used (Fig. 8), in which sections of rat liver, rich in nuclei, are taken as antigenic material, on which the patient’s serum and fluorescein-labeled antiglobulins are layered. For systemic K. century. the most characteristic is peripheral, edge luminescence (Fig. 8.2), due to the presence of antibodies to DNA, and a high titer of this reaction.

Antibodies to DNA are determined by various methods in RIGA (see Hemagglutination), in which sheep red blood cells are loaded with DNA, in the reaction of flocculation of bentonite particles (see Flocculation), also loaded with DNA; In addition, they use the method of radioimmune binding of iodine-labeled nDNA and immunofluorescence, where the culture of Crithidia luciliae is taken as an nDNA substrate.

With hron, polyarthritis and severe liver damage, positive reactions to rheumatoid factor can be detected in the Wohler-Rose reaction (see Rheumatoid arthritis) or latex agglutination (see Agglutination). It is also useful to study complement CH50 and its components, the decrease of which usually correlates with the activity of lupus nephritis. In almost all patients, ROE is significantly accelerated - up to 60-70 mm per hour. More than half of the patients have leukopenia (below 4000 in 1 μl) with a shift in the blood count to promyelocytes, myelocytes and young ones in combination with lymphopenia (5-10% of lymphocytes). Moderate hypochromic anemia is very often observed. In rare cases, hemolytic anemia develops with features of acquired hemolysis (see) and a positive Coombs reaction (see Coombs reaction). Thrombocytopenia (below 100,000 in 1 μl) is often observed, in rare cases - Werlhoff syndrome.

Thus, when establishing a diagnosis of systemic K. v. the whole wedge, the picture, the lab data should be taken into account. research methods and biopsy material of kidneys, synovium and skin.

For a more complete assessment of the patient’s condition, it is advisable to determine the degree of activity of the patol process. Klin, and lab. characterization of the degrees of activity of systemic To. v. is given in Table 1.

Treatment

Treatment started at the onset of the disease gives best effect. In the acute period, treatment is carried out in a hospital, where patients should be provided with adequate nutrition with sufficient amounts of vitamins B and C.

For individualization of treatment, a differentiated determination of the degrees of activity of the patol process is crucial (Table 1).

In case of patol, a process of III degree of activity, all patients, regardless of the course, are indicated for treatment with glucocorticosteroids in large doses (per day 40-60 mg of prednisolone or another drug in equivalent doses), in case of II degree - correspondingly smaller doses (30-40 mg per day). day), and for stage I - 15-20 mg per day. It is extremely important that the initial dose of glucocorticosteroids is sufficient to reliably suppress the activity of the patol process. Particularly large doses (50-60-80 mg per day of prednisolone) should be prescribed for nephrotic syndrome, meningoencephalitis and other diffuse processes in the nervous system - the so-called. lupus crisis. Treatment with glucocorticosteroids in the maximum dose is carried out until a pronounced effect occurs (according to a decrease in clinical and laboratory indicators of activity), and in case of nephrotic syndrome - for at least 2-3 months, then the dose of the hormone is slowly reduced, focusing on the proposed scheme (Table 2), but respecting the principle of individualization in order to prevent withdrawal syndrome or dose reduction syndrome.

Glucocorticosteroids should be prescribed in combination with potassium preparations, vitamins, anabolic hormones and symptomatic agents (diuretics, antihypertensives, ATP, cocarboxylase, etc.). When reducing their dose, salicylates, aminoquinolines and other drugs should be added. Treatment with hormones, as a rule, cannot be completely stopped due to rapidly developing deterioration of the condition (withdrawal syndrome), so it is important that the maintenance dose is minimal. The maintenance dose is usually 5-10 mg of the drug, but may be higher in case of unstable remission.

Such side symptoms that occur during treatment, such as Cushingoid, hirsutism, ecchymosis, stretch marks, acne, develop in many patients and do not require additional therapy. On the contrary, it is noted that persistent improvement in the condition usually occurs with the development of signs of hormone overdose. For persistent edema, diuretics, plasma and albumin transfusions may be recommended. Hypertension is relatively easily controlled with antihypertensive drugs.

Much more serious are complications such as steroid ulcers, exacerbations of focal infections, disorders of mineral metabolism with osteoporosis, etc., but they can also be prevented with systematic monitoring. An undoubted contraindication to continued treatment is steroid psychosis or increased seizures (epilepsy). Correction with psychotropic drugs is necessary.

If glucocorticosteroids are ineffective in patients with systemic K. v. Prescribe treatment with cytostatic immunosuppressants of the alkylating series (cyclophosphamide) or metabolites (azathioprine). Indications for the use of these drugs for systemic K. v. are: high (III) degree of disease activity with involvement of many organs and systems in the process, especially in adolescents; developed lupus nephritis (nephrotic and nephritic syndromes); the need to reduce the suppressive dose of glucocorticosteroid due to developed side effects this therapy.

Azathioprine (imuran) and cyclophosphamide are prescribed in doses of 1-3 mg per 1 kg of patient weight per day in combination with 10-40 mg of prednisolone per day to control extrarenal symptoms. Treatment with immunosuppressants should also be long-term, subject to regular medical supervision. Serious complications may develop during treatment with immunosuppressants, so monitoring of blood (including platelets) and urine is necessary, especially in the first 3 weeks. treatment. With inf. complications are treated with active antibiotic therapy. Other complications, including total alopecia, resolve when the dose of the immunosuppressant is reduced and symptomatic therapy is prescribed.

When hron, the course of systemic K. century. with predominant skin lesions of the discoid type. Recommend chloroquine, delagil or other quinoline drugs.

As signs of damage subside internal organs and a decrease in clinical and laboratory signs of activity to the first degree, treatment can be used. physical education and massage under the control of the general condition and condition of the internal organs. Physiotherapeutic and spa treatment for systemic K. v. not recommended due to the possibility of provoking the disease by ultraviolet irradiation, balneotherapy, and insolation.

Forecast

Prognosis for life with early recognition of systemic K. v. and adequate patol activity, the process of long-term treatment is satisfactory; 70-75% of patients return to active work at work and in the family. However, with the development of lupus nephritis, cerebrovasculitis and the addition of a secondary infection, the prognosis worsens.

Prevention

Prevention is aimed at preventing exacerbations and progression of the disease and the occurrence of the disease.

Prevention of disease progression (secondary) is carried out by timely, adequate, rational complex therapy, therefore patients should undergo regular medical examinations, take hormonal medications in a strictly prescribed dose, do not sunbathe or overcool, avoid surgical interventions, vaccinations, vaccines and serums (except for vital ones). In case of exacerbation of focal or intercurrent infection, bed rest, antibiotics, and desensitizing therapy are required. Treatment of focal infection should be persistent, predominantly conservative.

Primary prevention measures are especially important for family members of patients with systemic K. v., who have signs of photosensitivity, drug intolerance, and impaired humoral immunity. To prevent the disease or generalization of the process, these people should avoid ultraviolet irradiation, treatment with radioisotope gold, spa treatment, etc.

Features of the course of systemic lupus erythematosus in children

Predominantly girls of prepubertal and pubertal age are affected. The rise in incidence begins from the 9th year of life, its peak occurs at 12-14 years. Sometimes systemic K. v. occurs in children 5-7 years old; Cases of illness in children in the first months of life are described as casuistic. There are no cases of congenital disease.

In the vast majority of cases in children and adolescents, systemic K. v. begins and proceeds more acutely and severely, giving a higher mortality rate than in adults. This is due to the peculiarities of the reactivity of the growing organism, the uniqueness of connective tissue structures, organs of immunogenesis, the complement system, etc. Generalization of pathol, the process in children develops much faster, and damage to various organs is characterized by a predominance of the exudative component of inflammation in combination with signs of an intensively developing syndrome of intravascular coagulation disorder in in the form of hemorrhages and bleeding, collaptoid, soporous and shock conditions, thrombocytopenia.

At the onset of the disease, children most often complain of joint pain, weakness, and malaise. Along with this, fever is noted, dystrophy increases quite quickly, often reaching cachexia, significant changes in the blood appear, and signs of damage to many vital organs and systems are revealed.

Skin changes in manifestations typical of lupus are not always found. A combination of acute exudative and discoid changes is characteristic, as well as a tendency to merge individual lesions with the total spread of dermatitis, involving the entire skin and scalp. Hair falls out rapidly, which leads to alopecia areata or complete baldness, and breaks off, forming a kind of brush above the forehead line. The mucous membranes of the mouth, upper respiratory tract, and genitals may be affected. Nonspecific allergic manifestations in the form of urticaria and morbilliform rash or mesh-vascular pattern of the skin, as well as petechial-hemorrhagic elements are much more common and can be found in almost every patient in the active period of systemic K. v.

Articular syndrome, which is the most common and almost always one of the first signs of the disease, can be represented by arthralgias of a volatile nature, acute or subacute arthritis and periarthritis with mild ephemeral exudative manifestations. Articular syndrome is usually combined with damage to the tendon-muscular system, although myalgia and myositis are sometimes an independent sign of systemic K. v.

Involvement in patol, process serous membranes observed in almost all cases; In the clinic, pleurisy and pericarditis are most often recognized, usually in combination with perihepatitis, perisplenitis, and peritonitis. Massive effusion in the pleura and pericardium, requiring repeated punctures, are characteristic manifestations of systemic K. v.

One of the most common visceral signs of systemic K. v. is carditis; its combination with arthritis early stages The disease is almost always mistakenly interpreted as rheumatism. All three membranes of the heart can be affected, but in children and adolescents, symptoms of myocarditis predominate.

Lung lesions are diagnosed less frequently in the clinic than pleural lesions. Typical lupus pneumonitis is accompanied by an alveolar-capillary block, and percussion-auscultatory data are scanty, however, increasing hypoxia, phenomena of respiratory failure attract attention, confirm the presence of pneumonitis and rentgenol data.

Lupus nephritis occurs in children and adolescents more often than in adults (about 2/3 of cases) and in the vast majority of patients it is severe kidney damage with nephrotic syndrome, hematuria, a tendency to arterial hypertension, often accompanied by eclampsia. According to the nature of the course, lupus nephritis in children is close to the mixed form of hron, banal glomerulonephritis, often it is a variant of rapidly progressing glomerulonephritis and only in some patients it occurs in the form of minimal urinary syndrome.

Damage to the central and peripheral nervous system, generally similar to that in adults, includes a chorea-like syndrome with all the wedge, features inherent in minor chorea (see).

Quite often there are signs of damage to the colon. tract. Abdominal pain can be caused by intestinal damage, the development of peritonitis, perisplenitis, perihepatitis, as well as hepatitis and pancreatitis. Before establishing the diagnosis of systemic K. v. abdominal crises can be mistaken for banal acute appendicitis, cholecystitis, ulcerative colitis, dysentery, etc. Sometimes a picture of an acute abdomen develops (see). A symptom complex of malignant ongoing Crohn's disease is possible. The active period of the disease is accompanied by an increase in peripheral lymph nodes, sometimes so significant that for the purpose of differential diagnosis their puncture or biopsy is required.

In 2/3 of sick children and adolescents, systemic K. v. develops acutely or subacutely; There may also be cases of the most acute course of the disease, which is characterized by rapid development of hyperergic reactions, high fever incorrect type and other signs (damage to the skin, joints, lymph nodes), hemorrhagic diathesis, damage to the nervous system. Rapidly progressive vasculitis in short term leads to severe inflammatory-destructive and dystrophic changes in internal organs (heart, kidneys, lungs), with disruption of their functions and possible fatal in the first 3-9 months. from the onset of the disease. Death in such cases most often occurs due to symptoms of cardiopulmonary and (or) renal failure due to intoxication, profound disturbances of homeostasis, coagulopathic disorders, water and electrolyte imbalance, as well as the addition of a secondary infection.

With subacute systemic K. v., moderate in severity and duration, generalization of the process occurs in the first 3-6 months. from the onset of the disease, the course is persistent or wavy with constantly remaining signs of activity and relatively quickly joining function. inferiority of one or another organ.

In approximately 1/3 of children, a variant of the primary chronic course of the disease is observed, close to the picture of classical systemic K. v. adults, with a pre-systemic period lasting from one to 3 years, and with subsequent generalization of the process. Pre-systemic lupus manifestations in children most often include hemopathy, hemorrhagic and nephritic syndromes, arthropathy, and chorea. Other rarer monosyndromes are also possible.

Complications and diagnostic methods are the same as in adults.

Each child with pronounced clinical and laboratory signs of systemic To. v. activity. should be treated in a hospital setting. Corticosteroids and cytostatics are used to suppress immune hyperactivity. The size of their daily dose is determined not only by the age of the child, but also by the degree of activity of the patol process. For grade III activity with symptoms of nephritis, carditis, serositis, neurolupus, large doses of corticosteroids are prescribed (prednisolone at the rate of 1.25-2 mg or more per 1 kg of patient weight per day). If the indicated dose of prednisolone or an equivalent amount of a similar drug cannot be given to the patient, azathioprine or cyclophosphamide must be introduced into therapy at a rate of at least 1 - 3 mg per 1 kg per day. In case of nephrotic syndrome, autoimmune hemolytic anemia, hemorrhagic syndrome and crisis conditions, in all cases, combination immunosuppressive therapy in combination with heparin (250-600 units per 1 kg of body weight per day) is carried out from the very beginning. Upon achieving a clear clinical and laboratory improvement in the patient’s condition, the maximum immunosuppressive dose of prednisolone should be reduced (Table 2), heparin should be replaced with antiplatelet agents (chimes) and (or) indirect anticoagulants.

With a moderate degree of systemic activity. the immunosuppressive dose of corticosteroids should be lower (prednisolone - 0.5-1.2 mg per 1 kg of body weight per day), instead of heparin, chimes are prescribed at 6-8 mg per 1 kg of body weight per day, salicylates, quinoline drugs, methindole are used more widely . With hron, current and low degree of activity of systemic K. v. in the absence of clear symptoms of damage to the kidneys, blood, nervous system, heart, lungs, corticosteroids are prescribed in small doses (prednisolone - less than 0.5 mg per 1 kg of body weight per day) or not used at all.

After discharge from the hospital, children are under the supervision of a rheumatologist and continue to receive supportive immunosuppressive and symptomatic therapy. During the first year after the acute period of systemic K. century. It is not recommended to attend school, but home education can be arranged. It is necessary to cancel all scheduled preventive vaccinations.

With adequate treatment of patients, it is increasingly possible to achieve relative or complete remission. At the same time, the general physical Children's development is progressing more or less satisfactorily, secondary sexual characteristics appear in a timely manner, and girls begin menstruation on time. Mortality is most often associated with renal failure.

Discoid lupus erythematosus

Discoid K. v. (syn.: lupus erythematodes discoides s. chronicus, erythematodes, seborrhea congestiva, erythema atrophicans etc.) is the most common chronic form of K. v., in which the dominant pattern in the disease picture is damage to the skin and mucous membranes. The name “lupus erythematodes” was proposed by P. Cazenave in 1851, believing that the disease was a type of tuberculous lupus. It was first described by R. F. Rayer in 1827 as a rare form of sebaceous discharge (fluxus sebaceus). Discoid K. v. accounts for 0.25-1% of all dermatoses (M.A. Agronik et al.), more often found in countries with cold, humid climates, mainly in middle-aged people [Gertler (W. Gertler)]. Women get sick more often than men.

Etiology

The etiology has not been definitively established. The origin of the disease is assumed to be viral. Electron microscopy reveals tubuloreticular cytoplasmic inclusions in skin lesions.

Pathogenesis

In pathogenesis individual cases diseases are important genetic and immunol factors. In provoking discoid K. v. and its exacerbations, an important role is played by excessive insolation, medications, various types of injuries (mechanical, thermal, chemical).

Pathological anatomy

Discoid K. v. and its disseminated form is limited skin changes. With discoid K. v. the lesion is most often localized on the face. Microscopically (Fig. 9) one finds hyperkeratosis (see), follicular keratosis, vacuolar degeneration of the epidermis (see Vacuolar degeneration), acanthosis (see). Focal lymphoid-macrophage infiltrates with an admixture of neutrophils and plasma cells are visible in the dermis. The walls of blood vessels are impregnated with plasma proteins. The collagen fibers of the dermis are swollen, picrinophilic, and merge into fibrinoid masses. In the infiltration zone, elastic and collagen fibers are destroyed. During treatment, scarring occurs with atrophy and depigmentation of the skin.

For the disseminated cutaneous form of K. v. characterized by multiple rashes throughout the body, in which microscopic changes resemble those with discoid K. v., but are less pronounced, exudative reactions predominate over proliferative ones, cellular infiltration is less significant. The result is no scars or areas of skin atrophy.

Clinical picture

Discoid K. v. begins with the appearance of one or two pink, slightly swollen spots, which gradually increase in size, infiltrate, and become covered in the central zone with tightly packed whitish scales. Scraping the lesions causes pain (Besnier-Meshchersky symptom), because on the underside of the scale there is a horny spine (ladies heel symptom), which is fixed in the expanded mouth of the hair follicle. Subsequently, cicatricial atrophy develops in the central part of the lesion. In a long-existing lesion, three zones are clearly distinguished: a central atrophic zone, then a hyperkeratotic zone and an erythematous zone bordering it (tsvetn. Fig. 4). Within the latter there are often telangiectasia (see). Brown hyperpigmentation may be expressed to varying degrees along the periphery of the lesion. Erythema (see), hyperkeratosis and skin atrophy (see) are the cardinal symptoms of K. v. Infiltration, telangiectasia and pigmentation are common but not obligatory signs.

Rice. 5. Lupus “butterfly” on the face of a patient with discoid skin lesions.

The most typical localization of discoid K. v. is in areas of the skin exposed to insolation: face, ch. arr. its middle part is the noe, cheeks, zygomatic, preauricular areas. As well as for systemic K. v., the so-called butterfly (color fig. 5) - the lesion is on the back of the nose and cheeks. According to I.I. Lelis, who observed 518 patients, the primary foci of K. v. were located on the nose in 48%, on the cheeks in 33%, on the ears or adjacent skin - in 22.5%, on the forehead - in 16.5%, on the scalp - in 10%, on the red border lips, usually the lower - in 12.5%, on the oral mucosa - in 7%. Damage to the mucous membrane of the eyelids L. I. Mashkilleyson et al. observed in 3.4% of patients. More rare, including isolated localizations are known - on the chest, back, shoulders, etc. Lesions of the genital mucosa have been described, bladder, corneas, nail lesions. Along with typical discoid K. v. There are its varieties: hyperkeratotic K. v., with a cut hyperkeratosis is pronounced; papillomatous discoid K. v. - increased proliferation of dermal papillae, leading to the formation of a villous surface of the lesions; warty K. v. - papillomatosis is accompanied by severe keratinization; pigmented K. v. - excessive deposition of pigment, coloring the lesions dark brown; seborrheic K. v. - hair follicles are greatly expanded and filled with fatty, loose scales; tumor-like K. v. - bluish-red, highly elevated foci with edematous, clearly defined edges, mild hyperkeratosis and atrophy.

Rare varieties are telangiectatic discoid K. v. with multiple telangiectasias, hemorrhagic discoid K. v. with hemorrhages in the foci, mutilating. A special form of hron. K.v. is centrifugal erythema (erythema centrifugum Biett). It accounts for 5.2-11% in relation to all forms of K. v., characterized by clearly demarcated foci of erythema on the face, less often on other areas of the skin. They may have telangiectasia and slight swelling. There is no hyperkeratosis. Atrophy is absent or mild. Centrifugal erythema responds fairly quickly to treatment, but recurs easily. Some authors classify it, along with disseminated K. v., as forms intermediate between discoid and systemic.

In the foci of discoid K. v. On the oral mucosa, dark red erythema, telangiectasias, stripe-like, coarse network-like areas of epithelial opacification, erosion, and superficial ulcerations are observed. On the red border of the lips K. v. has the appearance of irregularly oval ribbon-like foci of erythema and hyperkeratosis, sometimes with cracks and erosions. Foci of discoid K. v. more often single, less often multiple. Without treatment, they exist for years and, as a rule, do not cause discomfort. Erosive and ulcerative rashes in the mouth cause pain. They occur especially persistently in smokers. Disseminated discoid K. v. characterized by scattered erythematous-edematous, papular elements or discoid-type lesions. Predominant localization: face, open part of the chest and back, hands, feet, skin over the elbow and knee joints. The general condition of patients with discoid and disseminated K. v., as a rule, does not noticeably suffer. However, during wedge examination, 20-50% of patients reveal arthralgia, functional disorders, disorders of the internal organs (heart, stomach, kidneys), nervous system, accelerated ROE, leukopenia, hypochromic anemia, changes in the composition of immunoglobulins, antinuclear antibodies, immune complexes in the area of ​​the dermoepidermal junction, etc.

Deep K. v. (L. e. profundus Kaposi - Irgang) is characterized by the simultaneous presence of typical skin lesions characteristic of discoid K. v., and nodes in the subcutaneous tissue, the skin over which is mostly unchanged. A number of authors, eg. Pautrier (L. M. Pautrier), consider this form as a combination of deep Darrieus-Russi sarcoids and discoid K. v.

Complications

Occasionally, skin cancer develops, mainly in lesions on the red border of the lower lip, very rarely - sarcoma, erysipelas; a serious complication, more often observed with disseminated discoid K. v., is its transition to systemic K. v. under the influence of unfavorable factors.

Diagnosis

The diagnosis in typical cases is established without difficulty. Foci of discoid K. v. may be similar to seborrheic eczema, rosacea, psoriasis, eosinophilic granuloma of the face, tuberculous lupus. Clear boundaries of the lesions, horny plugs in dilated hair funnels, tightly fitting scales, a positive Besnier-Meshchersky sign, and the development of atrophy indicate the presence of K. v. Foci of seborrheic eczema (see) do not have such sharp boundaries, their surface is covered with loose, fatty scales, they respond well to antiseborrheic therapy. Psoriatic lesions are usually numerous, covered with easily scraped off silvery scales (see Psoriasis). Both of them, in contrast to K. v. usually decrease under the influence of sunlight. With rosacea (see) there is diffuse erythema, telangiectasia is pronounced, nodules and pustules often appear. Eosinophilic granuloma of the face (see) is characterized by particular resistance to therapeutic effects. Its foci are often single, uniform brown-red color, without hyperkeratosis, with isolated telangiectasias. Tuberculous lupus (see Tuberculosis of the skin) usually begins in childhood, it is characterized by the presence of lupoma with the characteristic apple jelly and probe phenomena. In cases of erythematous tuberculous lupus erythematosus of Leloir, the wedge, the diagnosis is extremely difficult, a histol examination is necessary. Discoid K. v. should also be differentiated with Essner-Kanoff lymphocytic infiltration; manifestations of the cut are less persistent, tend to resolve in the center, lack of peeling, hyperkeratosis, and atrophy. K.v. on the scalp differentiated from pseudopelade (see). The latter is characterized by the absence of inflammation, horny spines, finger-like arrangement, and more superficial atrophy. Discoid K. v. on the oral mucosa should be distinguished from lichen planus, the rashes of which have a more delicate pattern and are not accompanied by atrophy.

Patients with discoid K. v., including limited forms, should be examined to exclude systemic damage internal organs and nervous system, as well as identifying concomitant diseases.

Treatment

Leading role in the treatment of discoid and disseminated K. v. belongs to aminoquinoline drugs - chloroquine, resokhin, delagil y, plaquenil y, etc. They are prescribed continuously or in cycles, usually 0.25 g 2 times, Plaquenil - 0.2 g 3 times a day after meals. The duration of cycles (5-10 days) and the intervals between them (2-5 days) depends on the tolerability of treatment. Repeated courses of treatment are recommended, especially in spring. Adding small doses of corticosteroids (2-3 tablets of prednisolone per day) to chloroquine improves treatment results and tolerability. This technique is recommended for particularly persistent cases of K. v., extensive damage skin.

It is useful to include vitamins B6, B12, calcium pantothenate, and nicotinic acid in the therapeutic complex. Treatment the effect occurs faster with the simultaneous administration of ointments with fluoride-containing corticosteroids (sinalar, flucinar, etc.), which, in case of limited lesions, can be the main method of therapy. It is also recommended to administer intradermally into the affected areas 5% chloroquine solution once every 5-7 days (4-6 injections per course). Limited lesions with a strong infiltrate and hyperkeratosis without signs of peripheral growth can be subjected to cryotherapy.

Forecast

The prognosis for life is favorable. With adequate treatment and patients following the recommended regimen, their ability to work remains for many years.

Prevention

Patients K. v. subject to medical examination. They must comply with the gig. mode of work, rest, nutrition, avoid physical activity. and nervous overload, exposure to the sun, wind, frost, use photoprotective creams and films with para-aminobenzoic acid, tannin, etc. It is necessary to sanitize foci of focal infection. For the treatment of concomitant diseases of patients with K. v. should not be directed to the south. resorts in the spring and summer, they should be prescribed physiotherapeutic procedures with caution, and vaccinated only for serious indications.

Drug-induced lupus erythematosus

Medicinal K. v. develops in connection with long-term use of apressin (hydralazine), procainamide (procainamide), diphenine (hydantoin), trimethine (trimethadione), carbazepine, isoniazid and chlorpromazine. Medicinal K. v. can develop in elderly people suffering from hypertension and arrhythmia, in patients with tuberculosis and epilepsy. The listed drugs are capable of causing the formation of antinuclear antibodies (ANF, antibodies to DNA), the appearance of which precedes the clinical manifestations of medicinal K. v., reminiscent of systemic K. v. When taking certain medications, a certain wedge or syndrome occurs. So, with apressin K. v. glomerulonephritis develops; with long-term use of nicotinamide, pleurisy and pneumonitis are very common, which are the beginning of the syndrome.

Among the mechanisms of development of medicinal To. v. The role of predisposition is discussed, since such a reaction occurs in approximately 10% of patients taking apressin and other drugs, as well as metabolic disorders, in particular the rate of acetylation of these drugs.

The diagnosis is made based on taking the listed medications.

Timely recognition of the disease and discontinuation of the drug that caused the medicinal K. v. leads to recovery, however, it may be necessary to prescribe corticosteroids in medium doses (20-30 mg of prednisolone per day), especially with isoniazid medicinal K. v. With the development of the clinic of systemic K. century. appropriate therapeutic tactics are necessary.

Tables

Table 1. Clinical and laboratory indicators of the degree of activity of systemic lupus erythematosus

Indicators	Level of activity of systemic lupus erythematosus
		(moderate severity)	(heavy)
Clinical signs
Temperature	Normal		38° or more
Weight loss	Absent	Moderate	Expressed
Trophic disturbance	May be missing	Moderate	Expressed
Skin lesions	Discoid lesions	Exudative	“Butterfly” and lupus-type erythema
Polyarthritis	Deforming, arthralgia	Subacute	Acute, subacute
Pericarditis	Adhesive		Vypotnoy
Myocarditis	Cardiosclerosis, myocardial dystrophy	Focal	Polyfocal, diffuse
Endocarditis	Mitral valve insufficiency	Damage to one (usually mitral) valve	Multiple valve damage
	Adhesive		Vypotnoy
Pneumonitis	Pneumofibrosis	Chronic (intermediate)	Acute (vasculitis)
	Chronic glomerulonephritis	Nephritic or urinary syndrome	Nephrotic syndrome
Nervous system	Polyneuritis	Encephaloneuritis	Acute encephalomyeloradiculoneuritis
Laboratory indicators
Hemoglobin (g%)	12 or more
ROE (mm per hour)			45 or more
Fibrinogen (g%)
Total protein (g%)
Albumins (%) Globulins (%):
LE cells (per 1 thousand leukocytes)	Single or absent
Antinuclear factor (in credits)			1:128 and higher
Glow type	Homogeneous	Homogeneous and edge
Antibodies to nDNA (in credits)

Table 2. Approximate diagram reducing the dose of prednisolone depending on the initial (maximum) dose

Initial (maximum) dose of prednisolone, mg per day	Reducing the dose of prednisolone by week, mg per day

Bibliography: Vinogradova O. M. Systemic lupus erythematosus in the clinic of internal diseases, Sov. med., No. 4, p. 15, 1958; Guseva L. L. and Luninskaya I. R. Psychopathological manifestations in systemic lupus erythematosus, Zhurn, neuropath, and psychiat., t. 75, century. 4, p. 562, 1975, bibliogr.; Davydovsky I.V. On the issue of lupus erythematodes disseminatus acutus, Rus. Vestn. derm., vol. 7, no. 5, p. 450, 1929, bibliogr.; I Smailov T. I. and F r u m k i-n a S. L. On psychopathology and pathogenesis symptomatic psychosis with systemic lupus erythematosus, Zhurn, neuropath, and psychiat., t. 72, no. 12, p. 1860, 1972; L e l and with I. I. Lupus erythematosus, L., 1970, bibliogr.; Meshchersky G.I. and Grinchar F.N. About the case of erythema faciei perstans (Kaposi - Kreibich’a) of tuberculous origin, Kharkov. pathologist Sat., dedicated. prof. M. N. Nikiforov, on the 25th anniversary of his scientist, activity, p. 406, M., 1911; Nasonova V. A. Systemic lupus erythematosus, M., 1972, bibliogr.; S e r about in V.V. and others. Immunomorphological characteristics of skin changes in lupus erythematosus, Soz. med., no. 9, p. 15, 1972; S e r about in V.V. and others. Electron microscopic characteristics of lupus nephritis, Arch. pathol., t. 36, no. 6, p. 21, 1974, bibliogr.; S k r i p k i n Yu. K., Somov B. A. and B u t o v Yu. S. Allergic dermatoses, With. 130, M., 1975, bibliogr.; With t r u-k about in A. I. and B e g l a r I n A. G. Pathological anatomy and pathogenesis of collagen diseases, p. 248, M., 1963; Tare-e in E. M. Collagenoses, M., 1965, bibliogr.; Tareeva I.E. Lupus nephritis, M., 1976, bibliogr.; Tareeva I. E., Serov V. V. and Kupriyanova L. A. Intraendothelial inclusions in systemic lupus erythematosus, Bull. Experiment, biol, and med., v. 77, no. 5, p. 119, 1974; O' C o n n o r J. F. a. Musher D. M. Central nervous system involvement in systemic lupus erythematosus, Arch. Neurol. (Chic.), v. 14, p. 157, 1966; Hargraves M. M., Richmond H. a. M o r t o n R. Presentation of two bone marrow elements, the “tart” cell and the “L. E." cell, Proc. Mayo Clin., v. 23, p. 25, 1948; Klemperer P., Pollack A. D. a. Baehr G. Pathology of disseminated lupus erythematosus, Arch. Path., v. 32, p. 569, 1941; Lupus erythematosus, ed. by E. L. Dubois, Los Angeles, 1974; Recent advances in rheumatology, ed. by W. W. Buchanan a. W. C. Dick, pt 1, Edinburgh -L., 1976; Ropes M. W. Systemic lupus erythematosus, Cambridge - L., 1976, bibliogr.

V. A. Nasonova; L. A. Isaeva (ped.), A. I. Strukov, L. V. Kaktursky (pat. an.), A. S. Tiganov (psychiat.), L. Ya. Trofimova (derm.).

This disease is accompanied by disruption of the immune system, resulting in inflammation of muscles, other tissues and organs. Lupus erythematosus occurs with periods of remission and exacerbation, and the development of the disease is difficult to predict; As the disease progresses and new symptoms appear, the disease leads to the formation of failure of one or more organs.

What is lupus erythematosus

This autoimmune pathology, which affects the kidneys, blood vessels, connective tissues and other organs and systems. If, under normal conditions, the human body produces antibodies that can attack foreign organisms entering from the outside, then in the presence of a disease, the body produces a large number of antibodies to the body’s cells and their components. As a result, an immune complex inflammatory process is formed, the development of which leads to dysfunction of various elements of the body. Systemic lupus affects internal and external organs, including:

• lungs;
• kidneys;
• skin;
• heart;
• joints;
• nervous system.

Reasons

The etiology of systemic lupus still remains unclear. Doctors suggest that the cause of the disease is viruses (RNA, etc.). In addition, a risk factor for the development of pathology is a hereditary predisposition to it. Women suffer from lupus erythematosus about 10 times more often than men, which is explained by the characteristics of their hormonal system (there is a high concentration of estrogen in the blood). The reason why the disease manifests itself less frequently in men is protective effect, which is exerted by androgens (male sex hormones). The following may increase the risk of SLE:

• bacterial infection;
• taking medications;
• viral infection.

Development mechanism

A normally functioning immune system produces substances to fight antigens of any infections. In systemic lupus, antibodies deliberately destroy the body's own cells, and they cause absolute disorganization of connective tissue. Typically, patients exhibit fibroid changes, but other cells are susceptible to mucoid swelling. In the affected structural units of the skin, the core is destroyed.

In addition to damage to skin cells, plasma and lymphoid particles, histiocytes, and neutrophils begin to accumulate in the walls of blood vessels. Immune cells settle around the destroyed nucleus, which is called the “rosette” phenomenon. Under the influence of aggressive complexes of antigens and antibodies, lysosome enzymes are released, which stimulate inflammation and lead to damage to connective tissue. New antigens with antibodies (autoantibodies) are formed from destruction products. As a result chronic inflammation sclerosis of the tissue occurs.

Forms of the disease

Depending on the severity of the symptoms of the pathology, the systemic disease has a certain classification. Clinical types of systemic lupus erythematosus include:

1. Acute form. At this stage, the disease progresses sharply, and the patient’s general condition worsens, while he complains of constant fatigue, high temperature (up to 40 degrees), pain, fever and muscle aches. The symptoms of the disease develop quickly, and within a month it affects all human tissues and organs. The prognosis for the acute form of SLE is not comforting: often the life expectancy of a patient with such a diagnosis does not exceed 2 years.
2. Subacute form. It can take more than a year from the onset of the disease to the onset of symptoms. This type of disease is characterized by frequent alternation of periods of exacerbation and remission. The prognosis is favorable, and the patient’s condition depends on the treatment chosen by the doctor.
3. Chronic. The disease is sluggish, the symptoms are mild, the internal organs are practically undamaged, so the body functions normally. Despite the mild course of the pathology, it is virtually impossible to cure it at this stage. The only thing that can be done is to alleviate a person’s condition with the help of medications during an exacerbation of SLE.

It is necessary to distinguish between skin diseases related to lupus erythematosus, but which are not systemic and do not have generalized lesions. Such pathologies include:

• discoid lupus (a red rash on the face, head, or other parts of the body that is slightly raised above the skin);
• drug-induced lupus (inflammation of the joints, rash, high fever, pain in the sternum associated with taking medications; symptoms go away after they are discontinued);
• neonatal lupus (rarely expressed, affects newborns when mothers have diseases of the immune system; the disease is accompanied by liver abnormalities, skin rashes, and heart pathologies).

How does lupus manifest?

The main symptoms of SLE include severe fatigue, skin rash, and joint pain. As the pathology progresses, problems with the functioning of the heart, nervous system, kidneys, lungs, and blood vessels become relevant. The clinical picture of the disease in each specific case is individual, since it depends on which organs are affected and what degree of damage they have.

On the skin

Tissue damage appears at the onset of the disease in approximately a quarter of patients; in 60-70% of patients with SLE, the skin syndrome is noticeable later, and in the rest it does not occur at all. As a rule, the localization of the lesion is characterized by areas of the body exposed to the sun - the face (butterfly-shaped area: nose, cheeks), shoulders, neck. The lesions are similar to erythematosus in that they appear as red, scaly plaques. At the edges of the rash there are dilated capillaries and areas with excess/lack of pigment.

In addition to the face and other areas of the body exposed to sunlight, systemic lupus affects hairy part heads. As a rule, this manifestation is localized in the temporal region, with hair falling out in a limited area of ​​the head (local alopecia). Increased sensitivity to sunlight (photosensitization) is noticeable in 30-60% of SLE patients.

In the kidneys

Very often, lupus erythematosus affects the kidneys: in about half of patients, damage to the renal apparatus is determined. A common symptom of this is the presence of protein in the urine; casts and red blood cells are usually not detected at the onset of the disease. The main signs that SLE has affected the kidneys are:

• membranous nephritis;
• proliferative glomerulonephritis.

In the joints

Rheumatoid arthritis is often diagnosed with lupus: in 9 out of 10 cases it is non-deforming and non-erosive. More often the disease affects the knee joints, fingers, and wrists. In addition, patients with SLE sometimes develop osteoporosis (low bone density). Patients often complain of muscle pain and muscle weakness. Immune inflammation is treated with hormonal medications (corticosteroids).

On mucous membranes

The disease manifests itself on the mucous membrane oral cavity and nasopharynx in the form of ulcers that do not cause pain. Damage to the mucous membranes is recorded in 1 out of 4 cases. This is typical for:

• decreased pigmentation, red border of the lips (cheilitis);
• ulcerations of the oral cavity/nasal cavity, pinpoint hemorrhages.

On vessels

Lupus erythematosus can affect all structures of the heart, including the endocardium, pericardium and myocardium, coronary vessels, and valves. However, damage to the outer lining of the organ occurs more often. Diseases that can result from SLE:

• pericarditis (inflammation of the serous membranes of the heart muscle, manifested by dull pain in the chest area);
• myocarditis (inflammation of the heart muscle, accompanied by rhythm disturbances, conduction nerve impulse, acute/chronic organ failure);
• heart valve dysfunction;
• damage to the coronary vessels (can also develop in early age in patients with SLE);
• damage to the inside of blood vessels (this increases the risk of developing atherosclerosis);
• damage to the lymphatic vessels (manifested by thrombosis of the extremities and internal organs, panniculitis - painful subcutaneous nodes, livedo reticularis - blue spots forming a mesh pattern).

On the nervous system

Doctors suggest that the failure of the central nervous system is caused by damage to the blood vessels of the brain and the formation of antibodies to neurons - cells that are responsible for nourishing and protecting the organ, as well as to immune cells (lymphocytes. Key signs that the disease has affected the nervous structures of the brain are :

• psychosis, paranoia, hallucinations;
• migraine, headaches;
• Parkinson's disease, chorea;
• depression, irritability;
• brain stroke;
• polyneuritis, mononeuritis, aseptic meningitis;
• encephalopathy;
• neuropathy, myelopathy, etc.

Symptoms

The systemic disease has an extensive list of symptoms, and is characterized by periods of remission and complications. The onset of pathology can be immediate or gradual. Signs of lupus depend on the form of the disease, and since it belongs to the multiorgan category of pathologies, clinical symptoms can be varied. Mild forms of SLE are limited only to damage to the skin or joints; more severe types of the disease are accompanied by other manifestations. TO characteristic symptoms illnesses include:

• swollen eyes, joints of the lower extremities;
• muscle/joint pain;
• enlarged lymph nodes;
• hyperemia;
• increased fatigue, weakness;
• red, allergic-like rashes on the face;
• causeless fever;
• blueness of fingers, hands, feet after stress, contact with cold;
• alopecia;
• pain when inhaling (indicates damage to the lining of the lungs);
• sensitivity to sunlight.

First signs

Early symptoms include temperature, which fluctuates between 38,039 degrees and can last for several months. After this, the patient develops other signs of SLE, including:

• arthrosis of small/large joints (may go away on its own, and then reappear with greater intensity);
• a butterfly-shaped rash on the face, rashes also appear on the shoulders and chest;
• inflammation of the cervical and axillary lymph nodes;
• In case of severe damage to the body, internal organs - kidneys, liver, heart - suffer, which results in disruption of their functioning.

In children

At an early age, lupus erythematosus manifests itself with numerous symptoms, progressively affecting different organs of the child. At the same time, doctors cannot predict which system will fail next. Primary signs of pathology may resemble common allergies or dermatitis; This pathogenesis of the disease causes difficulties in diagnosis. Symptoms of SLE children may have:

• dystrophy;
• thinning of the skin, photosensitivity;
• fever accompanied by profuse sweating and chills;
• allergic rashes;
• dermatitis, as a rule, is first localized on the cheeks, bridge of the nose (looks like warty rashes, blisters, swelling, etc.);
• joint pain;
• brittle nails;
• necrosis on the fingertips, palms;
• alopecia, up to complete baldness;
• convulsions;
• mental disorders(nervousness, moodiness, etc.);
• stomatitis that cannot be treated.

Diagnostics

To make a diagnosis, doctors use a system developed by American rheumatologists. To confirm that a patient has lupus erythematosus, the patient must have at least 4 of the 11 listed symptoms:

• erythema on the face in the shape of butterfly wings;
• photosensitivity (pigmentation on the face that worsens when exposed to sunlight or UV radiation);
• discoid rash on the skin (asymmetrical red plaques that peel and crack, with areas of hyperkeratosis having jagged edges);
• symptoms of arthritis;
• formation of ulcers on the mucous membranes of the mouth and nose;
• disturbances in the functioning of the central nervous system - psychosis, irritability, tantrums for no reason, neurological pathologies, etc.;
• serous inflammation;
• frequent pyelonephritis, the appearance of protein in the urine, the development of renal failure;
• false positive reaction of the Wasserman test, detection of titers of antigens and antibodies in the blood;
• reduction of platelets and lymphocytes in the blood, changes in its composition;
• causeless increase in antinuclear antibody levels.

The specialist makes a final diagnosis only if four or more signs from the list are present. When the verdict is in doubt, the patient is referred for a highly focused, detailed examination. Big role When diagnosing SLE, the doctor takes a history and study of genetic factors. The doctor must find out what diseases the patient had during the last year of life and how they were treated.

Treatment

SLE is a chronic disease in which complete cure of the patient is impossible. The goals of therapy are to reduce activity pathological process, restoration and preservation of the functionality of the affected system/organs, prevention of exacerbations to achieve longer life expectancy for patients and improve its quality of life. Treatment of lupus involves the mandatory use of medications, which are prescribed by the doctor to each patient individually, depending on the characteristics of the body and the stage of the disease.

Patients are hospitalized in cases where they have one or more of the following clinical manifestations of the disease:

• suspicion of stroke, heart attack, severe damage to the central nervous system, pneumonia;
• an increase in temperature above 38 degrees for a long time (fever cannot be eliminated with the help of antipyretics);
• depression of consciousness;
• a sharp reduction in leukocytes in the blood;
• rapid progression of disease symptoms.

If the need arises, the patient is referred to specialists such as a cardiologist, nephrologist or pulmonologist. Standard treatment for SLE includes:

• hormonal therapy (glucocorticoid drugs are prescribed, for example, Prednisolone, Cyclophosphamide, etc.);
• anti-inflammatory medications (usually Diclofenac in ampoules);
• antipyretics (based on Paracetamol or Ibuprofen).

To relieve burning and peeling of the skin, the doctor prescribes creams and ointments based on hormonal drugs. During the treatment of lupus erythematosus, special attention is paid to maintaining the patient’s immunity. During remission the patient is prescribed complex vitamins, immunostimulants, physiotherapeutic manipulations. Drugs that stimulate the immune system, such as Azathioprine, are taken only during the lull of the disease, otherwise the patient’s condition may worsen sharply.

Acute lupus

Treatment should begin in the hospital as early as possible. The therapeutic course should be long and constant (without breaks). During the active phase of the pathology, the patient is given glucocorticoids in large doses, starting with 60 mg of Prednisolone and increasing by another 35 mg over 3 months. Reduce the volume of the drug slowly, switching to tablets. Afterwards, a maintenance dose of medication (5-10 mg) is prescribed individually.

To prevent disturbances in mineral metabolism, potassium preparations (Panangin, potassium acetate solution, etc.) are prescribed simultaneously with hormonal therapy. After completion of the acute phase of the disease, complex treatment with corticosteroids is carried out in reduced or maintenance doses. In addition, the patient takes aminoquinoline medications (1 tablet of Delagin or Plaquenil).

Chronic

The earlier treatment is started, the greater the patient’s chances of avoiding irreversible consequences in the body. Therapy for chronic pathology necessarily includes taking anti-inflammatory drugs, drugs that suppress the activity of the immune system (immunosuppressants) and corticosteroid hormonal drugs. However, only half of patients achieve success in treatment. In the absence of positive dynamics, stem cell therapy is performed. As a rule, there is no autoimmune aggression after this.

Why is lupus erythematosus dangerous?

Some patients with this diagnosis develop severe complications - the functioning of the heart, kidneys, lungs, and other organs and systems is disrupted. The most dangerous form of the disease is systemic, which even damages the placenta during pregnancy, resulting in fetal growth retardation or death. Autoantibodies can cross the placenta and cause neonatal (congenital) disease in the newborn. At the same time, the baby develops a skin syndrome that goes away after 2-3 months.

How long do people live with lupus erythematosus?

Thanks to modern medicines, patients can live more than 20 years after diagnosis of the disease. The process of development of pathology occurs at different speeds: in some people, symptoms increase in intensity gradually, in others they increase quickly. Most patients continue to lead their usual lifestyle, but in severe cases of the disease, the ability to work is lost due to severe joint pain, high fatigue, and central nervous system disorders. The duration and quality of life in SLE depends on the severity of symptoms of multiple organ failure.

Video

Drug-induced lupus. Some drugs can cause a syndrome resembling SLE in people without obvious risk factors for it. The most dangerous from this point of view is novocainamide, the use of which causes the formation of antinuclear antibodies in 50-75% of patients several months after the start of treatment; 20% of such individuals develop so-called drug-induced lupus erythematosus. Hydralazine (apressin) causes the formation of antinuclear antibodies in 25 - 30% of individuals, and SLE-like symptoms in 10%. SLE caused by procainamide and hydralazine occurs more often in women of non-Negroid race and, as a rule, in persons with slow acetylation of drugs (this is especially true for hydralazine). In most patients, the syndrome manifests itself as polyarthralgia and general symptoms. In such cases, when taking hydralazine, polyarthritis occurs in 25-50% of patients, and pleuropericarditis - in 30%; when using procainamide, this percentage increases to 50. There are no other manifestations characteristic of idiopathic SLE (in particular, nephritis and central nervous system damage). All patients with drug-induced lupus erythematosus have a positive reaction to antinuclear antibodies; Most also have antibodies to histones. Antibodies to ds DNA and hypocomplementemia are rare, which can be a criterion for differentiating idiopathic and drug-induced lupus. In drug-induced lupus, anemia, leukopenia, thrombocytopenia, false-positive VDRL, positive direct Coombs test, and cryoglobulins may be present. rheumatoid factors, lupus anticoagulant. In such cases, the first step is to stop taking the suspected drug, and in most patients, improvement occurs within a few days or weeks. For persons with severe symptoms of drug-induced SLE, short-course treatment with glucocorticoids is indicated for 2-10 weeks. Clinical symptoms rarely persist for more than 6 months, but antinuclear antibodies can be detected for several years. Drug-induced lupus can also be caused by isoniazid, aminazine, d-ne-nicillamium, Practolol, methyldopa, oral contraceptives and probably hydantoins and Ehtosuximide. Most of these drugs that can cause drug-induced lupus can be safely prescribed to patients with idiopathic lupus if no suitable alternative is available.
Forecast. About 72% of SLE patients survive more than 10 years. Naturally, patients with severe damage to the brain, lungs, heart and kidneys have a much less favorable prognosis. The leading causes of disability and death are infectious complications and renal failure.
Treatment
It should be remembered that specific treatment does not exist in SLE. Complete remissions are extremely rare, so treatment is aimed at containing acute and pronounced exacerbations of the disease, albeit at the cost of the side effects of drug therapy. In 20-30% of patients, SLE is relatively mild, without life-threatening symptoms. However, even with this course of SLE, patients may lose their ability to work due to certain pain sensations or severe weakness. When treating such patients, glucocorticoids should not be used. For arthralgia, arthritis, myalgia, fever, mild serositis, non-steroidal anti-inflammatory drugs, including salicylates, are effective. However, toxic effects when using these drugs occur especially often in patients with SLE (hepatitis, aseptic meningitis, kidney damage). Antimalarial drugs are effective for cutaneous manifestations of SLE, including discoid lupus erythematosus and sometimes lupus arthritis. When taking 400 mg of chloroquine per day, after a few weeks, patients experience an improvement in the condition of their skin (in those who respond positively to treatment). Side effects of this treatment are known: toxic effects on the retina, skin rashes, myopathy and neuropathy. When taking chloroquine, an ophthalmological examination is necessary every 6 months, since the drug has a cumulative effect. Patients with SLE should avoid exposure to sunlight to prevent skin rashes, and if a rash has already appeared, it is recommended local application glucocorticoids. Systemic use of glucocorticoids is appropriate in cases of severe disabling skin lesions.
Patients with severe forms of SLE are treated with high doses of glucocorticoids (1 - 2 mgkg per day). If the disease is in the active phase, then glucocorticoids should be used every 8-12 hours, dividing the daily dose accordingly. After the activity of the process has decreased, the drug should be given to the patient only in the morning, gradually reducing the daily dose in accordance with the clinical manifestations of the disease. Ideally, patients should be switched to a glucocorticoid. short acting(prednisone, prednisolone, methylprednisolone) every other day, with one morning dose, in order to minimize the side effects of the therapy. However, when using an alternating method of using a glucocorticoid (i.e., every other day), new exacerbations of the disease may be observed. In such cases, you should try to find the optimal minimum dose of the drug, used once a day, which will prevent the occurrence of the corresponding symptoms. Side effects of long-term glucocorticoid therapy usually include Cushingoid habitus, weight gain, hypertension, certain infections, increased capillary fragility, acne and hirsutism, accelerated development of osteoporosis, aseptic necrosis bones, cataracts, glaucoma, diabetes mellitus, myopathies, hypokalemia, menstrual irregularities, irritability, insomnia and psychosis. Prednisone at a dose of 15 mg daily (or less) taken before noon usually does not cause suppression of the hypothalamic-pituitary-adrenal axis. Side effects of glucocorticoid therapy are partially preventable. Thus, hyperglycemia, hypertension, edema and hypokalemia may well be corrected by appropriate drug therapy. It is very important to promptly identify infectious processes in order to begin adequate therapy as early as possible. It is advisable and safe for patients with SLE in a stable stage to be immunized against influenza and pneumococcal infections. Prevention of osteoporosis and maintenance of stable bone mass in patients with normal daily calcium excretion in the urine and normal calcium levels in the blood serum, provided they take glucocorticoids in constant doses, is facilitated by additional intake of calcium (1000-1500 mg daily) and vitamin D (50,000 IU/week ). For some patients with acute SLE, including those with diffuse nephritis, it is advisable to carry out so-called pulse therapy, prescribing them 1000 mg of methylprednisolone intravenously for 3-5 days, and then switching to maintenance therapy with glucocorticoids taking the drugs daily or every other day. However, it is not yet clear whether such treatment has any benefits.

The diagnosis is based on characteristic laboratory criteria (determination of antinuclear factor, antinuclear antibodies, LE cells in the blood) and the connection of symptoms with the use of certain medications. Typically, manifestations of drug-induced lupus disappear after discontinuation of the causative drug; in severe cases, corticosteroid drugs are prescribed.

Drug-induced lupus

Drug-induced lupus (drug-induced lupus syndrome) is a symptom complex caused by side effects medicines and regressing after their abolition. Drug-induced lupus is similar in its clinical manifestations and immunobiological mechanisms to systemic lupus erythematosus. In rheumatology, drug-induced lupus is diagnosed approximately 10 times less frequently than idiopathic SLE. In most cases, drug-induced lupus-like syndrome develops in patients over the age of 50 years, with almost the same frequency in men and women.

Causes of drug-induced lupus

The development of drug-induced lupus can be provoked by long-term use or high dosages a wide range of drugs. Medicines with known side effects include antihypertensives (methyldopa, hydralazine, atenolol), antiarrhythmics (procainamide), antituberculosis (isoniazid), anticonvulsants(hydantoin, phenytoin), sulfonamides and antibiotics (penicillin, tetracycline), antipsychotics (chlorpromazine), lithium salts, gold and other drugs. Most often, drug-induced lupus occurs in patients suffering from arterial hypertension, arrhythmia, tuberculosis, epilepsy, rheumatoid arthritis, infectious diseases and taking the listed drugs. It is possible to develop lupus-like syndrome in women who have been using oral contraceptives for a long time.

The pathogenesis of drug-induced lupus is associated with the ability of these drugs to induce the formation of antinuclear antibodies (ANA) in the body. Genetic determination, namely the patient’s acetylating phenotype, plays a major role in predisposition to pathology. Slow acetylation of these drugs by liver enzymes is accompanied by the production of higher AHA titers and a more frequent development of drug-induced lupus. At the same time, in patients with lupus syndrome induced by procainamide or hydralazine, a slow type of acetylation is detected.

In general, the likelihood of developing drug-induced lupus depends on the dose of the drug and the duration of pharmacotherapy. With prolonged use of the drug, 10-30% of patients with antinuclear antibodies in the blood serum develop a lupus-like syndrome.

Symptoms of drug-induced lupus

In the clinic of drug-induced lupus, general manifestations, articular and cardiopulmonary syndromes predominate. The disease can manifest acutely or gradually with nonspecific symptoms such as malaise, myalgia, fever, and slight weight loss. 80% of patients suffer from arthralgia, less often – polyarthritis. In patients taking antiarrhythmic drugs (procainamide), serositis is observed ( exudative pleurisy, pericarditis), cardiac tamponade, pneumonitis, aseptic infiltrates in the lungs. In some cases, the development of lymphadenopathy, hepatomegaly, and the appearance of erythematous rashes on the skin are possible.

Unlike idiopathic systemic lupus erythematosus, butterfly erythema on the cheeks is rare in drug-induced syndrome. ulcerative stomatitis, Raynaud's syndrome, alopecia, nephrotic syndrome, neurological and mental disorders(convulsive syndrome, psychosis). However, drug-induced lupus caused by taking apressin is characterized by the development of glomerulonephritis.

Diagnosis of drug-induced lupus

From the moment the first appeared clinical symptoms Drug-induced lupus often takes several months to several years to be diagnosed. During this time, patients may be examined by a pulmonologist, cardiologist, or rheumatologist regarding individual manifestations of the syndrome to no avail. Correct diagnosis is possible with a comprehensive assessment clinical symptoms, comparing signs of illness with taking certain medications, conducting immunological tests.

The most specific laboratory criteria indicating drug-induced lupus are the presence in the blood of antinuclear antibodies (antibodies to histones), antinuclear factor, antibodies to single-stranded DNA, LE cells, and decreased complement levels. Less specific for lupus syndrome, but highly specific for SLE, are antibodies to ds DNA, anti-Ro/SS-A, Ab to Sm antigen, anti-La/SS-B. Differential diagnosis of drug-induced lupus must be carried out with idiopathic SLE, malignant tumors of the lungs and mediastinum.

Treatment of drug-induced lupus

Discontinuation of the drug that caused drug-induced lupus leads to a gradual regression of the clinical and laboratory signs of the syndrome. Disappearance of clinical symptoms usually occurs within several days or weeks after stopping the drug. Antinuclear antibodies disappear more slowly - within several months (sometimes up to 1 year or longer). In order to relieve articular syndrome, it is possible to prescribe non-steroidal anti-inflammatory drugs. In severe cases of drug-induced lupus and long-term persistence of clinical symptoms, the prescription of glucocorticoids is justified.

To avoid the development of drug-induced lupus, you should not take medications spontaneously and uncontrollably; the prescription of pharmacological agents must be justified and agreed with the attending physician. To prevent relapse of lupus syndrome, adequate replacement of the causative drug with an alternative drug is necessary.

Drug-induced lupus - treatment in Moscow

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Lupus erythematosus - forms (systemic, discoid, drug-induced, etc.), stages, symptoms and manifestations of the disease (photo). Features of symptoms in women and children

Forms of lupus erythematosus

Currently, the following four main forms of lupus erythematosus are distinguished:

3. Neonatal lupus erythematosus in newborn infants.

4. Drug-induced lupus syndrome.

Cutaneous lupus erythematosus (discoid, subacute)

With this form of lupus, only the skin, mucous membranes and joints are affected. Depending on the location and extent of the rash, cutaneous lupus erythematosus can be limited (discoid) or widespread (subacute cutaneous lupus).

Systemic lupus erythematosus

This form of lupus erythematosus is characterized by damage to internal organs with the development of their failure. It is systemic lupus erythematosus that manifests itself with various syndromes from various internal organs, described below in the “symptoms” section.

Neonatal lupus erythematosus

This form of lupus is systemic and develops in newborn infants. Neonatal lupus erythematosus in its course and clinical manifestations fully corresponds to the systemic form of the disease. Neonatal lupus is very rare and affects infants whose mothers suffered from either systemic lupus erythematosus or another immunological pathology during pregnancy. However, this does not mean that a woman with lupus will necessarily have an affected baby. On the contrary, in the vast majority of cases, women suffering from lupus carry and give birth to healthy children.

Drug-induced lupus syndrome

Taking certain medications (for example, Hydralazine, Procainamide, Methyldopa, Guinidine, Phenytoin, Carbamazepine, etc.) as side effects provokes a spectrum of symptoms (arthritis, rash, fever and chest pain) similar to those of systemic lupus erythematosus. It is because of the similarity of the clinical picture that these side effects are called drug-induced lupus syndrome. However, this syndrome is not a disease and goes away completely after discontinuation of the drug that triggered its development.

Symptoms of lupus erythematosus

General symptoms

The symptoms of systemic lupus erythematosus are very variable and diverse, since the inflammatory process damages various organs. Accordingly, for each organ damaged by lupus antibodies, corresponding clinical symptoms appear. And since in different people the pathological process may involve different number organs, then their symptoms will also differ significantly. This means that no two different people with systemic lupus erythematosus will have the same set of symptoms.

• Pain and swelling of the joints (especially large ones);
• Prolonged unexplained increase in body temperature;
• Chronic fatigue syndrome;
• Rashes on the skin (on the face, on the neck, on the torso);
• Chest pain that occurs when taking a deep breath or exhaling;
• Hair loss;
• Sharp and severe pallor or blue discoloration of the skin of the toes and hands in the cold or during a stressful situation (Raynaud's syndrome);
• Swelling of the legs and area around the eyes;
• Enlarged and painful lymph nodes;
• Sensitivity to solar radiation;
• Headaches and dizziness;
• Convulsions;
• Depression.

These general symptoms, as a rule, are present in various combinations in all people suffering from systemic lupus erythematosus. That is, everyone suffering from lupus experiences at least four of the above general symptoms. The general main symptoms of various organs in lupus erythematosus are shown schematically in Figure 1.

Figure 1 – General symptoms of lupus erythematosus from various organs and systems.

Symptoms of systemic lupus erythematosus from the skin and mucous membranes: red spots on the face, scleroderma with lupus erythematosus (photo)

Changes in the color, structure and properties of the skin or the appearance of rashes on the skin is the most common syndrome in systemic lupus erythematosus, which occurs in 85 - 90% of people suffering from this disease. Thus, currently there are approximately 28 different variants of skin changes in lupus erythematosus. Let's look at the most typical skin symptoms for lupus erythematosus.

Figure 2 – “Butterfly” shaped rash on the face.

• Vasculitic “butterfly” is a diffuse, pulsating redness with a bluish tint, localized on the nose and cheeks. This redness is unstable; it intensifies when the skin is exposed to frost, wind, sun or excitement, and, on the contrary, decreases when exposed to favorable environmental conditions (see Figure 3).
• "Butterfly" type of centrifugal erythema (erythema of Biette) is a collection of persistent red, swollen spots located on the cheeks and nose. Moreover, on the cheeks most often the spots are not located near the nose, but, on the contrary, in the temple area and along the imaginary beard growth line (see Figure 4). These spots do not go away and their intensity does not decrease under favorable environmental conditions. On the surface of the spots there is moderate hyperkeratosis (peeling and thickening of the skin).
• Kaposi's "butterfly" is a collection of bright pink, dense and swollen spots located on the cheeks and nose against a generally red face. A characteristic feature of this “butterfly” shape is that the spots are located on the swollen and red skin of the face (see Figure 5).
• A “butterfly” of discoid-type elements is a collection of bright red, swollen, inflamed, flaky spots located on the cheeks and nose. The spots with this “butterfly” shape are at first simply red, then they become swollen and inflamed, as a result of which the skin in this area thickens, begins to peel off and die. Further, when the inflammatory process passes, scars and areas of atrophy remain on the skin (see Figure 6).

Figure 4 – “Butterfly” type of centrifugal erythema.

Figure 5 – “Butterfly” by Kaposi.

Figure 6 – “Butterfly” with discoid elements.

Figure 7 - Capillaritis of the fingertips and palms with lupus erythematosus.

• Aphthous stomatitis;
• Enanthema of the oral mucosa (areas of the mucous membrane with hemorrhages and erosions);
• Oral candidiasis;
• Erosions, ulcers and whitish plaques on the mucous membrane of the mouth and nose.

“Dry syndrome” in lupus erythematosus is characterized by dryness of the skin and vagina.

Symptoms of systemic lupus erythematosus in bones, muscles and joints (lupus arthritis)

Damage to joints, bones and muscles is typical of lupus erythematosus and occurs in 90–95% of people with the disease. Muscular joint syndrome in lupus can manifest itself in the following clinical forms:

• Prolonged pain in one or more joints of high intensity.
• Polyarthritis involving the symmetrical interphalangeal joints of the fingers, metacarpophalangeal, wrist, and knee joints.
• Morning stiffness of the affected joints (in the morning, immediately after waking up, it is difficult and painful to move the joints, but after a while, after “warming up,” the joints begin to function almost normally).
• Flexion contractures of the fingers due to inflammation of the ligaments and tendons (the fingers freeze in a bent position, and it is impossible to straighten them due to the fact that the ligaments and tendons have shortened). Contractures are rare, occurring in no more than 1.5–3% of cases.
• Rheumatoid-like appearance of the hands (swollen joints with bent, non-extending fingers).
• Aseptic necrosis of the head of the femur, humerus and other bones.
• Muscle pain.
• Muscle weakness.
• Polymyositis.

Like skin, joint-muscular syndrome in lupus erythematosus can manifest itself in the above clinical forms in any combination and quantity. This means that one person with lupus may have only lupus arthritis, another may have arthritis + polymyositis, and another may have the full spectrum of clinical forms of musculoskeletal syndrome (muscle pain, arthritis, morning stiffness, etc.). ).

• With systemic lupus erythematosus, joint damage is migrating (arthritis of the same joint appears and disappears), and with rheumatoid arthritis, it is progressive (the same affected joint hurts constantly, and its condition worsens over time);
• Morning stiffness in systemic lupus erythematosus is moderate and is observed only during the period of active arthritis, and in rheumatoid arthritis it is constant, present even during remission, and very intense;
• Transient flexion contractures (the joint is deformed during a period of active inflammation, and then restores its normal structure in remission) are characteristic of lupus erythematosus and are absent in rheumatoid arthritis;
• Irreversible contractures and joint deformities almost never occur with lupus erythematosus and are characteristic of rheumatoid arthritis;
• The dysfunction of joints in lupus erythematosus is insignificant, and in rheumatoid arthritis it is pronounced;
• Bone erosions are absent in lupus erythematosus, but are present in rheumatoid arthritis;
• Rheumatoid factor in lupus erythematosus is not always detected, and only in 5–25% of people, and in rheumatoid arthritis it is always present in the blood serum in 80%;
• A positive LE test for lupus erythematosus occurs in 85%, and for rheumatoid arthritis only in 5–15%.

Symptoms of systemic lupus erythematosus from the lungs

Pulmonary syndrome in lupus erythematosus is a manifestation of systemic vasculitis (inflammation of blood vessels) and develops only during the active course of the disease against the background of involvement of other organs and systems in the pathological process in approximately 20–30% of patients. In other words, lung damage in lupus erythematosus occurs only simultaneously with skin and joint-muscular syndrome, and never develops in the absence of damage to the skin and joints.

• Lupus pneumonitis (pulmonary vasculitis) is an inflammation of the lungs that occurs with high body temperature, shortness of breath, silent moist rales and a dry cough, sometimes accompanied by hemoptysis. In lupus pneumonitis, inflammation does not affect the alveoli of the lungs, but the intercellular tissues (interstitium), as a result of which the process is similar to atypical pneumonia. X-rays with lupus pneumonitis reveal disc-shaped atelectasis (expansion), shadows of infiltrates and increased pulmonary pattern;
• Pulmonary hypertension syndrome (increased pressure in the pulmonary vein system) is manifested by severe shortness of breath and systemic hypoxia of organs and tissues. With lupus pulmonary hypertension, there are no changes on X-ray of the lungs;
• Pleurisy (inflammation of the pleural membrane of the lungs) - manifested by severe chest pain, severe shortness of breath and accumulation of fluid in the lungs;
• Thromboembolism pulmonary artery(TELA);
• Hemorrhages in the lungs;
• Fibrosis of the diaphragm;
• Lung dystrophy;
• Polyserositis is a migratory inflammation of the pleura of the lungs, pericardium of the heart and peritoneum. That is, a person alternately periodically experiences inflammation of the pleura, pericardium and peritoneum. These serosites are manifested by pain in the abdomen or chest, friction rub of the pericardium, peritoneum or pleura. But due to the low severity of clinical symptoms, polyserositis is often overlooked by doctors and patients themselves, who consider their condition to be a consequence of the disease. Each relapse of polyserositis leads to the formation of adhesions in the chambers of the heart, on the pleura and in the abdominal cavity, which are clearly visible on x-rays. Due to adhesive disease, an inflammatory process may occur in the spleen and liver.

Symptoms of systemic lupus erythematosus from the kidneys

With systemic lupus erythematosus, 50–70% of people develop inflammation of the kidneys, which is called lupus nephritis or lupus nephritis. As a rule, nephritis of varying degrees of activity and severity of kidney damage develops within five years from the onset of systemic lupus erythematosus. For many people, lupus nephritis is one of the initial manifestations of lupus, along with arthritis and butterfly dermatitis.

• Rapidly progressing lupus nephritis - manifested by severe nephrotic syndrome (edema, protein in the urine, bleeding disorders and a decrease in the level of total protein in the blood), malignant arterial hypertension and the rapid development of renal failure;
• Nephrotic form of glomerulonephritis (manifested by protein and blood in the urine in combination with arterial hypertension);
• Active lupus nephritis with urinary syndrome (manifested by protein in the urine more than 0.5 g per day, a small amount of blood in the urine and leukocytes in the urine);
• Nephritis with minimal urinary syndrome (manifested by protein in the urine less than 0.5 g per day, single erythrocytes and leukocytes in the urine).

The nature of the damage in lupus nephritis is different, as a result of which the World Health Organization identifies 6 classes of morphological changes in the structure of the kidneys characteristic of systemic lupus erythematosus:

• Class I – the kidneys have normal, unchanged glomeruli.
• Class II – the kidneys have only mesangial changes.
• Class III - in less than half of the glomeruli there is infiltration of neutrophils and proliferation (increase in the number) of mesangial and endothelial cells, narrowing the lumen of blood vessels. If necrosis processes occur in the glomeruli, then destruction of the basement membrane, disintegration of cell nuclei, hematoxylin bodies and blood clots in the capillaries are also detected.
• Class IV - changes in the structure of the kidneys are of the same nature as in class III, but they affect most of the glomeruli, which corresponds to diffuse glomerulonephritis.
• Class V - in the kidneys, thickening of the walls of the glomerular capillaries with expansion of the mesangial matrix and an increase in the number of mesangial cells is detected, which corresponds to diffuse membranous glomerulonephritis.
• Class VI - sclerosis of the glomeruli and fibrosis of the intercellular spaces are detected in the kidneys, which corresponds to sclerosing glomerulonephritis.

In practice, as a rule, when diagnosing lupus nephritis in the kidneys, class IV morphological changes are detected.

Symptoms of systemic lupus erythematosus from the central nervous system

Damage to the nervous system is a severe and unfavorable manifestation of systemic lupus erythematosus, caused by damage to various nervous structures in all parts (both in the central and peripheral nervous systems). The structures of the nervous system are damaged due to vasculitis, thrombosis, hemorrhages and infarctions resulting from a violation of the integrity of the vascular wall and microcirculation.

• Migraine-type headaches that are not relieved by non-narcotic and narcotic painkillers;
• Transient ischemic attacks;
• Cerebrovascular accident;
• Convulsive seizures;
• Chorea;
• Cerebral ataxia (disorder of coordination of movements, the appearance of uncontrolled movements, tics, etc.);
• Neuritis cranial nerves(visual, olfactory, auditory, etc.);
• Neuritis optic nerve with impaired or complete loss of vision;
• Transverse myelitis;
• Peripheral neuropathy (damage to sensory and motor fibers of nerve trunks with the development of neuritis);
• Impaired sensitivity – paresthesia (feeling of “pins and needles”, numbness, tingling);
• Organic brain damage, manifested by emotional instability, periods of depression, as well as significant deterioration of memory, attention and thinking;
• Psychomotor agitation;
• Encephalitis, meningoencephalitis;
• Persistent insomnia with short periods of sleep, during which a person sees colorful dreams;
• Affective disorders:
  • Anxious depression with vocal hallucinations of condemnatory content, fragmentary ideas and unstable, unsystematized delusions;
  • Manic-euphoric state with elevated mood, carelessness, self-satisfaction and lack of awareness of the severity of the disease;
• Delirious-oneiric clouding of consciousness (manifested by alternating dreams on fantastic themes with colorful visual hallucinations. Often people associate themselves as observers of hallucinatory scenes or victims of violence. Psychomotor agitation is confused and fussy, accompanied by immobility with muscle tension and a prolonged cry);
• Delirious clouding of consciousness (manifested by a feeling of fear, as well as vivid nightmares during the period of falling asleep and multiple colored visual and speech hallucinations of a threatening nature during waking moments);
• Strokes.

Symptoms of systemic lupus erythematosus from the gastrointestinal tract and liver

Lupus erythematosus causes damage to blood vessels in organs digestive tract and peritoneum, resulting in the development of dyspeptic syndrome (impaired digestion of food), pain syndrome, anorexia, inflammation of the abdominal organs and erosive and ulcerative lesions of the mucous membranes of the stomach, intestines and esophagus.

• Aphthous stomatitis and ulceration of the tongue;
• Dyspeptic syndrome, manifested by nausea, vomiting, lack of appetite, bloating, flatulence, heartburn and stool disorder (diarrhea);
• Anorexia, which occurs as a result of unpleasant dyspeptic symptoms that appear after eating;
• Expansion of the lumen and ulceration of the mucous membrane of the esophagus;
• Ulceration of the mucous membrane of the stomach and duodenum;
• Abdominal pain syndrome (abdominal pain), which can be caused by vasculitis large vessels abdominal cavity (splenic, mesenteric arteries, etc.), and inflammation of the intestines (colitis, enteritis, ileitis, etc.), liver (hepatitis), spleen (splenitis) or peritoneum (peritonitis). The pain is usually localized in the navel area, and is combined with rigidity of the muscles of the anterior abdominal wall;
• Enlarged abdominal lymph nodes;
• Increased size of the liver and spleen with possible development hepatitis, fatty hepatosis or splenitis;
• Lupus hepatitis, manifested by an increase in the size of the liver, yellowing of the skin and mucous membranes, as well as an increase in the activity of AST and ALT in the blood;
• Vasculitis of abdominal vessels with bleeding from the digestive tract;
• Ascites (accumulation of free fluid in the abdominal cavity);
• Serositis (inflammation of the peritoneum), which is accompanied by severe pain that mimics the picture “ acute abdomen».

Various manifestations of lupus from the digestive tract and abdominal organs are caused by vascular vasculitis, serositis, peritonitis and ulceration of the mucous membranes.

Symptoms of systemic lupus erythematosus from the cardiovascular system

With lupus erythematosus, the outer and inner membranes, as well as the heart muscle, are damaged and, in addition, inflammatory diseases of small vessels develop. Cardiovascular syndrome develops in 50–60% of people suffering from systemic lupus erythematosus.

• Pericarditis is an inflammation of the pericardium (the outer lining of the heart), in which a person experiences pain in the chest, shortness of breath, dull heart sounds, and he takes a forced sitting position (a person cannot lie down, it is easier for him to sit, so he even sleeps on a high pillow ). In some cases, you can hear a pericardial friction rub, which occurs when there is effusion in the chest cavity. The main method for diagnosing pericarditis is an ECG, which reveals a decrease in T wave voltage and a shift in the ST segment.
• Myocarditis is an inflammation of the heart muscle (myocardium), which very often accompanies pericarditis. Isolated myocarditis in lupus erythematosus is rare. With myocarditis, a person develops heart failure and is bothered by chest pain.
• Endocarditis is an inflammation of the lining of the chambers of the heart, and is manifested by atypical verrucous Libman-Sachs endocarditis. In lupus endocarditis, the mitral, tricuspid and aortic valves are involved in the inflammatory process with the formation of their insufficiency. Most often, mitral valve insufficiency occurs. Endocarditis and damage to the valvular apparatus of the heart usually occur without clinical symptoms, and therefore they are detected only during echocardiography or ECG.
• Phlebitis and thrombophlebitis are inflammation of the walls of blood vessels with the formation of blood clots in them and, accordingly, thrombosis in various organs and tissues. Clinically, these conditions manifest themselves pulmonary hypertension, arterial hypertension, endocarditis, myocardial infarction, chorea, myelitis, liver hyperplasia, thrombosis of small vessels with the formation of foci of necrosis in various organs and tissues, as well as infarctions of the abdominal organs (liver, spleen, adrenal glands, kidneys) and cerebrovascular accidents. Phlebitis and thrombophlebitis are caused by antiphospholipid syndrome, which develops in lupus erythematosus.
• Coronaritis (inflammation of the heart vessels) and atherosclerosis of the coronary vessels.
• Coronary heart disease and strokes.
• Raynaud's syndrome is a disorder of microcirculation, manifested by a sharp whitening or blue discoloration of the skin of the fingers in response to cold or stress.
• Marble pattern of the skin (livedo reticularis) due to impaired microcirculation.
• Necrosis of the fingertips (blue discoloration of the fingertips).
• Retinal vasculitis, conjunctivitis and episcleritis.

Course of lupus erythematosus

Systemic lupus erythematosus occurs in waves, with alternating periods of exacerbations and remissions. Moreover, during exacerbations, a person develops symptoms from various affected organs and systems, and during periods of remission there are no clinical manifestations of the disease. The progression of lupus is that with each subsequent exacerbation, the degree of damage in already affected organs increases, and other organs are involved in the pathological process, which entails the appearance of new symptoms that were not there before.

• Acute course - lupus erythematosus begins abruptly, with a sudden increase in body temperature. A few hours after the temperature rises, arthritis of several joints appears at once with sharp pain in them and rashes on the skin, including “butterfly”. Then, within just a few months (3 – 6), arthritis, dermatitis and fever are joined by polyserositis (inflammation of the pleura, pericardium and peritoneum), lupus nephritis, meningoencephalitis, myelitis, radiculoneuritis, strong weight loss and tissue nutritional disorders. The disease progresses rapidly due to the high activity of the pathological process; irreversible changes appear in all organs, as a result of which, 1–2 years after the onset of lupus, in the absence of therapy, multiple organ failure develops, ending in death. The acute course of lupus erythematosus is the most unfavorable, since pathological changes in organs develop too quickly.
• Subacute course - lupus erythematosus manifests itself gradually, first joint pain appears, then arthritis is accompanied by skin syndrome (“butterfly” on the face, rashes on the skin of the body) and body temperature rises moderately. For a long time, the activity of the pathological process is low, as a result of which the disease progresses slowly, and organ damage remains minimal for a long time. For a long time, there are injuries and clinical symptoms in only 1–3 organs. However, over time, all organs are still involved in the pathological process, and with each exacerbation, an organ that was not previously affected is damaged. Subacute lupus is characterized by long-term remissions – up to six months. The subacute course of the disease is due to the average activity of the pathological process.
• Chronic course - lupus erythematosus manifests itself gradually, first with arthritis and skin changes. Further, due to the low activity of the pathological process over many years, a person has damage to only 1–3 organs and, accordingly, clinical symptoms only on their part. After years (10–15 years), lupus erythematosus still leads to damage to all organs and the appearance of corresponding clinical symptoms.

Lupus erythematosus, depending on the speed of involvement of organs in the pathological process, has three degrees of activity:

• I degree of activity - the pathological process is inactive, organ damage develops extremely slowly (it takes up to 15 years for failure to form). For a long time, inflammation affects only the joints and skin, and the involvement of undamaged organs in the pathological process occurs slowly and gradually. The first degree of activity is characteristic of the chronic course of lupus erythematosus.
• II degree of activity - the pathological process is moderately active, organ damage develops relatively slowly (it takes up to 5-10 years for failure to form), involvement of unaffected organs in the inflammatory process occurs only with relapses (on average, once every 4-6 months). The second degree of activity of the pathological process is characteristic of the subacute course of lupus erythematosus.
• III degree of activity - the pathological process is very active, damage to organs and the spread of inflammation occurs very quickly. The third degree of activity of the pathological process is characteristic of the acute course of lupus erythematosus.

The table below shows the severity of clinical symptoms characteristic of each of the three degrees of activity of the pathological process in lupus erythematosus.

Symptoms of lupus erythematosus in women

Symptoms of lupus erythematosus in women fully correspond to the clinical picture of any form of the disease, which are described in the sections above. The symptoms of lupus in women do not have any specific features. The only features of the symptoms are the greater or lesser frequency of damage to one or another organ, unlike men, but the clinical manifestations of the damaged organ themselves are absolutely typical.

Lupus erythematosus in children

As a rule, the disease affects girls 9–14 years old, that is, those who are at the age of the onset and flourishing of hormonal changes in the body (the beginning of menstruation, the growth of pubic and armpit hair, etc.). In rare cases, lupus develops in children 5 to 7 years old.

Lupus erythematosus: symptoms of various forms and types of disease (systemic, discoid, disseminated, neonatal). Symptoms of lupus in children - video

Systemic lupus erythematosus in children and pregnant women: causes, consequences, treatment, diet (doctor’s recommendations) - video

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Autoimmune diseases caused by drugs

Lupus-like syndrome

The first convincing data on the development of lupus-like syndrome after the use of hydralazine in the treatment of hypertension appeared in 1953. A report of a SLE-like syndrome caused by the administration of procainamide was published in 1962, and today this drug is the most commonly cited cause of drug-induced lupus-like syndrome in the United States. Other drugs that can cause this pathology include isoniazid, chlorpromazine, methyldopa and quinidine. A possible connection with the development of the syndrome has been noted with the use of many anticonvulsants, β-blockers, antithyroid drugs, penicillamine, sulfasalazine and lithium preparations. The prevalence of lupus-like syndrome caused by drugs has not been precisely established. Among all patients with SLE, it is about 3%. Patients with idiopathic SLE have not been shown to have an increased predisposition to developing hypersensitivity to drugs that cause drug-induced lupus erythematosus. The disease begins acutely and is manifested by fever, general malaise, arthralgia, myalgia and slight loss of body weight. Cardiopulmonary disorders with signs of pleurisy and pleural effusion, pulmonary infiltrates, and effusion pericarditis are more often observed in patients taking procainamide. Unlike idiopathic SLE, drug-induced lupus erythematosus rarely causes the classic butterfly rash on the cheeks, discoid lesions, ulcerative defects of the oral mucosa, Raynaud's phenomenon, alopecia, as well as kidney and central nervous system lesions. Drug-induced lupus erythematosus usually has a more favorable course compared to idiopathic SLE. To diagnose the disease, a study should be conducted for the presence of antinuclear antibodies, since many clinical manifestations are nonspecific. Clinical improvement occurs within several days or weeks after stopping the drug that caused the development of the syndrome. After treatment, clinical signs of the disease do not appear for many months. In some cases, symptoms may persist or recur periodically for several months before disappearing. Antinuclear ATs usually disappear within a few weeks or months, but can sometimes persist for a year or more. The detection of antinuclear ATs in patients without clinical symptoms cannot serve as a basis for drug withdrawal. For mild symptoms, the use of NSAIDs is quite effective; more severe forms diseases may require GC therapy. In cases where treatment with the drug that caused the development of the disease is necessary, and a suitable alternative drug is not available, the use of drugs simultaneously with GC is allowed. If the reaction is caused by procainamide, it is replaced with the main acetylated metabolite of procainamide, N-acetylprocainamide, when transferred to treatment with which the symptoms of procainamide-induced lupus disappear. Other autoimmune disorders caused by hypersensitivity to drugs By binding to cell membranes, penicillamine as a hapten induces an autoaggressive T-cell response, B-lymphocyte proliferation and autoantibody synthesis with the subsequent development of autoimmune disorders. In addition to drug-induced lupus erythematosus, the use of penicillamine can lead to the development of Erb-Goldflam disease, polymyositis and dermatomyositis, pemphigus and pemphigoid, membranous glomerulonephritis, Goodpasser syndrome, and immune cytopenias.

Allergic vasculitis

The development of allergic vasculitis is characterized by inflammation and necrosis of blood vessels. Richly vascularized organs, particularly the skin, are most often affected. Compared with hypersensitivity vasculitis, skin lesions in systemic necrotizing vasculitis (periarteritis nodosa, allergic granulomatosis in Churg-Strauss syndrome) and granulomatous vasculitis (Wegener's granulomatosis, lymphomatoid granulomatosis, giant cell arteritis) are less common. In addition, systemic necrotizing and granulomatous vasculitis in most cases are not associated with the use of drugs. Allergic vasculitis can occur at any age, being most common among patients between 40 and 50 years of age. In elderly people, the likelihood of developing this syndrome is higher, which is associated with a higher frequency of use of drugs: cardiac drugs, diuretics, etc. Among the most common causes of hypersensitivity vasculitis are penicillin, sulfonamides, thiouracils, hydantoins, iodides and allopurinol. The latter, when prescribed to patients with kidney disease receiving thiazide diuretics, can cause the development of vasculitis, accompanied by fever, rash, leukocytosis and eosinophilia, signs of damage to the liver parenchyma and kidney damage (up to the development of renal failure), which leads to death up to 25%. sick. In many cases of hypersensitivity vasculitis, the cause cannot be determined. The most typical clinical sign of hypersensitivity vasculitis is a hemorrhagic rash. The rashes, varying in size and number, are periodic in nature, usually located symmetrically on the lower extremities and in the sacral area. The appearance of skin symptoms may be accompanied by fever, malaise, myalgia and anorexia. Most often, hypersensitivity vasculitis caused by drugs is limited only to skin manifestations, but in more severe cases, damage to the joints (arthralgia or arthritis), kidneys (glomerulonephritis) occurs; gastrointestinal bleeding poses an immediate threat to life; rare manifestations include pulmonary infiltrates and peripheral neurological disorders. The diagnosis of hypersensitivity vasculitis is confirmed by a biopsy of the affected areas of the skin, where characteristic neutrophilic infiltration of the walls of blood vessels is revealed, resulting in necrosis, breakdown of leukocytes (“atomization11 or fragmentation of nuclei), fibrinoid changes and extravasation of red blood cells. Inflammation affects small blood vessels, mainly post-capillary venules. Prognosis for most cases of hypersensitivity vasculitis, it is favorable; for the treatment of mild skin forms of the disease, it is enough to cancel the drug that caused the development of the syndrome. Patients with generalized manifestations and damage to internal organs are prescribed GC.