The susceptibility of women to the disease is several times higher than that of men, which is associated with structural features female body. The most critical age for the development of systemic lupus erythematosus (SLE) is considered to be puberty, during pregnancy and a certain 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 over 8 years of age, but this is not a determining parameter, because a congenital type of the disease or its manifestation in early life cannot be excluded.
What kind of disease is this?
Systemic lupus erythematosus (SLE, Libman-Sachs disease) (Latin lupus erythematodes, English systemic lupus erythematosus) is a diffuse connective tissue disease characterized by systemic immunocomplex damage to the connective tissue and its derivatives, with damage to the microvasculature.
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 is shaped like a butterfly), which was believed in the Middle Ages to resemble wolf bites.
Story
Lupus erythematosus gets its name from the Latin words “lupus” - wolf and “erythematosus” - red. This name was given due to the similarity of skin signs with damage after a bite from a hungry wolf.
History of the disease lupus erythematosus began in 1828. This happened after the French dermatologist Biett first described the skin signs. Much later, 45 years later, dermatologist Kaposhi noticed that some patients, along with skin symptoms, have diseases of internal organs.
In 1890 It was discovered by the English doctor Osler that systemic lupus erythematosus can occur without skin manifestations. 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 sick - antibodies that act against their own cells. This finding 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 identified.
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 rid of dangerous cells from the body). When potential pests are delayed, healthy cells and tissues are damaged. Another way is to disorganize the process of managing immune defense. The reaction of phagocytes becomes excessively strong, does not stop with the destruction of foreign agents, and their own cells are mistaken for “strangers.”
- Age - systemic lupus erythematosus affects people from 15 to 45 years of age, but there are cases that occur in childhood and in the elderly.
- Heredity - there are known cases of familial disease, probably transmitted from older generations. However, the risk of having a sick child remains low.
- Race - American studies have shown that the black population gets sick 3 times more often than whites, and this cause is also more pronounced among indigenous Indians, natives of Mexico, Asians, and Spanish women.
- Gender - There are 10 times more women than men among known patients, so scientists are trying to establish a connection with sex hormones.
Among external factors, the most pathogenic is intense solar radiation. Tanning can cause genetic changes. There is an opinion that people who are professionally dependent on activities in the sun, cold, sharp fluctuations environmental temperatures (sailors, fishermen, agricultural workers, builders).
In a significant proportion of patients, clinical signs of systemic lupus appear during the period of hormonal changes, against the background of pregnancy, menopause, or taking hormonal contraceptives, during the period of intense puberty.
The disease is also associated with a past infection, although it is not yet possible to prove the role and degree of influence of any pathogen (targeted work is underway on the role of viruses). Attempts to identify a connection with immunodeficiency syndrome or to 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 reduced function immune system a malfunction occurs in the body in which antibodies against the “native” cells of the body begin to be produced. That is, tissues and organs begin to be perceived by the body as foreign objects and a self-destruction program is launched.
This reaction of the body is pathogenic in nature, provoking the development of an inflammatory process and inhibition of healthy cells in different ways. Most often, blood vessels and connective tissue are affected by changes. The pathological process leads to a violation of the integrity of the skin, a change in its appearance and a decrease in blood circulation in the lesion. As the disease progresses, internal organs and systems of the entire body are affected.
Classification
Depending on the area affected and the nature of the course, the disease is classified into several types:
- Lupus erythematosus caused by taking certain drugs. Leads to the appearance of SLE symptoms, which may spontaneously disappear after discontinuation of the drugs. Medicines that can lead to the development of lupus erythematosus are becoming drugs for the treatment of arterial hypotension(arteriolar vasodilators), antiarrhythmics, anticonvulsants.
- Systemic lupus erythematosus. The disease tends to progress rapidly, affecting any organ or system of the body. Presents with fever, malaise, migraines, rashes on the face and body, and pain of different nature in any part of the body. The most common symptoms are migraines, arthralgia, and kidney pain.
- Neonatal lupus. Occurs in newborns, often combined with heart defects, serious disorders of the immune and circulatory systems, and liver development abnormalities. The disease is extremely rare; measures conservative therapy can effectively reduce the manifestations of neonatal lupus.
- Discoid lupus. The most common form of the disease is centrifugal erythema of Biette, the main manifestations of which are skin symptoms: 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 type of discoid is 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
Being systemic disease, lupus erythematosus is characterized by the following symptoms:
- chronic fatigue syndrome;
- swelling and tenderness of the joints, as well as muscle pain;
- unexplained fever;
- chest pain when breathing deeply;
- 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 white fingers and toes when exposed to cold or stress (Raynaud's syndrome).
Some people experience headaches, cramps, dizziness, and depression.
New symptoms may appear years after diagnosis. In some patients, one system of the body suffers (joints or skin, hematopoietic organs); in other patients, manifestations can affect many organs and be multi-organ in nature. The 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 blood vessels (vasculitis);
- pneumonia: pleurisy, pneumonitis;
- heart diseases: 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) occurs 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 among different patients. However commonplaces localization of lesions, as a rule, are the skin, joints (mainly hands and fingers), heart, lungs and bronchi, as well as digestive organs, nails and hair, which become more fragile and prone to loss, as well as the brain and nervous system.
Stages of the disease
Depending on the severity of the symptoms of the disease, systemic lupus erythematosus has several stages:
- Acute stage - at this stage of development, lupus erythematosus progresses sharply, the patient’s general condition worsens, he complains of constant fatigue, temperature rise to 39-40 degrees, fever, pain and aching muscles. The clinical picture develops rapidly; within 1 month the disease covers all organs and tissues of the body. Forecast at acute form lupus erythematosus is not comforting and often the patient’s life expectancy does not exceed 2 years;
- Subacute stage - the rate of disease progression and the severity of clinical symptoms is not the same as in acute stage and more than 1 year may pass from the moment of illness to the onset of symptoms. At this stage, the disease often gives way to periods of exacerbations and stable remission, the prognosis is generally favorable and the patient’s condition directly depends on the adequacy of the prescribed treatment;
- Chronic form - the disease has a sluggish course, clinical symptoms are mild, internal organs are practically not affected and the body as a whole functions normally. Despite the relatively mild course of 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 symptoms with the help of medications at the time of exacerbation.
Complications of SLE
The main complications caused by SLE:
1) Heart disease:
- pericarditis - inflammation of the heart sac;
- 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. Valve transplantation is often performed;
- myocarditis (inflammation of the heart muscle), causing severe arrhythmias, diseases of the heart muscle.
2) Renal pathologies (nephritis, nephrosis) develop in 25% of patients suffering from SLE. The first symptoms are swelling in the legs, the presence of protein and blood in the urine. Failure of the kidneys to work normally is extremely life-threatening. Treatment includes the use of strong drugs for SLE, dialysis, and kidney transplantation.
3) Blood diseases that are life-threatening.
- decrease in red blood cells (supply cells with oxygen), leukocytes (suppress infections and inflammation), platelets (promote blood clotting);
- hemolytic anemia caused by a lack of red blood cells or platelets;
- pathological changes in the hematopoietic organs.
4) Lung diseases (in 30%), pleurisy, muscle inflammation chest, joints, ligaments. Development of acute tuberculous lupus (inflammation of 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 of erythematosis may change over time, so it is difficult to make an accurate diagnosis based on them. It is necessary to use a set of additional examinations:
- general blood and urine tests;
- determination of liver enzyme levels;
- antinuclear body (ANA) analysis;
- chest x-ray;
- echocardiography;
- biopsy.
Differential diagnosis
Chronic lupus erythematosus is differentiated from lichen planus, tuberculous leukoplakia and lupus, early rheumatoid arthritis, Sjogren's syndrome (see dry mouth, dry eye syndrome, photophobia). When the red border of the lips is affected, chronic SLE is differentiated from abrasive precancerous Manganotti cheilitis and actinic cheilitis.
Since damage to internal organs is always similar in course to various infectious processes, SLE is differentiated from Lyme disease, syphilis, mononucleosis (infectious mononucleosis in children: symptoms), and 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;
- when life-threatening conditions occur: rapidly progressive renal failure, acute pneumonitis or pulmonary hemorrhage.
- when neurological complications occur.
- with a significant decrease in the number of platelets, red blood cells or lymphocytes.
- in cases where an exacerbation of SLE cannot be treated on an outpatient basis.
For the treatment of systemic lupus erythematosus during exacerbation, they are widely used. hormonal drugs(prednisolone) and cytostatics (cyclophosphamide) according to a certain scheme. If the organs of the musculoskeletal system are affected, 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, consultation with a specialist in this field is necessary.
Nutrition rules
Dangerous and harmful foods for lupus:
- large amounts of sugar;
- everything fried, fatty, salted, smoked, canned;
- products to which there are allergic reactions;
- sweet sodas, energy drinks and alcoholic drinks;
- if you have kidney problems, foods containing potassium are contraindicated;
- canned food, sausages and factory-cooked sausages;
- store-bought mayonnaise, ketchup, sauces, dressings;
- confectionery products with cream, condensed milk, and artificial fillers (factory-made jams, preserves);
- fast food and products with unnatural fillers, dyes, leavening agents, enhancers taste qualities and smell;
- foods containing cholesterol (buns, bread, red meat, high-fat dairy products, sauces, dressings and soups based on cream);
- products that have a long shelf life (we mean those products that quickly deteriorate, but thanks to various chemical additives in their composition, they can be stored for a very long time - this includes, as an example, dairy products with a one-year shelf life).
Eating these foods can speed up 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 become active, which will cause all the symptoms to worsen and your health to worsen significantly.
Lifespan
Survival rate 10 years after diagnosis of systemic lupus erythematosus is 80%, after 20 years - 60%. Main causes of death: lupus nephritis, neurolupus, intercurrent infections. There are cases of survival of 25-30 years.
In general, the quality and length of life with systemic lupus erythematosus depends on several factors:
- Patient's age: the younger the patient, the higher the activity of the autoimmune process and the more aggressive the disease is, which is associated with greater reactivity of the immune system at a young age (more autoimmune antibodies destroy their own tissues).
- Timeliness, regularity and adequacy of therapy: with long-term use of glucocorticosteroid hormones and other drugs, you 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 before complications develop.
- Variant of the course of the disease: acute course The course is extremely unfavorable and after a couple of years severe, life-threatening complications may arise. And with a chronic course, which is 90% of cases of SLE, you can live a full life until old age (if you follow all the recommendations of a rheumatologist and therapist).
- Compliance with the regimen significantly improves the prognosis of the disease. To do this, you need to be constantly monitored by a doctor, adhere to his recommendations, promptly contact doctors if any symptoms of 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 ways. But millions of people with more severe illnesses live bright, full of impressions lives! So you can too.
Prevention
The goal of prevention is to prevent the development of relapses and to maintain the patient in a state of stable remission for a long time. Prevention of lupus is based on an integrated approach:
- Regular medical examinations and consultations with a rheumatologist.
- Take medications strictly in the prescribed dose and at the specified intervals.
- Compliance with the work and rest regime.
- Get adequate sleep, at least 8 hours a day.
- A diet with limited salt and sufficient protein.
- Hardening, walking, gymnastics.
- The use of hormone-containing ointments (for example, Advantan) for skin lesions.
- Use of sunscreens (creams).
Systemic lupus erythematosus (SLE) affects several million people worldwide. These are people of all ages, from babies to the elderly. The reasons for the development of the disease are unclear, but many factors contributing to its occurrence have been well studied. There is no cure for lupus yet, but this diagnosis no longer sounds like a death sentence. Let's try to figure out whether Dr. House was right in suspecting this disease in many of his patients, whether there is a genetic predisposition to SLE and whether a certain lifestyle can protect against this disease.
We continue the series on autoimmune diseases - diseases in which the body begins to fight itself, producing autoantibodies and/or autoaggressive clones of lymphocytes. We talk about how the immune system works and why sometimes it starts to “shoot at its own people.” Separate publications will be devoted to some of the most common diseases. To maintain objectivity, we invited Dr. biological sciences, corresponding member RAS, professor of the Department of Immunology of Moscow State University Dmitry Vladimirovich Kuprash. In addition, each article has its own reviewer, who delves into all the nuances in more detail.
The reviewer of this article was Olga Anatolyevna Georginova, Candidate of Medical Sciences, rheumatologist, assistant at the Department of Internal Medicine, Faculty of Fundamental Medicine, Moscow State University named after M.V. Lomonosov.
Drawing by William Bagg from Wilson's atlas (1855)
Most often, a person comes to the doctor exhausted by febrile fever (temperature above 38.5 °C), and it is this symptom that serves as the reason for him to see a doctor. His joints swell and hurt, his whole body aches, his lymph nodes become enlarged and cause discomfort. The patient complains about fatigue and increasing weakness. Other symptoms reported at the appointment include mouth ulcers, alopecia and gastrointestinal dysfunction. Often the patient suffers from excruciating headaches, depression, severe fatigue. His condition negatively affects his work performance and social life. Some patients may even experience mood disorders, cognitive impairment, psychosis, movement disorders and myasthenia gravis.
It is not surprising that Josef Smolen from the Vienna General Hospital (Wiener Allgemeine Krankenhaus, AKH) called systemic lupus erythematosus “the most complex disease in the world” at a 2015 congress on the disease.
To assess disease activity and treatment success, clinical practice About 10 different indexes are used. They can be used to track changes in the severity of symptoms over a period of time. Each disorder is assigned a specific score, and the final score indicates the severity of the disease. The first such methods appeared in the 1980s, and now their reliability has long been confirmed by research and practice. The most popular of them are SLEDAI (Systemic Lupus Erythematosus Disease Activity Index), its modification used in the Safety of Estrogens in Lupus National Assessment (SELENA) study, BILAG (British Isles Lupus Assessment Group Scale), SLICC/ACR damage index (Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index) and ECLAM (European Consensus Lupus Activity Measurement). In Russia, they also use the assessment of SLE activity according to V.A.’s classification. Nasonova.
Main targets of the disease
Some tissues are more affected by attacks from autoreactive antibodies than others. In SLE, the kidneys and cardiovascular system are especially often affected.
Autoimmune processes also disrupt the functioning of blood vessels and the heart. According to the most conservative estimates, every tenth death from SLE is caused by circulatory disorders that develop as a result of systemic inflammation. The risk of ischemic stroke in patients with this disease increases twofold, the risk of intracerebral hemorrhage increases threefold, and the risk of subarachnoid hemorrhage increases almost fourfold. Survival after stroke is also much worse than in the general population.
The totality of manifestations of systemic lupus erythematosus is immense. In some patients, the disease may affect only the skin and joints. In other cases, patients are exhausted by excessive fatigue, increasing weakness throughout the body, prolonged febrile fever and cognitive impairment. This may be accompanied by thrombosis and severe organ damage, such as end-stage renal disease. Because of these different manifestations, SLE is called a disease with a thousand faces.
Family planning
One of the most important risks associated with SLE is the numerous complications during pregnancy. The vast majority of patients are young women of childbearing age, so family planning, pregnancy management and monitoring the condition of the fetus are now of great importance.
Before the development of modern methods of diagnosis and therapy, the mother’s illness often negatively affected the course of pregnancy: conditions arose that threatened the woman’s life, and the pregnancy often ended intrauterine death fetus, premature birth, preeclampsia. Because of this, for a long time, doctors strongly discouraged women with SLE from having children. In the 1960s, women lost their fetuses 40% of the time. By the 2000s, the number of such cases had more than halved. Today, researchers estimate this figure at 10–25%.
Now doctors advise getting pregnant only during remission of the disease, since the survival of the mother, the success of pregnancy and childbirth depends on the activity of the disease several months before conception and at the very moment of fertilization of the egg. Because of this, doctors consider counseling the patient before and during pregnancy a necessary step.
In rare cases now, a woman finds out that she has SLE while she is already pregnant. Then, if the disease is not very active, pregnancy can proceed favorably with maintenance therapy with steroid or aminoquinoline drugs. If pregnancy, coupled with SLE, begins to threaten health and even life, doctors recommend an abortion or an emergency caesarean section.
About one in 20,000 children develops neonatal lupus- a passively acquired autoimmune disease, known for more than 60 years (case incidence is given for the USA). It is mediated by maternal antinuclear autoantibodies to the Ro/SSA, La/SSB antigens or to the U1-ribonucleoprotein. The presence of SLE in the mother is not at all necessary: only 4 out of 10 women giving birth to children with neonatal lupus have SLE at the time of birth. In all other cases, the above antibodies are simply present in the mothers’ bodies.
The exact mechanism of damage to the baby's tissues is still unknown, and most likely it is more complex than simply the penetration of maternal antibodies through the placental barrier. The prognosis for the newborn's health is usually good, and most symptoms resolve quickly. However, sometimes the consequences of the disease can be very severe.
In some children, skin lesions are noticeable at birth, while in others they develop over several weeks. The disease can affect many body systems: cardiovascular, hepatobiliary, central nervous, and lungs. In the worst case scenario, the child may develop life-threatening congenital heart block.
Economic and social aspects of the disease
A person with SLE suffers not only from the biological and medical manifestations of the disease. A significant portion of the disease burden is social, and this can create a vicious cycle of worsening symptoms.
Thus, regardless of gender and ethnicity, poverty, low level education, lack of health insurance, insufficient social support and treatment contribute to the worsening of the patient’s condition. This, in turn, leads to disability, loss of productivity and a further decline in social status. All this significantly worsens the prognosis of the disease.
One should not discount the fact that treatment for SLE is extremely expensive, and the costs directly depend on the severity of the disease. TO direct expenses include, for example, the costs of inpatient treatment(time spent in hospitals and rehabilitation centers, and related procedures), outpatient treatment (treatment with prescribed mandatory and additional medications, visits to doctors, laboratory tests and other studies, ambulance calls), surgical operations, transportation to medical institutions and additional medical services. According to 2015 estimates, in the United States, a patient spends an average of $33 thousand per year on all of the above items. If he develops lupus nephritis, then the amount more than doubles - up to $71 thousand.
Indirect costs may even be higher than direct ones, since they include loss of working capacity and disability due to illness. Researchers estimate the amount of such losses at $20 thousand.
Russian situation: “for Russian rheumatology to exist and develop, we need state support”
In Russia, tens of thousands of people suffer from SLE - about 0.1% of the adult population. Traditionally, rheumatologists treat this disease. One of the largest institutions where patients can turn for help is the Research Institute of Rheumatology named after. V.A. Nasonova RAMS, founded in 1958. As the current director of the research institute, academician of the Russian Academy of Medical Sciences, Honored Scientist of the Russian Federation Evgeniy Lvovich Nasonov recalls, at first his mother, Valentina Aleksandrovna Nasonova, who worked in the rheumatology department, came home almost every day in tears, since four out of five patients died from in her hands. Fortunately, this tragic trend has been overcome.
Patients with SLE are also provided with assistance in the rheumatology department of the Clinic of Nephrology, Internal and Occupational Diseases named after E.M. Tareev, Moscow City Rheumatology Center, Children's City Clinical Hospital named after. FOR. Bashlyaeva Department of Health (Tushino Children's City Hospital), Scientific Center for Children's Health of the Russian Academy of Medical Sciences, Russian Children's Clinical Hospital and Central Children's Clinical Hospital of the FMBA.
However, even now it is very difficult to suffer from SLE in Russia: the availability of the latest biological drugs for the population leaves much to be desired. The cost of such therapy is about 500–700 thousand rubles per year, and the medication is taken for a long time, and is by no means limited to one year. However, such treatment is not included in the list of vital drugs (VED). The standard of care for patients with SLE in Russia is published on the website of the Ministry of Health of the Russian Federation.
Currently, therapy with biological drugs is used at the Research Institute of Rheumatology. First, the patient receives them for 2–3 weeks while he is in the hospital; compulsory medical insurance covers these costs. After discharge, he needs to submit an application at his place of residence for additional drug provision to the regional department of the Ministry of Health, and the final decision is made by a local official. Often his answer is negative: in some regions, patients with SLE are not of interest to the local health department.
At least 95% of patients have autoantibodies, recognizing fragments of the body’s own cells as foreign (!) and therefore posing a danger. It is not surprising that the central figure in the pathogenesis of SLE is considered B cells producing autoantibodies. These cells are an essential part of adaptive immunity, with the ability to present antigens T cells and secreting signaling molecules - cytokines. It is assumed that the development of the disease is triggered by the hyperactivity of B cells and their loss of tolerance to the body's own cells. As a result, they generate a variety of autoantibodies that are directed at nuclear, cytoplasmic and membrane antigens contained in the blood plasma. As a result of the binding of autoantibodies and nuclear material, immune complexes, which are deposited in tissues and are not effectively removed. Many clinical manifestations of lupus are the result of this process and subsequent organ damage. The inflammatory response is exacerbated by the fact that B cells secrete about inflammatory cytokines and present T-lymphocytes not with foreign antigens, but with antigens of their own body.
The pathogenesis of the disease is also associated with two other simultaneous events: with an increased level of apoptosis(programmed cell death) of lymphocytes and with the deterioration of the processing of waste material arising during autophagy. This “littering” of the body leads to an incitement of the immune response towards its own cells.
Autophagy- the process of recycling intracellular components and replenishing the supply of nutrients in the cell - is now on everyone’s lips. In 2016, for the discovery of the complex genetic regulation of autophagy, Yoshinori Ohsumi ( Yoshinori Ohsumi) was awarded the Nobel Prize. The role of self-food is to maintain cellular homeostasis, recycle damaged and old molecules and organelles, and maintain cell survival under stressful conditions. You can read more about this in the article on “biomolecule”.Recent research shows that autophagy is important for the normal functioning of many immune responses: for example, the maturation and function of immune cells, pathogen recognition, and antigen processing and presentation. There is now more and more evidence that autophagic processes are associated with the occurrence, course and severity of SLE.
It has been shown that in vitro macrophages from SLE patients ingest less cellular debris compared to macrophages healthy people from the control group. Thus, if disposal is unsuccessful, apoptotic waste “attracts the attention” of the immune system, and pathological activation of immune cells occurs (Fig. 3). It turned out that some types of drugs that are already used for the treatment of SLE or are at the stage of preclinical studies act specifically on autophagy.
In addition to the above features, patients with SLE are characterized by increased expression of type I interferon genes. The products of these genes are a very well-known group of cytokines that play antiviral and immunomodulatory roles in the body. It is possible that an increase in the amount of type I interferons affects the activity of immune cells, which leads to a malfunction of the immune system.
Figure 3. Current ideas about the pathogenesis of SLE. One of the main causes of clinical symptoms of SLE is the deposition in tissues of immune complexes formed by antibodies that have bound fragments of cell nuclear material (DNA, RNA, histones). This process provokes a strong inflammatory response. In addition, with increased apoptosis, NETosis, and decreased efficiency of autophagy, unutilized cell fragments become targets for cells of the immune system. Immune complexes via receptors FcγRIIa enter plasmacytoid dendritic cells ( pDC), where the nucleic acids of the complexes activate Toll-like receptors ( TLR-7/9) , . Activated in this way, pDC begin the powerful production of type I interferons (including IFN-α). These cytokines, in turn, stimulate the maturation of monocytes ( Mø) to antigen-presenting dendritic cells ( DC) and the production of autoreactive antibodies by B cells, prevent apoptosis of activated T cells. Monocytes, neutrophils and dendritic cells under the influence of type I IFN increase the synthesis of the cytokines BAFF (a stimulator of B cells, promoting their maturation, survival and antibody production) and APRIL (an inducer of cell proliferation). All this leads to an increase in the number of immune complexes and even more powerful activation of pDC - the circle closes. The pathogenesis of SLE also involves abnormal oxygen metabolism, which increases inflammation, cell death and the influx of autoantigens. This is largely the fault of mitochondria: disruption of their work leads to increased formation of reactive oxygen species ( ROS) and nitrogen ( RNI), deterioration protective functions neutrophils and netosis ( NETosis)
Finally, oxidative stress, together with abnormal oxygen metabolism in the cell and disturbances in the functioning of mitochondria, can also contribute to the development of the disease. Due to the increased secretion of pro-inflammatory cytokines, tissue damage and other processes that characterize the course of SLE, an excessive amount of reactive oxygen species(ROS), which further damage surrounding tissues, promote a constant influx of autoantigens and specific suicide of neutrophils - netozu(NETosis). This process ends with the formation neutrophil extracellular traps(NETs) designed to trap pathogens. Unfortunately, in the case of SLE, they play against the host: these network-like structures are composed predominantly of major lupus autoantigens. Interaction with the latter antibodies makes it difficult to cleanse the body of these traps and enhances the production of autoantibodies. This creates a vicious circle: increasing tissue damage as the disease progresses entails an increase in the amount of ROS, which destroys tissue even more, enhances the formation of immune complexes, stimulates the synthesis of interferon... The pathogenetic mechanisms of SLE are presented in more detail in Figures 3 and 4.
Figure 4. The role of programmed neutrophil death - NETosis - in the pathogenesis of SLE. Immune cells typically do not encounter most of the body's own antigens because potential self-antigens are found within cells and are not presented to lymphocytes. After autophagic death, the remains of dead cells are quickly disposed of. However, in some cases, for example, with an excess of reactive oxygen and nitrogen species ( ROS And RNI), the immune system encounters autoantigens “nose to nose”, which provokes the development of SLE. For example, under the influence of ROS, polymorphonuclear neutrophils ( PMN) are exposed netozu, and a “network” is formed from the remains of the cell. net), containing nucleic acids and proteins. This network becomes the source of autoantigens. As a result, plasmacytoid dendritic cells are activated ( pDC), releasing IFN-α and provoking an autoimmune attack. Other symbols: REDOX(reduction-oxidation reaction) - imbalance of redox reactions; ER- endoplasmic reticulum; DC- dendritic cells; B- B cells; T- T cells; Nox2- NADPH oxidase 2; mtDNA- mitochondrial DNA; black up and down arrows- amplification and suppression, respectively. To see the picture in full size, click on it.
Who's to blame?
Although the pathogenesis of systemic lupus erythematosus is more or less clear, scientists find it difficult to name its key cause and therefore consider a combination of various factors that increase the risk of developing this disease.
In our century, scientists turn their attention primarily to hereditary predisposition to the disease. SLE did not escape this either - which is not surprising, because the incidence varies greatly by gender and ethnicity. Women suffer from this disease approximately 6–10 times more often than men. Their incidence peaks at 15–40 years of age, that is, during childbearing age. Prevalence, course of disease and mortality are associated with ethnicity. For example, a butterfly rash is typical in white patients. In African Americans and Afro-Caribbeans, the disease is much more severe than in Caucasians; relapses of the disease and inflammatory disorders of the kidneys are more common among them. Discoid lupus is also more common in dark-skinned people.
These facts indicate that genetic predisposition may play a role important role in the etiology of SLE.
To clarify this, the researchers used a method genome-wide association search, or GWAS, which allows thousands of genetic variants to be correlated with phenotypes—in this case, disease manifestations. Thanks to this technology, it was possible to identify more than 60 susceptibility loci for systemic lupus erythematosus. They can be roughly divided into several groups. One such group of loci is associated with the innate immune response. These are, for example, the pathways of NF-kB signaling, DNA degradation, apoptosis, phagocytosis, and utilization of cellular debris. It also includes variants responsible for the function and signaling of neutrophils and monocytes. Another group includes genetic variants involved in the work of the adaptive part of the immune system, that is, associated with the function and signaling networks of B and T cells. In addition, there are loci that do not fall into these two groups. Interestingly, many risk loci are common to SLE and other autoimmune diseases (Fig. 5).
Genetic data could be used to determine the risk of developing SLE, its diagnosis or treatment. This would be extremely useful in practice, since due to the specifics of the disease, it is not always possible to identify it from the patient’s first complaints and clinical manifestations. Selecting treatment also takes some time, because patients respond to therapy differently, depending on the characteristics of their genome. So far, however, genetic tests are not used in clinical practice. An ideal model for assessing disease susceptibility would take into account not only specific gene variants, but also genetic interactions, levels of cytokines, serological markers, and many other data. In addition, it should, if possible, take into account epigenetic features - after all, according to research, they make a huge contribution to the development of SLE.
Unlike the genome, epi the genome is relatively easily modified under the influence external factors. Some believe that without them, SLE may not develop. The most obvious is ultraviolet radiation, as patients often experience redness and rashes on their skin after exposure to sunlight.
The development of the disease, apparently, can provoke viral infection . It is possible that in this case autoimmune reactions arise due to molecular mimicry of viruses- the phenomenon of similarity of viral antigens with the body’s own molecules. If this hypothesis is correct, then the Epstein-Barr virus becomes the focus of research. However, in most cases, scientists find it difficult to name the specific culprits. It is believed that autoimmune reactions are provoked not by specific viruses, but by common mechanisms combat this type of pathogen. For example, the activation pathway of type I interferons is common in the response to viral invasion and in the pathogenesis of SLE.
Factors such as smoking and drinking alcohol, however, their influence is ambiguous. It is likely that smoking can increase the risk of developing the disease, exacerbating it and increasing organ damage. Alcohol, according to some data, reduces the risk of developing SLE, but the evidence is quite contradictory, and it is better not to use this method of protection against the disease.
There is not always a clear answer regarding the influence occupational risk factors. If contact with silicon dioxide, according to a number of studies, provokes the development of SLE, then there is no exact answer about exposure to metals, industrial chemicals, solvents, pesticides and hair dyes. Finally, as mentioned above, lupus can be triggered by medication use: Common triggers include chlorpromazine, hydralazine, isoniazid, and procainamide.
Treatment: past, present and future
As already mentioned, it is not yet possible to cure “the most difficult disease in the world.” The development of a drug is hampered by the multifaceted pathogenesis of the disease, which involves different parts of the immune system. However, with competent individual selection of maintenance therapy, deep remission can be achieved, and the patient will be able to live with lupus erythematosus simply as with a chronic disease.
Treatment for various changes in the patient’s condition can be adjusted by a doctor, or rather, by doctors. The fact is that in the treatment of lupus, the coordinated work of a multidisciplinary group of medical professionals is extremely important: a family doctor in the West, a rheumatologist, a clinical immunologist, a psychologist, and often a nephrologist, hematologist, dermatologist, neurologist. In Russia, a patient with SLE first of all goes to a rheumatologist, and depending on the damage to systems and organs, he may require additional consultation with a cardiologist, nephrologist, dermatologist, neurologist and psychiatrist.
The pathogenesis of the disease is very complex and confusing, so many targeted drugs are currently in development, while others have shown their failure at the trial stage. Therefore, in clinical practice, nonspecific drugs are still most widely used.
Standard treatment includes several types of medications. First of all, they write out immunosuppressants- to suppress excessive activity of the immune system. The most commonly used of them are cytostatic drugs methotrexate, azathioprine, mycophenolate mofetil And cyclophosphamide. In fact, these are the same drugs that are used for cancer chemotherapy and act primarily on actively dividing cells (in the case of the immune system, on clones of activated lymphocytes). It is clear that such therapy has many dangerous side effects.
During the acute phase of the disease, patients usually take corticosteroids- nonspecific anti-inflammatory drugs that help calm the most violent storms of autoimmune reactions. They have been used in the treatment of SLE since the 1950s. Then they moved the treatment of this autoimmune disease to a qualitatively new level, and still remain the basis of therapy for lack of an alternative, although many side effects are also associated with their use. Most often, doctors prescribe prednisolone And methylprednisolone.
For exacerbation of SLE, it has also been used since 1976. pulse therapy: the patient receives impulses in high doses methylprednisolone and cyclophosphamide. Of course, over 40 years of use, the regimen of such therapy has changed greatly, but is still considered the gold standard in the treatment of lupus. However, it has many severe side effects, which is why it is not recommended for some groups of patients, for example, people with poorly controlled arterial hypertension and those suffering from systemic infections. In particular, the patient may develop metabolic disorders and behavior changes.
When remission is achieved, it is usually prescribed antimalarial drugs, which have been successfully used for a long time to treat patients with lesions of the musculoskeletal system and skin. Action hydroxychloroquine, one of the best known substances of this group, for example, is explained by the fact that it inhibits the production of IFN-α. Its use provides long-term reduction in disease activity, reduces damage to organs and tissues, and improves pregnancy outcome. In addition, the drug reduces the risk of thrombosis - and this is extremely important given the complications that arise in the cardiovascular system. Thus, the use of antimalarial drugs is recommended for all patients with SLE. However, there is also a fly in the ointment. In rare cases, retinopathy develops in response to this therapy, and patients with severe renal or hepatic impairment are at risk for hydroxychloroquine-associated toxicity.
Used in the treatment of lupus and newer ones, targeted drugs(Fig. 5). The most advanced developments target B cells: the antibodies rituximab and belimumab.
Figure 5. Biological drugs in the treatment of SLE. Apoptotic and/or necrotic cell debris accumulate in the human body, for example due to viral infection and exposure to ultraviolet radiation. This "garbage" can be taken up by dendritic cells ( DC), whose main function is the presentation of antigens to T and B cells. The latter acquire the ability to respond to autoantigens presented to them by DCs. This is how the autoimmune reaction begins, the synthesis of autoantibodies starts. Many biological drugs are currently being studied - drugs that affect the regulation of the body's immune components. The innate immune system is targeted anifrolumab(anti-IFN-α receptor antibody), sifalimumab And Rontalizumab(antibodies to IFN-α), infliximab And etanercept(antibodies to tumor necrosis factor, TNF-α), sirukumab(anti-IL-6) and tocilizumab(anti-IL-6 receptor). Abatacept (cm. text), belatacept, AMG-557 And IDEC-131 block costimulatory molecules of T cells. Fostamatinib And R333- splenic tyrosine kinase inhibitors ( SYK). Various B cell transmembrane proteins are targeted rituximab And ofatumumab(antibodies to CD20), epratuzumab(anti-CD22) and blinatumomab(anti-CD19), which also blocks plasma cell receptors ( PC). Belimumab (cm. text) blocks the soluble form BAFF, tabalumab and blisibimod are soluble and membrane-bound molecules BAFF, A
Another potential target of anti-lupus therapy is type I interferons, which were already discussed above. Some antibodies to IFN-α have already shown promising results in patients with SLE. Now the next, third, phase of their testing is being planned.
Also, among the drugs whose effectiveness in SLE is currently being studied, it should be mentioned abatacept. It blocks costimulatory interactions between T and B cells, thereby restoring immunological tolerance.
Finally, various anti-cytokine drugs are being developed and tested, e.g. etanercept And infliximab- specific antibodies to tumor necrosis factor, TNF-α.
Conclusion
Systemic lupus erythematosus remains a daunting challenge for the patient, a challenge for the physician, and an underexplored area for the scientist. However medical side The question should not be limited. This disease provides a huge field for social innovation, since the patient needs not only medical care, but also various types of support, including psychological. Thus, improving methods of providing information, specialized mobile applications, platforms with accessible information significantly improve the quality of life of people with SLE.
They help a lot in this matter patient organizations- public associations of people suffering from some disease and their relatives. For example, the Lupus Foundation of America is very famous. The activities of this organization are aimed at improving the quality of life of people diagnosed with SLE through special programs, scientific research, education, support and assistance. Its primary goals include reducing time to diagnosis, providing patients with safe and effective treatment, and increasing access to treatment and care. In addition, the organization emphasizes the importance of educating health care personnel, communicating concerns to government officials, and raising social awareness regarding systemic lupus erythematosus.
The global burden of SLE: prevalence, health disparities and socioeconomic impact. Nat Rev Rheumatol. 12 , 605-620;
The autoimmune process leads to inflammation of the vascular walls and various tissues. The course of the disease may be mild. But most people diagnosed with the disease must visit their doctor regularly and take medications regularly.
Lupus erythematosus syndrome may accompany systemic organ damage. There are other forms of the disease, for example, discoid, drug-induced damage or the red form of pathology in newborns.
The lesion occurs due to the formation of antibodies in the blood to the body's own tissues. They cause inflammation of various organs. The most common type of such antibodies are antinuclear antibodies (ANA), which react with sections of the DNA of body cells. They are determined when a blood test is ordered.
Lupus is a chronic disease. It is accompanied by damage to many organs: kidneys, joints, skin and others. Violations of their functions intensify in the acute period of the disease, which is then replaced by remission.
The disease is not contagious. More than 5 million people suffer from it worldwide, 90% of them are women. Pathology occurs between the ages of 15 and 45 years. There is no cure, but symptoms can be controlled with medications and lifestyle changes.
Systemic lupus erythematosus
Systemic lupus erythematosus has an autoimmune mechanism of development. The patient's B lymphocytes ( immune cells) produce antibodies to the tissues of their own body. In addition to direct damage to cells, autoantibodies combined with autoantigens form circulating immune complexes that are transported in the blood and settle in the kidneys and walls of small vessels. Inflammation develops.
The process is systemic in nature, that is, disorders can occur in almost any organ. Skin, kidneys, head and spinal cord, peripheral nerves. Clinical manifestations of the disease are also caused by involvement of joints, muscles, heart, lungs, mesentery, and eyes. In a third of patients, the disease causes the development of antiphospholipid syndrome, which in women is accompanied by miscarriage.
Pathology analysis reveals specific antinuclear antibodies, antibodies to cell DNA and Sm antigen. The activity of the disease is determined using a blood test, and therapy mainly depends on it.
Causes of the disease
The exact causes of lupus are unknown. Doctors believe that the onset of the disease is caused by a combination of external and internal factors, including hormonal imbalances, genetic changes and environmental influences.
Some studies have examined the relationship between estrogen levels and the disease in women. The disease often worsens in the period before menstruation and during pregnancy, when the secretion of these hormones is higher. However, the effect of increased estrogen levels on the occurrence of lesions has not been proven.
The causes of the disease may be related to genetic changes, although no specific gene mutation has been detected. The probability of the same diagnosis in both identical twins is 25%, in fraternal twins - 2%. If there are people in the family with this disease, the risk of their relatives getting sick is 20 times higher than the average.
Symptoms and causes of pathology are often associated with the action of external factors:
- ultraviolet radiation in a solarium or tanning, as well as from fluorescent lamps;
- the effect of silica dust in production;
- taking sulfa drugs, diuretics, tetracycline preparations, penicillin antibiotics;
- viruses, in particular Epstein-Barr, hepatitis C, cytomegalovirus and other infections;
- exhaustion, injuries, emotional stress, surgical interventions, pregnancy, childbirth and other causes of stress;
- smoking.
Under the influence of these factors, the patient develops autoimmune inflammation, which occurs in the form of nephritis, changes in the skin, nervous system, heart and other organs. Body temperature usually rises slightly, so people who are sick do not immediately see a doctor, and the disease gradually progresses.
Lupus symptoms
Common signs are weakness, lack of appetite, weight loss. The lesion may develop over 2 to 3 days or gradually. With an acute onset it is noted elevated temperature body, joint inflammation, butterfly redness on the face. The chronic course is characterized by polyarthritis; after a few years, during exacerbation, the kidneys, lungs, and nervous system are involved.
Symptoms of lupus are much more common in women than in men. Manifestations of the disease occur in young patients. They are associated with immune disorders in which the body produces antibodies against its own cells.
Symptoms of the disease:
- red rash on the face in the form of a butterfly;
- pain and swelling of the joints of the hand, wrist and ankle;
- small skin rash on the chest, rounded areas of redness on the extremities;
- hair loss;
- ulcers on the ends of the fingers, their gangrene;
- stomatitis;
- fever;
- headache;
- muscle pain;
- chest pain when breathing;
- the appearance of paleness of the fingers when exposed to cold (Raynaud's syndrome).
Changes can affect different body systems:
- kidneys: half of patients develop glomerulonephritis and renal failure;
- the nervous system suffers in 60% of patients: headaches, weakness, convulsions, sensory disturbances, depression, memory and intelligence impairment, psychosis;
- heart: pericarditis, myocarditis, arrhythmias, heart failure, thromboendocarditis with the spread of blood clots through the vessels to other organs;
- respiratory organs: dry pleurisy and pneumonitis, shortness of breath, cough;
- digestive organs: abdominal pain, diarrhea, vomiting, possible intestinal perforation;
- eye damage can cause blindness within a few days;
- antiphospholipid syndrome: thrombosis of arteries, veins, spontaneous abortions;
- blood changes: bleeding, decreased immunity.
Discoid pathology is a milder form of the disease, accompanied by skin lesions:
- redness;
- edema;
- peeling;
- thickening;
- gradual atrophy.
The tuberculous form of the disease received this name because of the similarity of the skin lesions to red. This is a different disease, it is caused by Mycobacterium tuberculosis and is accompanied by spots, bumpy rashes on the skin. Most often children get sick. This disease is contagious.
Diagnosis of the disease
Diagnosis of lupus erythematosus is carried out taking into account clinical signs illness and laboratory changes.
When examining a general blood test, the following abnormalities are detected:
- hypochromic anemia;
- decrease in the number of leukocytes, appearance of LE cells;
- thrombocytopenia;
- increase in ESR.
Diagnosis of the disease necessarily includes a urine test. With the development of autoimmune glomerulonephritis, red blood cells, protein and casts are found in it. In severe cases, a kidney biopsy is prescribed. The examination includes blood biochemistry with determination of the level of protein, liver enzymes, C-reactive protein, creatinine, and urea.
Immunological studies to help confirm the diagnosis:
- antinuclear antibodies are found in 95% of patients, but they are also recorded in some other diseases;
- a more accurate analysis for pathology is the determination of antibodies to native DNA and the Sm antigen.
Disease activity is assessed by the severity of the inflammatory syndrome. To confirm the diagnosis, the criteria of the American Rheumatological Association are used. If 4 out of 11 signs of the disease are present, the diagnosis is considered confirmed.
Differential diagnosis is carried out with the following diseases:
- rheumatoid arthritis;
- dermatomyositis;
- drug reaction to taking penicillamine, procainamide and other drugs.
Treatment of pathology
The disease requires treatment by a rheumatologist. The disease is accompanied by prolonged exacerbations, when signs of inflammation, weakness and other symptoms are expressed. Remission is usually short-lived, but with constant medication use, the anti-inflammatory effect of therapy is more pronounced.
How to treat the disease? First, the doctor determines the activity of the autoimmune process depending on clinical signs and changes in tests. Treatment for lupus erythematosus depends on its severity and includes the following medications:
- non-steroidal anti-inflammatory drugs;
- for rashes on the face - antimalarials (chloroquine);
- glucocorticoids orally, in severe cases - in large doses, but for a short course (pulse therapy);
- cytostatics (cyclophosphamide);
- for antiphospholipid syndrome - warfarin under INR control.
After the patient's signs of exacerbation pass, the dose of medication is gradually reduced. These drugs are quite effective, but cause many side effects.
If renal failure develops, hemodialysis is prescribed.
The disease in children is very rare, but is accompanied by damage to many systems, severe clinical manifestations, and a crisis course. The main drugs for treating the disease in children are glucocorticoid hormones.
Pathology during pregnancy often increases its activity. It carries the risk of complications for the mother and fetus. Therefore, they continue to take prednisolone, because this medicine does not cross the placenta and does not harm the baby.
The cutaneous form of the disease is a milder variant, manifested only by changes in the skin. Antimalarial drugs are prescribed, but if a transition to a systemic form is suspected, more serious treatment is necessary.
Treatment with folk remedies is ineffective. They can be used as an addition to conventional therapy, rather for a psychological effect. Recommended decoctions and infusions of the following plants:
- burnet;
- peony;
- calendula flowers;
- celandine;
- mistletoe leaves;
- hemlock;
- nettle;
- cowberry.
Such mixtures help reduce inflammation, prevent bleeding, soothe, and saturate the body with vitamins.
Video about lupus
Systemic lupus erythematosus is a chronic disease with many symptoms, which is based on constant autoimmune inflammation. Young girls and women aged 15 to 45 years are most often affected. Prevalence of lupus: 50 people per 100,000 population. Despite the fact that the disease is quite rare, knowing its symptoms is extremely important. In this article we will also talk about the treatment of lupus, which is usually prescribed by doctors.
Causes of systemic lupus erythematosus
Excessive exposure to ultraviolet radiation on the body contributes to the development of the pathological process.- Excessive exposure to ultraviolet radiation (especially “chocolate” tans and tans leading to sunburn).
- Stressful situations.
- Episodes of hypothermia.
- Physical and mental overload.
- Acute and chronic viral infection (herpes simplex virus, Epstein-Barr virus, cytomegalovirus).
- Genetic predisposition. If one of your relatives has or is suffering from lupus in the family, then for everyone else the risk of getting sick increases significantly.
- Deficiency of complement component C2. Complement is one of the “participants” of the body’s immune response.
- Presence of HLA All, DR2, DR3, B35, B7 antigens in the blood.
A number of studies show that lupus does not have one specific cause. Therefore, the disease is considered multifactorial, that is, its occurrence is due to the simultaneous or sequential influence of a number of causes.
Classification of systemic lupus erythematosus
According to the development of the disease:
- Acute onset. Against the background of complete health, lupus symptoms suddenly appear.
- Subclinical onset. Symptoms appear gradually and may mimic another rheumatic disease.
Course of the disease:
- Spicy. Usually, patients can tell within a few hours when their first symptoms appeared: the temperature rose, typical redness of the facial skin (“butterfly”) appeared, and joint pain began. Without proper treatment, the nervous system and kidneys are affected within 6 months.
- Subacute. The most common course of lupus. The disease begins nonspecifically, the general condition begins to worsen, and skin rashes may appear. The disease occurs cyclically, with each relapse involving new organs in the process.
- Chronic. Lupus for a long time manifests itself as relapses of only those symptoms and syndromes with which it began (polyarthritis, skin syndrome), without involving other organs and systems in the process. The chronic course of the disease has the most favorable prognosis.
Symptoms of systemic lupus erythematosus
Joint damage
It is observed in 90% of patients. It manifests itself as migrating pain in the joints and alternating inflammation of the joints. It is very rare for the same joint to constantly hurt and become inflamed. Mainly the interphalangeal, metacarpophalangeal and wrist joints are affected, and less commonly the ankle joints. Large joints(for example, knees and elbows) suffer much less frequently. Arthritis is usually accompanied by severe muscle pain and inflammation.
Skin syndrome
The most common type of lupus “butterfly” is redness of the skin in the cheekbones and dorsum of the nose.
There are several options for skin damage:
- Vasculitic (vascular) butterfly. It is characterized by unstable diffuse redness of the facial skin, with a blue discoloration in the center, intensified by cold, wind, excitement, and ultraviolet radiation. Foci of redness can be either flat or raised above the surface of the skin. After healing, no scars remain.
- Multiple skin rashes due to photosensitivity. They appear on open areas of the body (neck, face, décolleté, arms, legs) under the influence of sunlight. The rash goes away without a trace.
- Subacute lupus erythematosus. Areas of redness (erythema) appear after sun exposure. Erythemas are raised above the surface of the skin, can be ring-shaped, crescent-shaped, and almost always peel off. A patch of depigmented skin may remain at the site of the spot.
- Discoid lupus erythematosus. First, patients develop small red plaques, which gradually merge into one large lesion. The skin in such places is thin, and excessive keratinization is noted in the center of the lesion. Such plaques appear on the face and extensor surfaces of the limbs. After healing, scars remain at the site of the lesions.
TO skin manifestations Hair loss (up to complete), nail changes, and ulcerative stomatitis may occur.
Damage to the serous membranes
Such a defeat applies to diagnostic criteria, as it occurs in 90% of patients. These include:
- Pleurisy.
- Peritonitis (inflammation of the peritoneum).
Damage to the cardiovascular system
- Lupus.
- Pericarditis.
- Libman-Sachs endocarditis.
- Coronary artery disease and development.
- Vasculitis.
Raynaud's syndrome
Raynaud's syndrome is manifested by spasm of small vessels, which in patients with lupus can lead to necrosis of the fingertips of the hands, severe arterial hypertension, retinal damage.
Lung damage
- Pleurisy.
- Acute lupus pneumonitis.
- Damage to the connective tissue of the lungs with the formation of multiple foci of necrosis.
- Pulmonary hypertension.
- Pulmonary embolism.
- Bronchitis and.
Kidney damage
- Urinary syndrome.
- Nephrotic syndrome.
- Nephritic syndrome.
Damage to the central nervous system
- Astheno-vegetative syndrome, which manifests itself as weakness, fatigue, depression, irritability, and sleep disturbances.
- During the period of relapse, patients complain of decreased sensitivity, paresthesia (“goosebumps”). On examination, a decrease in tendon reflexes is noted.
- In severely ill patients, meningoencephalitis may develop.
- Emotional lability (weakness).
- Decreased memory, deterioration of intellectual abilities.
- Psychosis, seizures.
Diagnosis of systemic lupus erythematosus
In order to make a diagnosis of systemic lupus erythematosus, it is necessary to confirm that the patient has at least four criteria from the list.
- Rashes on the face. Flat or raised erythema localized to the cheeks and cheekbones.
- Discoid rashes. Erythematous spots, with peeling and hyperkeratosis in the center, leaving behind scars.
- Photosensitivity. Skin rashes appear as an excessive reaction to ultraviolet radiation.
- Ulcers in the mouth.
- Arthritis. Damage to two or more peripheral small joints, pain and inflammation in them.
- Serositis. Pleurisy, pericarditis, peritonitis or their combinations.
- Kidney damage. Changes in (appearance of traces of protein, blood), increased blood pressure.
- Neurological disorders. Convulsions, psychoses, seizures, emotional disturbances.
- Hematological changes. At least 2 clinical blood tests in a row must show one of the following indicators: leukopenia (decreased number of white blood cells), lymphopenia (decreased number of lymphocytes), thrombocytopenia (decreased number of platelets).
- Immunological disorders. Positive LE test ( high quantity antibodies to DNA), false-positive reaction to, moderate or high levels of rheumatoid factor.
- Presence of antinuclear antibodies (ANA). Detected by enzyme immunoassay.
What should be considered for differential diagnosis?
Due to the wide variety of symptoms, systemic lupus erythematosus has many common manifestations with other rheumatological diseases. Before making a diagnosis of lupus, it is necessary to exclude:
- Other diffuse connective tissue diseases (scleroderma, dermatomyositis).
- Polyarthritis.
- Rheumatism (acute rheumatic fever).
- Still's syndrome.
- Kidney lesions are not of lupus nature.
- Autoimmune cytopenias (decrease in the number of leukocytes, lymphocytes, platelets in the blood).
Treatment of systemic lupus erythematosus
The main goal of treatment is to suppress the body’s autoimmune reaction, which underlies all symptoms.
Patients are prescribed different types of drugs.
Glucocorticosteroids
Hormones are the drugs of choice for lupus. They are the ones that best relieve inflammation and suppress the immune system. Before glucocorticosteroids were introduced into the treatment regimen, patients lived a maximum of 5 years after diagnosis. Now life expectancy is much longer and depends to a greater extent on the timeliness and adequacy of the prescribed treatment, as well as how carefully the patient follows all the instructions.
The main indicator of the effectiveness of hormone treatment is long remissions with maintenance treatment with small doses of drugs, a decrease in the activity of the process, stable stabilization of the condition.
The drug of choice for patients with systemic lupus erythematosus is Prednisolone. It is prescribed on average at a dose of up to 50 mg/day, gradually reducing to 15 mg/day.
Unfortunately, there are reasons why hormonal treatment is ineffective: irregularity in taking pills, incorrect dosage, late start of treatment, very serious condition of the patient.
Patients, especially teenagers and young women, may be reluctant to take hormones because of possible side effects, mainly worrying about possible weight gain. In the case of systemic lupus erythematosus, there is really no choice: take it or not take it. As mentioned above, without hormone treatment, life expectancy is very low, and the quality of this life is very poor. Don't be afraid of hormones. Many patients, especially those with rheumatological diseases, take hormones for decades. And not all of them develop side effects.
Other possible side effects from taking hormones:
- Steroid erosions and.
- Increased risk infection.
- Increased blood pressure.
- Increased blood sugar levels.
All these complications also develop quite rarely. The main condition for effective hormonal treatment with minimal risk of side effects is the correct dose, regular use of pills (otherwise withdrawal syndrome is possible) and self-control.
Cytostatics
These drugs are prescribed in combination with when hormones alone are not effective enough or do not work at all. Cytostatics are also aimed at suppressing the immune system. There are indications for the use of these drugs:
- High activity of lupus with a rapidly progressing course.
- Involvement of the kidneys in the pathological process (nephrotic and nephritic syndromes).
- Low efficiency isolated therapy hormones.
- The need to reduce the dose of Prednisolone due to poor tolerability or sudden development of side effects.
- The need to reduce the maintenance dose of hormones (if it exceeds 15 mg/day).
- Formation of dependence on hormone therapy.
Most often, patients with lupus are prescribed azathioprine (Imuran) and cyclophosphamide.
Criteria for the effectiveness of treatment with cytostatics:
- Reducing the intensity of symptoms;
- Disappearance of dependence on hormones;
- Reduced disease activity;
- Persistent remission.
Nonsteroidal anti-inflammatory drugs
Prescribed to relieve joint symptoms. Most often, patients take Diclofenac and Indomethacin tablets. Treatment with NSAIDs lasts until body temperature normalizes and joint pain disappears.
Additional treatments
Plasmapheresis. During the procedure, metabolic products and immune complexes that provoke inflammation are removed from the patient's blood.
Prevention of systemic lupus erythematosus
The goal of prevention is to prevent the development of relapses and to maintain the patient in a state of stable remission for a long time. Prevention of lupus is based on an integrated approach:
- Regular medical examinations and consultations with a rheumatologist.
- Take medications strictly in the prescribed dose and at the specified intervals.
- Compliance with the work and rest regime.
- Get adequate sleep, at least 8 hours a day.
- A diet with limited salt and sufficient protein.
- The use of hormone-containing ointments (for example, Advantan) for skin lesions.
- Use of sunscreens (creams).
How to live with a diagnosis of systemic lupus erythematosus?
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 ways. But millions of people with more severe illnesses live bright, full of impressions lives! So you can too.
What needs to be done for this?
- Listen to yourself. If you are tired, lie down and rest. You may need to rearrange your daily schedule. But it’s better to take a nap several times a day than to work yourself to the point of exhaustion and increase the risk of relapse.
- Learn all the signs of when the disease may become acute. Usually this severe stress, prolonged exposure to the sun, and even the consumption of certain foods. If possible, avoid provoking factors, and life will immediately become a little more fun.
- Give yourself a moderate physical activity. It is best to do Pilates or yoga.
- Quit smoking and try to avoid secondhand smoke. Smoking does not improve your health at all. And if you remember that smokers more often get colds, bronchitis and pneumonia, overload their kidneys and heart... You shouldn’t risk many years of your life because of a cigarette.
- Accept your diagnosis, learn everything about the disease, ask your doctor everything you don’t understand and breathe easy. Lupus today is not a death sentence.
- If necessary, do not hesitate to ask your family and friends to support you.
What can you eat and what should you avoid?
In fact, you need to eat in order to live, and not vice versa. It's also best to eat foods that will help you fight lupus effectively and protect your heart, brain, and kidneys.
What to limit and what to give up
- Fats. Deep-fried dishes, fast food, dishes with a large number butter, vegetable or olive oils. All of them sharply increase the risk of developing complications from cardiovascular system. Everyone knows that fatty foods provoke cholesterol deposits in blood vessels. Avoid unhealthy fatty foods and protect yourself from a heart attack.
- Caffeine. Coffee, tea, and some drinks contain large amounts of caffeine, which irritates the gastric mucosa, prevents you from falling asleep, overloading the central nervous system. You will feel much better if you stop drinking cups of coffee. At the same time, the risk of developing erosion will be significantly reduced.
- Salt. Salt should be limited in any case. But this is especially necessary in order not to overload the kidneys, which may already be affected by lupus, and not to provoke a rise in blood pressure.
- Alcohol. harmful in itself, and in combination with medications usually prescribed to patients with lupus, it is generally an explosive mixture. Give up alcohol and you will immediately feel the difference.
What you can and should eat
- Fruits and vegetables. An excellent source of vitamins, minerals and fiber. Try to eat seasonal vegetables and fruits, they are especially healthy and also quite cheap.
- Foods and supplements high in calcium and vitamin D. They will help prevent diarrhea, which can develop while taking glucocorticosteroids. Eat fat-free or low-fat fermented milk products, cheeses and milk. By the way, if you take the tablets with milk rather than water, they will irritate the stomach lining less.
- Whole grain cereals and baked goods. These foods contain a lot of fiber and B vitamins.
- Protein. Protein is necessary for the body to effectively fight disease. It is better to eat lean, dietary varieties of meat and poultry: veal, turkey, rabbit. The same applies to fish: cod, pollock, lean herring, pink salmon, tuna, squid. In addition, seafood contains a lot of omega-3 unsaturated fatty acids. They are vital for normal brain and heart function.
- Water. Try to drink at least 8 glasses of clean, still water per day. This will improve your general condition, improve the functioning of the gastrointestinal tract, and help control hunger.
So, systemic lupus erythematosus in our time is not a death sentence. There is no need to give in to despair if you have been diagnosed with this; rather, it is necessary to “pull yourself together”, follow all the recommendations of the attending physician, lead a healthy lifestyle, and then the quality and life expectancy of the patient will increase significantly.
Which doctor should I contact?
Given the variety of clinical manifestations, sometimes it is quite difficult for a sick person to figure out which doctor to see at the onset of the disease. For any changes in your health, it is recommended to consult a therapist. After conducting tests, he will be able to suggest a diagnosis and refer the patient to a rheumatologist. Additionally, consultation with a dermatologist, nephrologist, pulmonologist, neurologist, cardiologist, or immunologist may be required. Since systemic lupus erythematosus is often associated with chronic infections, it will be useful to be examined by an infectious disease specialist. A nutritionist will provide assistance in treatment.
Systemic lupus erythematosus is a disease in which the human immune system perceives its own structures as foreign elements. This is a disease of young women. 9 out of 10 patients are young girls aged 15 to 26 years.
Other names for the pathology are SLE, Libaman-Sachs disease, diffuse connective tissue disorder. The disease got its main name because of its characteristic symptom - a rash on the face that resembles the silhouette of a butterfly. And in the Middle Ages, the affected area was associated with a place for wolf bites.
When systemic lupus erythematosus begins to progress, symptoms arise very quickly and form into entire symptom complexes. During the development of the disease, substances are formed in the body that have a detrimental effect on absolutely all organs and tissues. The only difference is that the target organs are destroyed faster than other tissues and structures.
Possible precipitating factors
The reasons why such an unfavorable autoimmune resolution process occurs are still not clear. There are only theories, as well as possible assumptions regarding the etiological role of certain factors. We are talking about the following aspects.
- Chronic viral infection. We are talking about RNA viruses and retroviruses. In many patients with SLE, colossal amounts of antibodies to these viruses were found in the blood, which indicates a previously raging infectious process.
- Genetic predisposition. It has been proven that cases of lupus erythematosus are recorded more often in those patients whose close relatives suffered from a similar disease.
- Individual susceptibility to drugs can also cause serious disruption in the functioning of the entire body at the cellular level. We are talking about specific types of intolerance - a negative reaction to vaccines, serums.
- Photosensitivity and ultrasonic irradiation can cause various malfunctions.
The fact that young women get sick in the vast majority of cases suggests “purely female” provoking factors. These include frequent and difficult pregnancies, a history of multiple births, abortions and miscarriages.
Pathogenetic aspects of the development of systemic lupus erythematosus
The pathogenesis of SLE is based on dysregulation of basic defense processes by the immune system. For example, how antigens perceive protein components, primarily DNA. As a result of adhesion, chronic immune failure leads to the fact that even those cells that were previously completely free of immune complexes begin to suffer.
Classification of the disease according to the IBC
Systemic lupus erythematosus, according to the MBC classification, can be of the following types:
- SLE caused by taking pharmacological products;
- SLE, accompanied by damage to other organs and systems;
- lupus erythematosus, unspecified;
- other forms of SLE.
Clinical classification divides different types illness according to other criteria. First of all, the nature of the flow is taken into account:
- spicy;
- subacute;
- relapsing polyarthritis;
- discoid lupus erythematosus;
- S-mRaynaud;
- S-mVerlgof;
- S-mSjögren;
- antiphospholipid syndrome.
The disease can be active to varying degrees. Depending on how rapidly the disease progresses, experts use the following designation methods:
- 0 – there is no activity at all;
- I – minimum;
- II – moderate;
- III – high.
The table below shows another complex classification diseases. SLE is considered from the perspective of the severity of clinical manifestations, depending on which system is most affected.
| Type of SLE according to severity of clinical manifestations | Clinical manifestations |
| Mild SLE | Erythematous rash, discoid rashes, Raynaud's phenomenon, baldness, ulcerative processes on the mucous membranes of the mouth, nasopharynx, febrile conditions varying degrees of severity, pain in the joints and muscles, “dry” pericarditis, pleurisy with a small amount of exudative fluid. |
| Severe SLE | Myocarditis, pericarditis, pleurisy with a large amount of exudate, nephrotic syndrome, pulmonary hemorrhages, thrombocytopenic purpura, hemolytic anemia, vasculitis, lesions of the central nervous system of varying severity. |
Systemic lupus erythematosus in children and patients over 40 years of age is relatively rare. The risk group is formed by women aged 15 to 26 years, as was previously mentioned.
Initial manifestations that patients may notice:
- weakness;
- weight loss;
- low-grade fever.
However, the progression of the disease forms a complex and quite vivid clinical picture. Let us consider separately each system involved in the pathological process.
Skin and mucous membranes
The most common symptom of lupus is characteristic skin changes. The pathological process can affect the tissues of the dermal tissue and mucous membranes in 28 different ways. The most typical of them are presented below.
- Erythematous spots of various sizes and shapes, which can be located isolated from each other or merge into one conglomerate. The color is intensely bright. The tissues are swollen and inflamed.
- Erythematous lesions with infiltration, dermal atrophy, peeling. Sometimes there are signs of hyperkeratosis. The symptom is characteristic of chronic form diseases.
- Lupus cheilitis is redness of the lips, which over time become covered with crusts, erosions, followed by deep tissue atrophy.
- Capillaritis is swelling spots localized in the area of the fingertips, palms, and soles.
- Enanthema of the mucous tissues of the oral cavity is an erythematous rash accompanied by severe hemorrhages and erosions.
- Trophic disorders are pathological processes in which a person loses hair, nails, skin tissue becomes excessively dry.
- Ring-shaped rash with telangiectasia.
Musculoskeletal system
Damage to connective tissue greatly affects the functional potential of the musculoskeletal system. The following symptoms indicate that bones and joints are involved in the pathological process:
- joint pain (prolonged, intense);
- polyarthritis of a symmetrical type, which is accompanied by involvement of the interphalangeal joints of the hands in the inflammatory process;
- joint stiffness, especially in the morning;
- formation of flexion contractures of the fingers;
- development of aseptic necrotic processes in inert and cartilaginous tissues.
The muscles are no less subject to “aggression” of the autoimmune process. Patients complain of constant myalgia, as well as severe muscle weakness. Polymyositis may develop.
Respiratory organs
When considering how lupus erythematosus affects life expectancy, it is worth familiarizing yourself with clinical picture the patient's affected lungs. The disease is accompanied by the following phenomena:
- dry or effusive pleurisy accompanied by severe shortness of breath, difficulty breathing, severe pain;
- lupus pneumonitis is manifested by shortness of breath, pain, dry cough, hemoptysis;
- pulmonary hypertension syndrome;
- PE as one of the types of complications.
Cardiovascular system
Pancarditis, or dry pericarditis, often develops. Diffuse myocarditis develops in the most active form of lupus. This pathological process is further complicated by circulatory failure.
Endocarditis affects the valve apparatus of the heart. Mitral insufficiency gradually develops, and then aortic valve insufficiency.
Vessels are also involved in a total destructive process. Vessels of medium and small caliber are primarily affected. Thrombophlebitis and phlebitis often occur. In case you are injured coronary arteries, the risk of myocardial infarction increases.
Organs of the digestive tract
Patients complain about constant feeling nausea, frequent vomiting. There is no appetite at all. Over time, the mucous membrane of the digestive canal begins to be affected by erosive processes of varying severity.
If the vessels of the mesentery are affected, the patient begins to experience very severe pain. Localization - around the navel. Rigidity of the abdominal muscles is observed. Liver destruction is expressed as follows: pathological phenomena– organ enlargement, jaundice.
Kidney damage
There is a whole classification according to which lupus nephritis is divided into several global categories:
- I – absence of any structural abnormalities in the biopsy;
- II – mesangial nephritis;
- III – focal glomerulonephritis of proliferative type;
- IV – diffuse proliferative glomerulonephritis;
- V – membranous glomerulonephritis;
- VI – sclerosing glomerulonephritis.
The table discusses key aspects regarding the main laboratory research. The diagnostic results allow you to accurately establish the diagnosis.
The diagnosis is based on a combination of specific symptoms. There are 11 specific criteria. If at least 4 of them are observed in a patient, he is diagnosed with systemic lupus erythematosus:
- butterfly rash;
- excessive skin sensitivity to ultraviolet rays;
- discoid rash;
- ulcerative processes localized on the mucous membranes;
- arthritis;
- pleurisy;
- kidney damage;
- damage to the central nervous system;
- immunological disorders;
- problems with blood cells (leukopenia, anemia);
- antinuclear bodies.
A number of special pharmacological products are used to treat diagnosed systemic lupus erythematosus. The table discusses all five groups of drugs that are relevant in modern regimens therapeutic effects on patients.
| Name of the group of pharmacological products | Examples of specific drugs | Features of purpose and application |
| Nonsteroidal anti-inflammatory drugs (NSAIDs) | Ibuprofen, Naproxen, Aspirin | Medicines are prescribed only for a short period of time to eliminate the active inflammatory process. |
| Antimalarials | Hydroxychloroquine | The funds are prescribed for a long period. The effect of taking medications occurs only after 1-2 months. They have proven themselves in the treatment of SLE with pronounced sensitivity to ultraviolet radiation. |
| Corticosteroids | Prednisone, prednisolone | Medicines reduce the activity of the immune system, suppress inflammatory reactions. Reception begins with minimal doses, gradually increasing to the maximum. Cancellation is also carried out gradually, reducing the number of medicinal products. |
| Antirheumatic drugs | Azathioprine, Methotrexate, Cyclophosphamide | The drugs suppress inflammation. The effect is similar to hormonal medications, but the mechanism of action on the tissues of the human body is different. |
| Biological PPMTB | Rituximab | Biological agents whose action is aimed at B-lymphocytes that synthesize antibodies. |
Duration of therapy and use of non-standard treatment approaches
Treatment should continue for as long as the symptoms of the disease are observed. Therapy cannot be interrupted under any pretext.
Today, patients are offered many alternative treatment options for such complex disease. Every untested method, methods associated with a number of risks and complications, or not approved by official medicine, do more harm than good.
Systemic lupus erythematosus can only be treated with medication under the close supervision of the attending physician. This is the only way to get rid of the disease and lead a full life.
Prognosis for patients
The prognosis is favorable if the disease was detected as early as possible, when tissues and organs have not yet undergone severe destruction. Mild rashes and arthritis, for example, are very easy to control.
Advanced forms of the disease require aggressive treatment with large doses of various pharmacological products. In this case, it is not known what harms the body more: colossal doses of drugs or the pathological process itself.
Preventive measures
Prevention of systemic lupus erythematosus is aimed solely at preventing relapses of the disease, as well as stopping further progression pathological processes. The essence of secondary preventive measures is timely and adequate treatment of the disease.
Patients undertake to undergo regular medical examinations and take hormonal medications in the correctly prescribed dosage. Do not sunbathe in the sun, do not overcool, avoid injuries, do not get vaccinated. If the disease worsens, you should immediately contact a specialist who has previously treated this person.
Conclusion
Thus, systemic lupus erythematosus is a rather serious autoimmune disease that requires an integrated approach to treatment. If diagnosed early, there is a chance to live a full life. That is why it is so important to contact a specialist in time at the first unfavorable signals.
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