What does lupus erythematosus look like? Systemic lupus erythematosus

Systemic lupus erythematosus- a chronic systemic disease, with the most pronounced manifestations on the skin; The etiology of lupus erythematosus is not known, but its pathogenesis is associated with a violation of autoimmune processes, resulting in the production of antibodies to healthy cells of the body. The disease is more susceptible to middle-aged women. Incidence lupus erythematosus not high - 2-3 cases per thousand people. Treatment and diagnosis of systemic lupus erythematosus is carried out jointly by a rheumatologist and a dermatologist. The diagnosis of SLE is made on the basis of typical clinical signs and laboratory test results.

General information

Systemic lupus erythematosus- a chronic systemic disease, with the most pronounced manifestations on the skin; The etiology of lupus erythematosus is not known, but its pathogenesis is associated with a violation of autoimmune processes, resulting in the production of antibodies to healthy cells of the body. The disease is more susceptible to middle-aged women. The incidence of lupus erythematosus is not high - 2-3 cases per thousand people of the population.

Development and suspected causes of systemic lupus erythematosus

The exact etiology of lupus erythematosus has not been established, but antibodies to the Epstein-Barr virus were found in most patients, which confirms the possible viral nature of the disease. Features of the body, due to which autoantibodies are produced, are also observed in almost all patients.

The hormonal nature of lupus erythematosus has not been confirmed, but hormonal disorders worsen the course of the disease, although they cannot provoke its occurrence. Women diagnosed with lupus erythematosus are not recommended to take oral contraceptives. In people with a genetic predisposition and in identical twins, the incidence of lupus erythematosus is higher than in other groups.

The pathogenesis of systemic lupus erythematosus is based on impaired immunoregulation, when protein components of the cell, primarily DNA, act as autoantigens, and as a result of adhesion, even those cells that were originally free of immune complexes become targets.

Clinical picture of systemic lupus erythematosus

With lupus erythematosus, connective tissue, skin and epithelium are affected. An important diagnostic sign is a symmetrical lesion of large joints, and if joint deformity occurs, then due to the involvement of ligaments and tendons, and not due to erosive lesions. Myalgia, pleurisy, pneumonitis are observed.

But the most striking symptoms of lupus erythematosus are noted on the skin, and it is precisely for these manifestations that the diagnosis is made in the first place.

In the initial stages of the disease, lupus erythematosus is characterized by a continuous course with periodic remissions, but almost always goes into a systemic form. More often there is erythematous dermatitis on the face like a butterfly - erythema on the cheeks, cheekbones and always on the back of the nose. Hypersensitivity to solar radiation appears - photodermatoses are usually round in shape, are of a multiple nature. In lupus erythematosus, a feature of photodermatosis is the presence of a hyperemic corolla, an area of ​​atrophy in the center, and depigmentation of the affected area. Pityriasis scales, which cover the surface of erythema, are tightly soldered to the skin and attempts to separate them are very painful. At the stage of atrophy of the affected skin, the formation of a smooth, delicate alabaster-white surface is observed, which gradually replaces the erythematous areas, starting from the middle and moving to the periphery.

In some patients with lupus erythematosus, the lesions extend to the scalp, causing total or partial alopecia. If the lesions affect the red border of the lips and the mucous membrane of the mouth, then the lesions are bluish-red dense plaques, sometimes with pityriasis scales on top, their contours have clear boundaries, the plaques are prone to ulceration and cause pain during eating.

Lupus erythematosus has a seasonal course, and in the autumn-summer periods, the skin condition deteriorates sharply due to more intense exposure to solar radiation.

In the subacute course of lupus erythematosus, psoriasis-like foci are observed throughout the body, telangiectasias are pronounced, a reticular livedio (tree-like pattern) appears on the skin of the lower extremities. Generalized or focal alopecia, urticaria and pruritus are observed in all patients with systemic lupus erythematosus.

In all organs where there is connective tissue, pathological changes begin over time. With lupus erythematosus, all membranes of the heart, renal pelvis, gastrointestinal tract and central nervous system are affected.

If, in addition to skin manifestations, patients suffer from recurrent headaches, joint pains without any connection with injuries and weather conditions, there are violations of the work of the heart and kidneys, then on the basis of the survey, one can assume deeper and systemic disorders and examine the patient for the presence of lupus erythematosus. An abrupt change in mood from a euphoric state to a state of aggression is also characteristic manifestation lupus erythematosus.

In elderly patients with lupus erythematosus, skin manifestations, renal and arthralgic syndromes are less pronounced, but Sjögren's syndrome is more often observed - this is an autoimmune lesion. connective tissue, manifested by hyposecretion of the salivary glands, dryness and pain in the eyes, photophobia.

Children with neonatal lupus erythematosus, born of sick mothers, have an erythematous rash and anemia already in infancy, so a differential diagnosis should be made with atopic dermatitis.

Diagnosis of systemic lupus erythematosus

If systemic lupus erythematosus is suspected, the patient is referred for a consultation with a rheumatologist and a dermatologist. Lupus erythematosus is diagnosed by the presence of manifestations in each symptomatic group. Criteria for diagnosis from the skin: butterfly-shaped erythema, photodermatitis, discoid rash; on the part of the joints: symmetrical damage to the joints, arthralgia, "pearl bracelets" syndrome on the wrists due to deformation of the ligamentous apparatus; on the part of the internal organs: serositis of various localization, persistent proteinuria and cylindruria in the analysis of urine; from the central nervous system: convulsions, chorea, psychosis and mood changes; from the function of hematopoiesis, lupus erythematosus is manifested by leukopenia, thrombocytopenia, lymphopenia.

The Wasserman reaction can be false positive, like other serological studies, which sometimes leads to the appointment of inadequate treatment. With the development of pneumonia, an x-ray of the lungs is performed, if pleurisy is suspected -

Patients with lupus erythematosus should avoid direct sunlight, wear clothing that covers the entire body, and apply creams with a high protective UV filter to exposed areas. Corticosteroid ointments are applied to the affected areas of the skin, since the use non-hormonal drugs brings no effect. Treatment must be carried out intermittently so that hormone-induced dermatitis does not develop.

In uncomplicated forms of lupus erythematosus, non-steroidal anti-inflammatory drugs are prescribed to eliminate pain in the muscles and joints, but aspirin should be taken with caution, as it slows down the blood clotting process. It is obligatory to take glucocorticosteroids, while the doses of drugs are selected in such a way as to minimize side effects to protect internal organs from damage.

A method when a patient is taken stem cells, and then immunosuppressive therapy is carried out, after which for recovery immune system reintroduced stem cells, effective even in severe and hopeless forms of lupus erythematosus. With such therapy, autoimmune aggression in most cases stops, and the patient's condition with lupus erythematosus improves.

A healthy lifestyle, avoiding alcohol and smoking, adequate physical activity, balanced diet and psychological comfort allow patients with lupus erythematosus to control their condition and prevent disability.

Diseases skin type can occur quite often in people and manifest themselves in the form of extensive symptoms. The nature and causal factors that give rise to these phenomena often remain the objects of controversy among scientists. long years. One of the diseases that has an interesting nature of origin is lupus. Disease has many characteristic features and several basic causes of occurrence, which will be discussed in the material.

What is systemic lupus erythematosus?

Lupus is also known as SLE, Systemic Lupus Erythematosus. What is lupus- This is a serious diffuse disease associated with the functioning of the connective tissue, manifested by a systemic type lesion. The disease has an autoimmune character, during which healthy cellular elements are damaged by antibodies produced in the immune system, and this leads to the presence of a vascular component with connective tissue damage.

The name of the disease was due to the fact that it is characterized by the formation of special symptoms, the most important of which is. It is localized in different places, and in shape resembles a butterfly.

According to data from the medieval era, the lesions resemble places with wolf bites. Lupus disease widespread, its essence boils down to the peculiarities of the body's perception of its own cells, or rather, a change in this process, therefore, damage to the whole organism occurs.

According to statistics, SLE affects 90% of female representatives, the manifestation of the first signs is carried out in young age from 25 to 30 years old.

Often, the disease comes uninvited during or after pregnancy, so there is an assumption that female hormones act as the dominant factors in education.

The disease has a familial property, but cannot have a hereditary factor. Many sick people who have previously suffered from allergies to food or drugs are at risk of contracting the disease.

Lupus causes the disease

Modern medical representatives have lengthy discussions regarding the nature of the origin this disease. The most common belief is the extensive influence of family and hereditary factors, viruses and other elements. The immune system of persons susceptible to the disease is most sensitive to external influences. The disease that occurs from drugs is rare, therefore, after stopping the drug, its effect stops.

Causal factors that most often lead to the formation of the disease include.

1. Prolonged exposure to sunlight.
2. Chronic phenomena that carry a viral nature.
3. Stress and emotional overstrain.
4. Significant hypothermia of the body.

To reduce the risk factors for the development of the disease, it is recommended to prevent the impact of these factors and their detrimental effects on the body.

Lupus erythematosus symptoms and signs

Affected persons usually suffer from uncontrolled temperature changes on the body, pain in the head and weakness. Often there is rapid fatigue, and pain in the muscles area is manifested. These symptoms are ambiguous, but lead to an increased likelihood of SLE. The nature of the lesion is accompanied by several factors within which the lupus disease manifests itself.

Dermatological manifestations

Skin formations occur in 65% of affected people, but only in 50% - this is a characteristic "butterfly" on the cheeks. In some patients, the lesion manifests itself in the form of a symptom such as localized on the trunk itself, limbs, vagina, mouth, nose.

Often the disease is characterized by the formation trophic ulcers. In women, hair loss is observed, and nails become very brittle.

Manifestations of the orthopedic type

Many people suffering from the disease experience significant joint pain, traditionally small items on the hands and wrists are affected. There is such a thing as severe arthralgia, but with SLE there is no destruction of bone tissue. Deformed joints are damaged, and this is irreversible in about 20% of patients.

Hematological signs of the disease

In men and the fair sex, as well as in children, the formation of an LE-cell phenomenon occurs, accompanied by the formation of new cells. They contain the main fragments of the nuclei of other cellular elements. Half of the patients suffer from anemia, leukopenia, thrombocytopenia, acting as a consequence of a systemic disease or a side effect of ongoing therapy.

Manifestations of a cardiac character

These symptoms may even occur in children. Patients may experience pericarditis, endocarditis, mitral valve damage, and atherosclerosis. These diseases do not always occur, but are at an increased risk in people who have been diagnosed with SLE the day before.

Sign Factors Associated with Kidneys

In the disease, lupus nephritis often manifests itself, which is accompanied by damage to the renal tissues, there is a noticeable thickening of the basement membranes of the glomeruli, and fibrin is deposited. The only symptom is often hematuria, proteinuria. Early diagnosis contributes to the fact that the incidence of acute kidney failure of all symptoms is no more than 5%. There may be deviations in work in the form of nephritis - this is one of the most serious lesions of organs with a frequency of formation that depends on the degree of disease activity.

Manifestations of a neurological nature

There are 19 syndromes that are characteristic of the disease in question. These are complex diseases in the form of psychosis, convulsive syndromes, paresthesia. Diseases are accompanied by a particularly persistent nature of the course.

Factors for diagnosing the disease

• Rash in the cheekbones (“lupus butterfly”) - and upper limbs manifests itself extremely rarely (in 5% of cases), with facial lupus it is not localized there;
• Erythema and enanthema, characterized by ulcers in the region of the mouth;
• Arthritis in peripheral joints;
• Pleurisy or pericarditis in acute manifestations;
• The defeat phenomena with kidneys;
• Difficulties in the work of the central nervous system, psychosis, convulsive state of special frequency;
• The formation of significant hematological disorders.

The question also arises: lupus erythematosus - does it itch or not. In fact, disease doesn't hurt or itch. If there are at least 3-4 criteria from the above list at any time from the beginning of the development of the picture, the doctors make an appropriate diagnosis.

• The red decollete zone of the fair sex;

• rashes in the form of a ring on the body;

• inflammatory processes on the mucous membranes;

• damage to the heart and liver, as well as the brain;
• noticeable pain in the muscles;
• sensitivity of the limbs to temperature changes.

If timely measures of therapy are not taken, the general mechanism of the functioning of the body will come to a violation, which will provoke many problems.

Is lupus a disease contagious or not?

Many are interested in the question is lupus contagious? The answer is negative, since the formation of the disease occurs exclusively inside the body and does not depend on whether the sick person has contacted people infected with lupus or not.

What to get tested for lupus erythematosus

The main analyzes are ANA and complement, as well as general analysis blood fluid.

1. Blood donation will help determine the presence of a enzyme element, so it must be taken into account for the initial and subsequent assessment. In 10% of situations, anemia can be detected, indicating a chronic course of the process. ESR indicator is of great importance in illness.
2. Analysis of ANA and complement will reveal serological parameters. Identification of ANA acts as key moment, since the diagnosis is often differentiated from diseases of an autoimmune nature. In many laboratories, the content of C3 and C4 is determined, since these elements are stable and do not need to be processed.
3. Experimental analyzes are done in order to identify and form specific (specific) markers in the urine, so that it becomes possible to determine the disease. They are needed to form a picture of the disease and make decisions on treatment.

How to take this analysis, you need to ask your doctor. Traditionally, the process occurs, as with other suspicions.

Lupus erythematosus treatment

The use of medicines

The disease involves taking medications to increase immunity and generally improve the quality parameters of cells. A complex of drugs or medications is prescribed separately to eliminate the symptoms and treat the causes of the formation of the disease.

Treatment of systemic lupus carried out by the following methods.

• With minor manifestations of the disease and the need to eliminate symptoms, the specialist prescribes glucocorticosteroids. The most successfully used drug is prednisolone.
• Immunosuppressants of a cytostatic nature are relevant if the situation is aggravated by the presence of other symptomatic factors. The patient must drink azathioprine, cyclophosphamide.
• The most promising action, aimed at suppressing symptoms and consequences, have blockers, which include infliximab, etanercept, adalimumab.
• The means of extracorporeal detoxification have proven themselves widely and relevantly - hemosorption, plasmapheresis.

If the disease is characterized by the simplicity of the form in which a traditional skin lesion occurs (or), it is enough to use a simple set of drugs that help eliminate ultraviolet radiation. If cases are started, funds are accepted hormone therapy and immunosuppressive drugs. Due to the presence of acute contraindications and side effects, they are prescribed by a physician. If the cases are especially severe, therapy is prescribed with cortisone.

Therapy with folk remedies

Treatment folk remedies also true for many sick people.

1. Decoctions of mistletoe from birch prepared by using washed and dried leaves collected in winter. Raw materials, previously brought to a fine state, are poured into glass containers and stored in a dark place. In order for the broth to be prepared with high quality, you need to dial 2 tsp. collection and pour boiling water in the amount of 1 cup. Cooking will take 1 minute, insisting will drag on for 30 minutes. Having expressed the finished composition, you need to divide it into 3 doses and drink everything in a day.
2. Willow root decoction suitable for use by children and adults. The main criterion is the young age of the plant. The washed roots should be dried in the oven, chopped. Raw materials for cooking will require 1 cl. l., the amount of boiling water - a glass. Steaming lasts for a minute, the infusion process - 8 hours. Having expressed the composition, you need to take it in 2 tablespoons, the interval is equal time intervals for 29 days.
3. Therapy tarragon ointment. For cooking, you need fresh fat, melted in a water bath, tarragon is added to it. Mixing of components is carried out in a ratio of 5:1, respectively. In the oven, all this must be kept for about 5-6 hours at low temperature conditions. After filtering and cooling, the mixture is sent to the refrigerator and can be used for 2-3 months to lubricate the lesions 2-3 times a day.

By using the tool correctly, lupus disease can be eliminated within a short period of time.

Complications of the disease

In different individuals, the disease proceeds in a special way, and the complexity always depends on the severity and on the organs affected by the disease. Most often, the legs, as well as rashes on the face, are not the only symptoms. Usually the disease localizes its damage in the kidney area, sometimes you have to take the patient to dialysis. Other common consequences are vascular and heart disease. which can be seen in the material, is not the only manifestation, since the disease has a deeper nature.

Forecast values

After 10 years after diagnosis, the survival rate is 80%, and after 20 years this figure drops to 60%. Common causes of death are factors such as lupus nephritis, infectious processes.

Have you experienced illness lupus? Was the disease cured and overcome? Share your experience and opinion on the forum for everyone!

One of the most serious diseases is systemic lupus erythematosus (SLE). It is characterized by autoimmune inflammation with many other symptoms. This disease is dangerous for its complications. With it, organs of many body systems suffer, but most of all problems occur with the musculoskeletal system and kidneys.

Description of the disease

Lupus develops due to a malfunction of the immune system, in which antibodies are formed that adversely affect healthy cells and tissues. This leads to negative changes in the vessels and connective tissue.

The term "lupus" was once used to refer to red spots that appear on the face. They resembled the bites of wolves or she-wolves, which often attacked people and sought to get to unprotected parts of the body, like the nose or cheeks. Even one of the symptoms of the disease is called "lupus butterfly". Today, the name is associated with the cute word "wolf cub".

Autoimmune disease develops against the background of hormonal disorders. Increased estrogens play an important role, so lupus is most often observed in the fair sex. The disease is usually diagnosed in adolescent girls and young women under 26 years of age.

In men, SLE is more severe, and remissions are rare, but in them the disease occurs 10 times less often, since androgens have a protective effect. Some symptoms may be more pronounced in different sexes. For example, in women, the joints are more affected, and in men, the central nervous system and kidneys.

Lupus can be congenital. Symptoms of SLE appear in children in the first years of life.

The disease develops in waves, with periods of exacerbations and remissions alternating. SLE is characterized by an acute onset, rapid progression, and early spread of the disease process. In children, the symptoms of systemic lupus erythematosus are the same as in adults.

Causes

There is more than one reason for the occurrence and development of lupus. It is caused by the simultaneous or sequential influence of several factors at once. Scientists have been able to identify the main causes of the disease:

Scientists do not include the last factor in common causes the occurrence of SLE, but they believe that the relatives of the patient are at risk.

Classification by stages

SLE has a wide range of symptoms. During the illness, exacerbations and remissions occur.

Lupus is classified according to the forms of its course:

The stages of the disease are also distinguished. The minimum is characterized by weak headaches and joint pains, high fever, malaise and the first manifestations of lupus on the skin.

In the moderate phase, the face and body are severely affected, and then the vessels, joints and internal organs. At a pronounced stage, the work of various body systems is disrupted.

Symptoms of the disease

At the onset of SLE, only 20% of patients are bothered by skin lesions. In 60% of patients, symptoms appear later. Some people don't have them at all. Signs of the disease can be seen on the face, neck and shoulders. A rash appears on the back of the nose and cheeks in the form of reddish plaques with peeling, reminiscent of wolf bites in the past. It is called the "lupus butterfly" because it looks like this insect. The patient's skin sensitivity to ultraviolet radiation increases.

Some people with lupus lose their hair in the temple areas and break their nails. Mucous membranes are affected in 25% of cases. Lupus-cheilitis appears, characterized by dense swelling of the lips in the form of grayish scales. On the border, small ulcers of red or pink color may appear. In addition, the oral mucosa is affected.

Lupus affects various body systems:

Common symptoms of lupus in women and men are CNS lesions. The disease is characterized by fatigue, weakness, decreased memory and performance, deterioration of intellectual abilities. A person suffering from an autoimmune disease manifests irritability, depression, headaches, and.

The patient may be less sensitive. Seizures, psychosis and convulsions also develop against the background of lupus.

Diagnostic methods

The diagnosis of lupus can be confirmed by differential diagnosis. It is done because each manifestation speaks of the pathology of a particular organ. For this, a system developed by the American Rheumatological Association of Specialists is used.

The diagnosis of SLE is confirmed with four or more of the following:

After a preliminary diagnosis is made, the patient is referred to a specialist with a narrow focus, for example, to a nephrologist, pulmonologist or cardiologist.

A detailed examination includes a thorough history taking. The doctor needs to learn about all the patient's previous diseases and methods of their treatment.

Methods of treatment

Drug therapy for a patient with SLE is selected individually. Methods of treatment depend on the stage and form of the disease, the symptoms and characteristics of the patient's body.

It will be necessary to hospitalize a person suffering from lupus only in certain cases: a constant temperature above 38 degrees, a decrease, as well as if a stroke, heart attack, or severe CNS damage is suspected. If the clinical signs of the disease progress, then the patient will also be referred for hospitalization.

Treatment of lupus erythematosus includes:

Hormonal creams and ointments eliminate peeling and burning sensations that have arisen in certain areas of the skin.

Particular attention is paid to the patient's immune system. During remission, the patient is treated with immunostimulants in combination with vitamin complexes and physiotherapy procedures.

Comorbidities and complications are also taken into account. Since kidney problems occupy the first place in cases of lethality, they must be constantly monitored in SLE. It is necessary to treat lupus arthritis and heart disease in a timely manner.

Dandelion P acts as a natural chondroprotector, which prevents the joints from collapsing and restores cartilage tissue. It lowers blood cholesterol levels and cleanses the body of toxins. Dihydroquercetin Plus is used to strengthen the walls of blood vessels. It also eliminates bad cholesterol and improves blood microcirculation.

With lupus, people are prescribed foods that will ease the symptoms of the disease. The patient should give preference to food that can protect the brain, heart and kidneys.

A person diagnosed with lupus must be consumed in sufficient quantities:

Protein will help fight the disease. Doctors recommend eating veal, turkey and other dietary meats and poultry. The diet should include cod, pollock, pink salmon, tuna, squid, low-fat herring. Fish contains omega-3 unsaturated fatty acids, vital for normal operation brain and heart.

Drink at least 8 glasses of pure water per day. She gets the job done gastrointestinal tract, controls hunger and improves general state.

Some foods will need to be abandoned or limited in your diet:

• Fatty meals. Foods that contain a lot of butter or vegetable oil increase the risk of exacerbations from of cardio-vascular system. Due to fatty foods, cholesterol is deposited in the vessels. Because of it, it can develop acute infarction myocardium.
• Caffeine. This component in in large numbers present in coffee, tea and some other drinks. Because of caffeine, the mucous membrane of the stomach is irritated, the heart beats more often and the nervous system is overloaded. If you stop drinking cups of drinks with this substance, it will be possible to avoid the occurrence of erosions in the duodenum.
• Salt. food product should be limited as it overloads the kidneys and increases blood pressure.

People suffering from lupus erythematosus should avoid alcoholic beverages and cigarettes. They are already harmful on their own, and in combination with drugs can lead to disastrous consequences.

Patient Predictions

The prognosis will be favorable if the disease is detected at an early stage of its development. At the very beginning of the course of lupus, tissues and organs do not undergo severe deformation. A mild rash or arthritis is easily controlled by specialists.

At running forms SLE will require aggressive treatment with high doses of various medications. In this case, it is not always possible to determine what causes more harm to the body: large dosages of drugs or the pathological process itself.

Lupus erythematosus cannot be completely cured, but this does not mean that one cannot live happily with it. If timely apply for medical care, serious problems can be avoided. While doing medical advice and the right way of life, the patient will not have to limit himself in many ways.

Complication and progression of the disease is possible if a person has chronic infectious diseases. Frequent vaccinations and colds also have an impact. Therefore, such a patient should take care of his health and avoid factors that adversely affect his body.

Preventive actions

Prevention of SLE will help prevent recurrence of the disease and stop the further progression of pathological processes. Secondary measures contribute to the timely and adequate treatment of lupus.

Patients should regularly undergo dispensary examinations and consult a rheumatologist. Drugs must be taken in the prescribed dosage for a certain period of time.

The state of stable remission can be maintained with hardening, therapeutic gymnastics and regular walks in the fresh air. The patient must observe the regime of work and rest, avoiding unnecessary psychological and physical stress. Good sleep and proper nutrition are important not only for improving the state of illness, but also for the normal functioning of the whole organism.

If isolated areas of affected skin have been identified in a person, it is necessary to find out if any of his relatives have been diagnosed with the disease. A person with lupus should avoid ultraviolet light and stay away from direct sunlight. In the warm season, you need to use special ointments that can protect the skin from the negative effects of the sun. A person suffering from SLE must give up bad habits that only aggravate his condition.

Lupus erythematosus is an autoimmune pathology in which damage occurs blood vessels and connective tissue, and as a result, human skin. The disease wears systemic character, i.e. there is a disturbance in several body systems, providing Negative influence on it in general and on individual organs in particular, including the immune system.

The susceptibility of women to the disease is several times higher than that of men, which is associated with the peculiarities of the structure of the female body. The most critical age for the development of systemic lupus erythematosus (SLE) is considered to be puberty, during pregnancy and some interval after it, while the body goes through the recovery phase.

In addition, a separate category for the occurrence of pathology is considered to be children's age in the period of 8 years, but this is not a determining parameter, because the congenital type of the disease or its manifestation in the early stages of life is not excluded.

What is this disease?

Systemic lupus erythematosus (SLE, Liebman-Sachs disease) (Latin lupus erythematodes, English systemic lupus erythematosus) is a diffuse connective tissue disease characterized by systemic immunocomplex lesions of the connective tissue and its derivatives, with damage to the vessels of the microvasculature.

A systemic autoimmune disease in which antibodies produced by the human immune system damage the DNA of healthy cells, predominantly connective tissue is damaged with the obligatory presence of a vascular component. The disease got its name because of its characteristic feature - a rash on the bridge of the nose and cheeks (the affected area resembles a butterfly in shape), which, as they believed in the Middle Ages, resembles the places of wolf bites.

Story

Lupus erythematosus got its name from the Latin word "lupus" - wolf and "erythematosus" - red. This name was given because of the similarity of skin signs with lesions after being bitten by a hungry wolf.

The history of lupus erythematosus began in 1828. This happened after the French dermatologist Biett first described skin symptoms. Much later, after 45 years, the dermatologist Kaposhi noticed that some of the sick, along with skin symptoms, have diseases of the internal organs.

In 1890 It was discovered by the English physician Osler that systemic lupus erythematosus can occur without skin manifestations. The description of the phenomenon of LE- (LE) cells is the detection of cell fragments in the blood, in 1948. made it possible to identify patients.

In 1954 certain proteins were found in the blood of the diseased - antibodies that act against their own cells. This detection has been used in the development of sensitive tests for diagnosing systemic lupus erythematosus.

Causes

The causes of the disease have not been fully elucidated. Only putative factors contributing to the occurrence of pathological changes have been established.

Genetic mutations - a group of genes associated with specific immune disorders and predisposition to systemic lupus erythematosus has been identified. They are responsible for the process of apoptosis (getting the body rid of dangerous cells). When potential pests are delayed, healthy cells and tissues are damaged. Another way is the disorganization of the immune defense management process. The reaction of phagocytes becomes excessively strong, does not stop with the destruction of foreign agents, their own cells are taken for "aliens".

1. Age - the most systemic lupus erythematosus affects people from 15 to 45 years old, but there are cases that have arisen in childhood and in the elderly.
2. Heredity - cases of a family disease are known, probably transmitted from older generations. However, the risk of having an affected child remains low.
3. Race - American studies have shown that blacks are sick 3 times more often than whites, and this reason is more pronounced in native Indians, natives of Mexico, Asians, Spaniards.
4. Gender - among the known sick women are 10 times more than men, so scientists are trying to establish a connection with sex hormones.

Among external factors the most pathogenic is intense solar radiation. Passion for sunburn can provoke genetic changes. There is an opinion that people who are professionally dependent on activities in the sun, frost, and sharp fluctuations in environmental temperature (sailors, fishermen, agricultural workers, builders) are more likely to suffer from systemic lupus.

In a significant proportion of patients, clinical signs of systemic lupus appear during hormonal changes, against the background of pregnancy, menopause, taking hormonal contraceptives during a period of intense puberty.

The disease is also associated with the infection, although it is still impossible to prove the role and degree of influence of any pathogen (targeted work is underway on the role of viruses). Attempts to identify a link with immunodeficiency syndrome or establish the contagiousness of the disease have so far been unsuccessful.

Pathogenesis

How does systemic lupus erythematosus develop in a seemingly healthy person? Under the influence of certain factors and a reduced function of the immune system, a failure occurs in the body, in which antibodies begin to be produced against the "native" cells of the body. That is, tissues and organs begin to be perceived by the body as foreign objects and a program of self-destruction is launched.

This reaction of the body is pathogenic, provoking the development inflammatory process and inhibition of healthy cells in many ways. Most often, blood vessels and connective tissue are affected. The pathological process leads to a violation of the integrity of the skin, changes in its appearance and reduced blood circulation in the lesion. With the progression of the disease, the internal organs and systems of the whole organism are affected.

Classification

Depending on the area of ​​the lesion and the nature of the course, the disease is classified into several types:

1. Lupus erythematosus caused by taking certain drugs. Leads to the appearance of symptoms of SLE, which may spontaneously disappear after discontinuation of drugs. Drugs that can lead to the development of lupus erythematosus are drugs for the treatment of arterial hypotension (arteriolar vasodilators), antiarrhythmic, anticonvulsants.
2. Systemic lupus erythematosus. The disease is prone to rapid progression with damage to any organ or body system. It proceeds with fever, malaise, migraines, rashes on the face and body, as well as pains of a different nature in any part of the body. The most characteristic migraine, arthralgia, pain in the kidneys.
3. neonatal lupus. Occurs in newborns, often combined with heart defects, serious disorders of the immune and circulatory systems, anomalies in the development of the liver. The disease is extremely rare; conservative therapy measures can effectively reduce the manifestations of neonatal lupus.
4. Discoid lupus. The most common form of the disease is Biett's centrifugal erythema, the main manifestations of which are skin symptoms: a red rash, thickening of the epidermis, inflamed plaques that transform into scars. In some cases, the disease leads to damage to the mucous membranes of the mouth and nose. A variety of discoid is a deep Kaposi-Irgang lupus, which is characterized by a recurrent course and deep lesions of the skin. A feature of the course of this form of the disease are signs of arthritis, as well as a decrease in human performance.

Symptoms of lupus erythematosus

As a systemic disease, lupus erythematosus is characterized by the following symptoms:

• chronic fatigue syndrome;
• swelling and soreness of the joints, as well as muscle pain;
• unexplained fever;
• chest pain with deep breathing;
• increased hair loss;
• red, skin rashes on the face or discoloration of the skin;
• sensitivity to the sun;
• swelling, swelling of the legs, eyes;
• swollen lymph nodes;
• blue or whitening of the fingers, toes, being in the cold or at the time of stress (Raynaud's syndrome).

Some people experience headaches, convulsions, dizziness, depression.

New symptoms may appear years later and after diagnosis. In some patients, one system of the body suffers (joints or skin, hematopoietic organs), in other patients, the manifestations can affect many organs and be multi-organ in nature. The very severity and depth of damage to body systems is different for everyone. The muscles and joints are often affected, causing arthritis and myalgia (muscle pain). Skin rashes are similar in different patients.

If the patient has multiple organ manifestations, then the following pathological changes occur:

• inflammation in the kidneys (lupus nephritis);
• inflammation of the blood vessels (vasculitis);
• pneumonia: pleurisy, pneumonitis;
• heart disease: coronary vasculitis, myocarditis or endocarditis, pericarditis;
• blood diseases: leukopenia, anemia, thrombocytopenia, risk of blood clots;
• damage to the brain or central nervous system, and this provokes: psychosis (change in behavior), headache, dizziness, paralysis, memory impairment, vision problems, convulsions.

What does lupus erythematosus look like, photo

The photo below shows how the disease manifests itself in humans.

The manifestation of symptoms of this autoimmune disease can vary significantly in different patients. However, skin, joints (mainly of the hands and fingers), heart, lungs and bronchi, as well as digestive organs, nails and hair, which become more fragile and prone to falling out, as well as the brain and nervous system.

Stages of the course of the disease

Depending on the severity of the symptoms of the disease, systemic lupus erythematosus has several stages of the course:

1. Acute stage - at this stage of development, lupus erythematosus progresses sharply, the patient's general condition worsens, he complains of constant fatigue, fever up to 39-40 degrees, fever, pain and aching muscles. Clinical picture develops rapidly, already in 1 month the disease covers all organs and tissues of the body. The prognosis for acute lupus erythematosus is not comforting and often the patient's life expectancy does not exceed 2 years;
2. Subacute stage - the rate of progression of the disease and the severity of clinical symptoms are not the same as in acute stage and more than 1 year may elapse from the onset of illness to the onset of symptoms. At this stage, the disease is often replaced by periods of exacerbations and persistent remission, the prognosis is generally favorable and the patient's condition directly depends on the adequacy of the prescribed treatment;
3. Chronic form - the disease has a sluggish course, clinical symptoms weakly expressed, the internal organs are practically not affected and the body as a whole functions normally. Despite relatively easy current lupus erythematosus, it is impossible to cure the disease at this stage, the only thing that can be done is to alleviate the severity of the symptoms with the help of medications at the time of exacerbation.

Complications of SLE

The main complications that SLE provokes are:

1) Heart disease:

• pericarditis - inflammation of the heart bag;
• hardening of the coronary arteries that supply the heart due to the accumulation of thrombotic clots (atherosclerosis);
• endocarditis (infection of damaged heart valves) due to hardening of the heart valves, accumulation of blood clots. Often, valves are transplanted;
• myocarditis (inflammation of the heart muscle), causing severe arrhythmias, diseases of the heart muscle.

2) Renal pathologies(nephritis, nephrosis) develop in 25% of patients with SLE. The first symptoms are swelling in the legs, the presence of protein in the urine, blood. The failure of the kidneys to function normally is extremely life-threatening. Treatment includes strong drugs for SLE, dialysis, and a kidney transplant.

3) Blood diseases that are life-threatening.

• decrease in red blood cells (which supply cells with oxygen), white blood cells (suppress infection and inflammation), platelets (contribute to blood clotting);
• hemolytic anemia caused by a lack of red blood cells or platelets;
• pathological changes in the hematopoietic organs.

4) Diseases of the lungs (in 30%), pleurisy, inflammation of the muscles of the chest, joints, ligaments. The development of acute lupus erythematosus (inflammation of the lung tissue). Pulmonary embolism- blockage of arteries by emboli (blood clots) due to increased blood viscosity.

Diagnostics

The assumption of the presence of lupus erythematosus can be made on the basis of red foci of inflammation on the skin. External signs erythematoses can change over time, so it is difficult to make an accurate diagnosis. It is necessary to use a complex of additional examinations:

• general blood and urine tests;
• determination of the level of liver enzymes;
• analysis for antinuclear bodies (ANA);
• chest x-ray;
• echocardiography;
• biopsy.

Differential Diagnosis

Chronic lupus erythematosus is differentiated from lichen planus, tuberculous leukoplakia and lupus, early rheumatoid arthritis, Sjögren's syndrome (see dry mouth, dry eye syndrome, photophobia). With the defeat of the red border of the lips, chronic SLE is differentiated from the abrasive precancerous cheilitis of Manganotti and actinic cheilitis.

Since the defeat of internal organs is always similar in course to various infectious processes, SLE is differentiated from Lyme disease, syphilis, mononucleosis (infectious mononucleosis in children: symptoms), HIV infection.

Treatment of systemic lupus erythematosus

Treatment should be as appropriate as possible for the individual patient.

Hospitalization is necessary in the following cases:

• with a persistent increase in temperature for no apparent reason;
• in the event of life-threatening conditions: rapidly progressive kidney failure, acute pneumonitis or pulmonary hemorrhage.
• with neurological complications.
• with a significant decrease in the number of platelets, erythrocytes or blood lymphocytes.
• in the case when the exacerbation of SLE cannot be cured on an outpatient basis.

For the treatment of systemic lupus erythematosus during an exacerbation are widely used hormonal preparations(prednisolone) and cytostatics (cyclophosphamide) according to a certain scheme. In organ damage musculoskeletal system, as well as with an increase in temperature, non-steroidal anti-inflammatory drugs (diclofenac) are prescribed.

For adequate treatment of a disease of a particular organ, it is necessary to consult a specialist in this field.

Nutrition rules

Dangerous and harmful foods for lupus:

• a large amount of sugar;
• everything fried, fatty, salty, smoked, canned;
• products for which there are allergic reactions;
• sweet soda, energy drinks and alcoholic drinks;
• in the presence of problems with the kidneys, food containing potassium is contraindicated;
• canned food, sausages and sausages of factory preparation;
• store-bought mayonnaise, ketchup, sauces, dressings;
• confectionery with cream, condensed milk, with artificial fillers (factory jams, marmalade);
• fast food and products with non-natural fillers, dyes, rippers, taste and smell enhancers;
• foods containing cholesterol (buns, bread, red meat, high-fat dairy products, sauces, dressings and soups based on cream);
• products with a long shelf life (meaning those products that quickly deteriorate, but due to various chemical additives in the composition, they can be stored for a very long time- here, as an example, dairy products with an annual expiration date can be attributed).

Eating these foods can accelerate the progression of the disease, which can lead to death. These are the maximum consequences. And, at a minimum, the dormant stage of lupus will turn into an active one, because of which all the symptoms will worsen and the state of health will worsen significantly.

Lifespan

Survival rate 10 years after the diagnosis of systemic lupus erythematosus is 80%, after 20 years - 60%. Main causes of death: lupus nephritis, neuro-lupus, intercurrent infections. There are cases of survival of 25-30 years.

In general, the quality and duration of life in systemic lupus erythematosus depends on several factors:

1. Age of the patient: the younger the patient, the higher the activity of the autoimmune process and the more aggressive the disease, which is associated with a greater reactivity of immunity at a young age (more autoimmune antibodies destroy their own tissues).
2. Timeliness, regularity and adequacy of therapy: with long-term use hormones of glucocorticosteroids and other drugs can achieve a long period of remission, reduce the risk of complications and, as a result, improve the quality of life and its duration. Moreover, it is very important to start treatment even before the development of complications.
3. Variant of the course of the disease: the acute course is extremely unfavorable and after a couple of years severe, life threatening complications. And with a chronic course, and this is 90% of SLE cases, you can live until old age full life(if you follow all the recommendations of a rheumatologist and therapist).
4. Compliance with the regimen significantly improves the prognosis of the disease. To do this, you must constantly be observed by a doctor, follow his recommendations, consult a doctor in a timely manner if any symptoms of an exacerbation of the disease appear, avoid contact with sunlight, limit water procedures, lead a healthy lifestyle and follow other rules for preventing exacerbations.

Just because you've been diagnosed with lupus doesn't mean your life is over. Try to defeat the disease, maybe not in the literal sense. Yes, you will probably be limited in some way. But millions of people with more serious illnesses live bright, full of impressions life! So you can too.

Prevention

The purpose of prevention is to prevent the development of relapses, to maintain the patient in a state of stable remission for a long time. Prevention of lupus is based on an integrated approach:

1. Regular dispensary examinations and consultations with a rheumatologist.
2. Taking drugs strictly in the prescribed dose and at specified intervals.
3. Compliance with the regime of work and rest.
4. Full sleep, at least 8 hours a day.
5. Diet with limited salt and enough protein.
6. Hardening, walks, gymnastics.
7. The use of hormone-containing ointments (for example, Advantan) for skin lesions.
8. The use of sunscreen (creams).

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The onset of the disease is often characterized by weakness, weight loss, impaired trophism, and fever. Subsequently, a polysyndromic picture develops with typical features for each syndrome.

Skin damage in systemic lupus erythematosus (SLE) very diverse and often has a paramount diagnostic value. Only in 10-15% of patients, skin changes may be absent. In 20-25% of patients, skin syndrome is the initial sign of the disease, in 60-70% of patients it appears at different stages of the disease.

E. Dubois (1976) identifies up to 28 variants of skin changes in SLE: from erythematous patches to severe bullous eruptions. Skin lesions in SLE can be divided into specific and non-specific.

Typical for the skin form of lupus erythematosus, discoid foci with hyperemia, infiltration, follicular hyperkeratosis and cicatricial atrophy occur in 25% of cases, as a rule, with systemic lupus erythematosus of a chronic course.

Discoid lupus erythematosus is characterized by three cardinal clinical symptoms: erythema, hyperkeratosis and atrophy. The onset is characterized by the appearance of a small pink or red spot with clear boundaries, which gradually becomes covered in the center with dense grayish-white dry scales. The scales are firmly held due to the presence of spike-like protrusions on their lower surface, immersed in the expanded follicular orifices (follicular hyperkeratosis). Characterized by the appearance of pain when removing scales (symptom of Besnier-Meshchersky).

Gradually, cicatricial atrophy begins to appear in the center of the focus and the focus takes on a pathognomonic appearance for discoid lupus: in the center there is a smooth, delicate white atrophic scar, further to the periphery there is a zone of hyperkeratosis and infiltration, and outside there is a corolla of hyperemia. Localization is typical in open areas of the skin: face (especially on the nose and cheeks with the formation of a butterfly figure), auricles, neck. Often the scalp and the red border of the lips are affected (Fig. 4.2). Perhaps the location of lesions on the mucous membrane of the oral cavity, where they can ulcerate.

With central Biett's erythema (a superficial form of lupus erythematosus), of the three main skin symptoms, only hyperemia is clearly expressed, while scales and cicatricial atrophy are absent. The lesions are usually located on the face and often mimic the figure of a butterfly (Fig. 4.3).

Multiple foci of discoid lupus or Biett's centrifugal erythema, scattered over various parts of the skin, characterize disseminated lupus erythematosus.

Rashes on the skin are usually not accompanied by any subjective sensations, however, erosive lesions on the oral mucosa are painful when eating. The cutaneous form of lupus erythematosus is characterized by a long-term continuous course with deterioration in spring and summer due to photosensitivity. Among the rare skin forms, deep Kaposi-Irgang lupus erythematosus is distinguished, where, along with the usual foci, there is one or more sharply demarcated dense mobile nodes covered with normal skin. Sometimes these nodes turn into typical lupus erythematosus lesions.

Rice. 4.2. Discoid-type foci in combination with lupuscheilitis, broken "columns" of hair in a patient with chronic systemic lupus erythematosus

Rice. 4.3. Butterfly type centrifugal Biett's erythema in chronic systemic lupus erythematosus

Most common skin lesions in SLE- isolated or confluent erythematous spots of various shapes and sizes, edematous, sharply demarcated from the surrounding healthy skin. They are identical to the superficial cutaneous form of lupus erythematosus and are usually observed on the face, neck, chest, elbows, knees and ankles. The location of such lesions on the nose and cheeks with the formation of a “butterfly” figure (“lupus butterfly”) is considered pathognomonic.

Less often, a vascular "butterfly" is observed in the form of an unstable, pulsating diffuse redness with a cyanotic tinge in the middle zone of the face, which is aggravated by exposure to insolation, wind, frost, or excitement (Fig. 4.4). It is almost indistinguishable from febrile erythema of the face. Sometimes the "butterfly" looks like a persistent erysipelas with severe swelling of the face, especially of the eyelids. Skin lesions with an abundance of erythematous, sharply edematous ring-shaped rashes can simulate multiforme exudative erythema. This pathology is known as Rowell's syndrome.

Other skin manifestations of SLE include lupus cheilitis(congestive hyperemia with dense dry grayish scales, sometimes crusts and erosions, resulting in atrophy on the red border of the lips), the so-called capillaritis (edematous erythema with telangiectasias and atrophy on the fingertips, palms and soles of the feet and enanthema - erythematous areas with hemorrhagic inclusions and erosion on the oral mucosa.

Rice. 4.4. Vasculitic "butterfly" in a patient with acute course systemic lupus erythematosus

Rarer lesions include: pernial foci (lupus-chill), bullous, nodular, urticarial, hemorrhagic and papulonecrotic rashes, reticular and branched livedo with ulceration and other forms of vasculitis.

In addition, patients with systemic lupus erythematosus often have trophic disorders: general dry skin, diffuse hair loss, deformation and brittle nails.

It is advisable to take a closer look at the skin manifestations that are included in the diagnostic criteria of the American Rheumatological Association. Alopecia refers to non-specific skin signs of SLE, but is the most common among them, occurs in 50% of SLE patients, and not only the hair of the head, but also the eyebrows, eyelashes, etc. are affected. There are cicatricial and non-cicatricial alopecia.

Cicatricial alopecia is characteristic of chronic systemic lupus erythematosus and usually develops at the site of discoid lesions. Non-scarring alopecia is manifested by diffuse thinning of the hair and is usually observed during a severe exacerbation of SLE. Diffuse forms of alopecia are usually reversible. The "columns" formed from broken hair along the edge of the growth zone are pathognomonic signs of acute or subacute SLE. With adequate therapy, normal hair is restored.

Urticaria-like lesions in systemic lupus erythematosus are never seen in pure cutaneous lupus erythematosus and represent urticarial vasculitis. Unlike ordinary urticaria, wheals exist for more than 24 hours. Severe visceral changes are usually not observed in this group of patients.

photosensitization- frequent and important feature lupus erythematosus, observed in 30-60% of patients with cutaneous form and SLE, is one of the diagnostic criteria RA. Characterized by localization mainly in open areas of the skin. Special studies in the experiment have shown that patients are sensitive to both the A- and B-zones. ultraviolet rays, the fact of detection of antibodies to DNA denatured by ultraviolet light in patients with SLE, and the absence of such antibodies in cutaneous lupus and other photodermatosis, was also confirmed.

Mucosal involvement is also included in the criteria of the American Rheumatological Association. On the mucous membrane of the nose and mouth, there may be whitish plaques of irregular outlines or silvery-white cicatricial foci. Often observed erosive and / or ulcerative foci with a whitish keratotic rim and intense erythema. Possible perforation of the nasal septum due to vasculitis. Immunofluorescent examination of a biopsy from the lesion usually reveals deposits of immunoglobulins and / or complement in the dermoepidermal junction, and sometimes in the vessel wall. Histological examination reveals classic leukocytoclastic angiitis.

Telangiectasias- a frequent symptom in all diffuse diseases of the connective tissue. Three types of telangiectasias have been described in systemic lupus erythematosus: 1) small linear telangiectasias on the posterior ridge of the nail bed and on areas of the presenting skin; 2) irregular shape, twisted at the fingertips; 3) in the form of scattered spots on the palms and fingers. Histologically, telangiectasias are only vasodilation without signs of inflammation.

AT last years a significant number of works have appeared on subacute cutaneous lupus erythematosus, which was described by R. Sontheimer in 1979, giving it the name Subacute Cutaneus Lupus Erythematosus (SCLE). Clinical symptoms of skin lesions in SCLE are characterized by widespread annular lesions forming polycyclic areas on the face, chest, neck, and extremities. In the center of the focus - telangiectasia, hypopigmentation. There are no scars left.

Sometimes the rash can be papulosquamous, resembling lesions in psoriasis. Usually, the systemic manifestations of the disease are not so pronounced and are characterized by a musculoskeletal syndrome, approximately 50% of patients meet the criteria of the American Rheumatological Association. However, there are quite a few descriptions severe forms involving the central nervous system (20%), kidneys (10%). Via immunological research in 70% of patients, specific antibodies to the Ro (SSA) antigen were detected; later, a statistically significant association of SCLE with HLADR3 and B8 was established.

Mention should also be made of the characteristic eruptions in neonatal lupus ( neonatal lupus erythematosus). This is a very rare syndrome. T. Zizic (1983) believes that no more than 100 cases are described in the literature, however, it is necessary to know about this form. The newborn may have classic discoid erythema annulare, telangiectasias, skin atrophy, follicular plugs, and scales. Changes disappear within the first 6 months of life, sometimes leaving cicatricial atrophy, persistent hyper or hypopigmentation.

Such a skin lesion is usually combined with partial or complete heart block due to fibrosis of its pathways, which is often the cause lethal outcome newborn. Of the systemic signs, hepatosplenomegaly, Coombs-positive hemolytic anemia, antibodies to La (SSB) and / or Ro (SSA) antigens and RNA are observed. Antinuclear factor and LE cells are often absent.

Immunological changes usually also disappear within 6 months, sometimes they are the only sign of neonatal lupus erythematosus. Approximately 20% of mothers who give birth to such children subsequently develop lupus erythematosus or so-called incomplete lupus erythematosus, but most of them remain clinically asymptomatic throughout their subsequent life, while the above antibodies can be detected in the blood serum.

There are conflicting views as to whether SLE and discoid lupus erythematosus are variants of the same disease.

Their similarity is determined by the following provisions: 1) skin manifestations in SLE and discoid lupus erythematosus may be clinically and pathologically indistinguishable; 2) certain clinical symptoms are found in both diseases; 3) similar hematological, biochemical and immunological disorders can occur in both diseases; 4) discoid lupus erythematosus sometimes turns into systemic lupus erythematosus (3-12%); 5) in patients with SLE, typical discoid foci appear when the acute phase of the disease subsides.

At the same time, some facts require explanation: 1) a relatively small percentage of the transformation of the discoid form into the system; 2) the presence of laboratory changes in discoid lupus erythematosus is not an indication of a predisposition to the transition to SLE (hematological disorders were noted in 50% of 77 patients with discoid lupus, but after 5 years of observation they did not develop systemic lupus erythematosus); 3) complement deposits are detected in unaffected skin in SLE and are not detected in discoid lupus; 4) the majority of patients with discoid lupus without complications endure physical trauma, ultraviolet radiation, stress, they do not develop systemic manifestations; 5) the age and sex ratio in the occurrence of SLE is significantly different from that in discoid lupus erythematosus.

N. Rowell (1988) gives a comparative frequency of some clinical and laboratory signs with discoid lupus erythematosus and systemic lupus erythematosus (Table 4.1).

Discoid lupus erythematosus, like SLE, is believed to result from a somatic mutation of the lymphocyte population in susceptible individuals, but there is a difference in their genetic condition. Thus it is independent diseases rather than variants of a single disease. At the same time, both of these nosological forms have several subtypes, also genetically determined.

The question of the possibility and frequency of transformation of discoid lupus into SLE remains not fully clarified. It is believed that in the presence of a genotype characteristic only for discoid lupus, there is never a transition to systemic lupus erythematosus, even under the influence of various external and stress factors. However, if HLA-B8 is detected in patients with discoid lupus, there is a high risk of developing SLE, especially at the age of 15-40 years.

Table 4.1. The frequency of clinical and laboratory data in groups of patients with discoid lupus erythematosus and systemic lupus erythematosus,%

Indicator	DHQ (n = 120)	SLE (n = 40)
Skin rashes	100	80
Joint pain	23	70
Increase in body temperature	0	40
Raynaud's syndrome	14	35
"Oznobyshi"	22	22
ESR > 20 mm/h	20	85
Serum y-globulin more than 30 g/l	29	76
LE cells	1,7	83
antinuclear factor	35	87
glow homogeneous	24	74
» mottled	11	26
» nucleolar	0	5,4
precipitating autoantibodies	4	42
Positive Wasserman reaction	5	22
Positive RF	15	37
Positive direct Coombs reaction	2,5	15
Leukopenia	12,5	37
Thrombocytopenia	5	21

Damage to the joints and periarticular tissues

Arthralgias occur in almost 100% of patients. Pain in one or more joints can last from a few minutes to several days. With a high activity of the disease, pain can be more persistent, with the development of inflammation, most often in the proximal interphalangeal joints of the hands, metacarpophalangeal, carpometacarpal, knee joints, and other joints may be affected. The process is usually symmetrical.

Morning stiffness and dysfunction of the joints in the acute phase of the disease are significantly pronounced, but quickly decrease with a decrease in the activity of the process under the influence of adequate therapy. The composition of synovial fluid in acute and subacute arthritis in patients with systemic lupus erythematosus significantly differs from that in RA. Synovial fluid is usually clear, viscous, with a small number of leukocytes and a predominance of mononuclear cells.

In the articular syndrome, damage to the ligamentous apparatus should also be included.- tendonitis, tendovaginitis, often causing transient flexion contractures of the fingers in SLE. In the chronic course of SLE with a predominant lesion of the joints and periarticular tissues, flexion contractures become irreversible and can cause dysfunction of the hand. 5% of the patients observed by us had fibrosing tendinitis with pronounced contractures. Along with fibrosis of some tendons, their strength is significantly reduced.

We observed several cases of rupture of the calcaneal tendon, avulsion of the patella. Significant damage to the periarticular soft tissues leads, with a long chronic course of arthritis, to the formation of a rheumatoid-like hand (Fig. 4.10). X-ray examination reveals erosions only in 1-5% of cases, and they are not as pronounced as in RA. Our observations revealed rheumatoid-like lesions of the hand in 20% of patients with systemic lupus erythematosus with chronic arthritis. In table. 4.2 shows the differences between chronic polyarthritis in SLE and RA.

In SLE, there are aseptic bone necrosis. The head is most commonly affected. femur, according to our observations up to 25%. However, the head of the humerus may be involved, as was the case in our observation (Fig. 4.11) in a man who fell ill with systemic lupus erythematosus at the age of 40 with the development of aseptic necrosis already 6 months after the onset of the disease. Multiple aseptic necrosis is possible with damage to the bones of the wrist, knee joint, and foot. The formation of aseptic bone necrosis can be due to both high disease activity and massive corticosteroid therapy.

Myalgia is observed in 35-45% of patients, but signs of focal myositis are quite rare. In some patients, pronounced muscle weakness requires differentiation from dermatomyositis. In myasthenic syndrome associated with SLE, as a rule, the activity of ALT, AST, and creatine phosphokinase is not increased. Biopsy reveals perivascular infiltrates, vacuolization of muscle fibers, and/or muscle atrophy. Muscle damage in SLE in some cases practically does not differ from that in classical dermatomyositis.

Table 4.2. Differences between chronic polyarthritis in systemic lupus erythematosus and rheumatoid arthritis

signs	Systemic lupus erythematosus	Rheumatoid arthritis
The nature of the damage to the joints	migratory	Progressive
morning stiffness	Uncharacteristic	Expressed
Transient flexion contractures	Characteristic	Uncharacteristic
Joint deformity	Minimum late	Significant
Mechanism of development of deformations	Predominant lesion	Articular destruction
	tendon-ligament apparatus and muscles	surfaces
Impaired function	Minor	Significant
bone erosion	Uncharacteristic	typical
ankylosis	uncharacteristic	Characteristic
Morphological picture	subacute synovitis with	Chronic hyperplastic
	nuclear pathology	synovitis with pannus formation
Rheumatoid factor	Fickle, at low	Persistent, in high credits
	titers in 5-25% of patients	in 80% of patients
Positive LE cell test	In 86% of patients	In 5-15% of patients

Rice. 4.10. Rheumatoid-like hand (Jaccous syndrome) in chronic systemic lupus erythematosus

Lung injury

In 50-80% of cases with SLE, there is dry or effusion pleurisy. Patients are concerned about pain in chest, slight dry cough, shortness of breath. With a small amount of effusion, pleurisy can proceed unnoticed, and only an X-ray examination reveals a thickening of the pleura or fluid in the pleural cavities, usually on both sides, and an elevation of the diaphragm. There is also a rather massive effusion, reaching 1.5-2 liters. Cases of SLE are described, when the effusion on both sides reached the third rib and, according to vital indications, repeated punctures had to be performed.

Inadequate treatment usually leads to the formation of massive adhesions and obliteration of the pleural cavities, which further sharply reduces the vital capacity of the lungs. Due to massive adhesions, the diaphragm is deformed, its tone decreases, it is pulled up with the formation of a high standing on both sides, but more often on the right. Pleurisy in lupus is an important diagnostic feature, as is a tightened diaphragm. The effusion may show LE cells, low complement, and high immunoglobulins.

The composition of the effusion is an exudate containing more than 3% protein, 0.55% glucose. In pathological anatomical examination, almost all patients have signs of adhesive pleurisy, a significant thickening of the pleura. Microscopically in the pleura reveal accumulations of macrophages and lymphocytes. In some cases, perivascular fibrinoid necrosis with neutrophilic and mononuclear infiltration is possible.

Rice. 4.11. Aseptic necrosis of the head of the humerus

Sigidin Ya.A., Guseva N.G., Ivanova M.M.