Risk factors for systemic lupus erythematosus. Systemic lupus erythematosus. Treatment

Violation of the coordinated work of autoimmune mechanisms in the body, the formation of antibodies to one's own healthy cells is called lupus. Lupus erythematosus disease affects the skin, joints, blood vessels, internal organs, often has neurological manifestations. A characteristic symptom of the disease is a rash that looks like a butterfly, located on the cheekbones, bridge of the nose. It can manifest itself in any person, in a child or an adult.

What is lupus

Liebmann-Sachs disease is an autoimmune disease that affects connective tissue and the cardiovascular system. One of the recognizable symptoms is the appearance of red spots on the cheekbones, cheeks and bridge of the nose, which look like butterfly wings, and in addition, patients complain of weakness, fatigue, depression, fever.

Lupus disease - what is it? The reasons for its appearance and development are still not fully understood. It is proved that this is a genetic disease that can be inherited. The course of the disease alternates between acute periods and remissions, when it does not manifest itself. In most cases, the cardiovascular system, joints, kidneys, and nervous system suffer, and changes in the composition of the blood are manifested. There are two forms of the disease:

• discoid (only the skin suffers);
• systemic (damage to internal organs).

discoid lupus

Chronic discoid form affects only the skin, manifests itself in rashes on the face, head, neck and other exposed surfaces of the body. It develops gradually, starting with small rashes, ending with keratinization and a decrease in tissue volume. The prognosis for the treatment of the consequences of discoid lupus is positive, with its timely detection, remission is a long period.

Systemic lupus

What is systemic lupus erythematosus? The defeat of the immune system leads to the appearance of foci of inflammation in many body systems. The heart, blood vessels, kidneys, central nervous system, skin suffer, therefore, in the initial stages, the disease can be easily confused with arthritis, lichen, pneumonia, etc. Timely diagnosis can reduce the negative manifestations of the disease, increase the stages of remission.

The systemic form is a disease that is completely incurable. With the help of properly selected therapy, timely diagnosis, compliance with all the prescriptions of doctors, it is possible to improve the quality of life, reduce the negative impact on the body, and extend the time of remission. The disease can affect only one system, for example, the joints or the central nervous system, then the remission may be longer.

Lupus - the causes of the disease

What is lupus erythematosus? The main version is disturbances in the functioning of the immune system, as a result of which healthy cells of the body perceive each other as foreign and begin to fight among themselves. The disease lupus, the causes of which have not yet been fully studied, is now widespread. There is a safe variety of the disease - a drug that appears while taking medications and disappears after they are canceled. It can be passed from mother to child at the genetic level.

Lupus erythematosus - symptoms

What is lupus disease? The main symptom is the appearance of a rash, eczema or hives on the face and scalp. If febrile conditions occur with anxiety, fever, pleurisy, weight loss, and joint pain that recur regularly, doctors may refer additional blood tests to help identify the presence of Liebman-Sachs disease.

Lupus erythematosus, symptoms for diagnosis:

• dry mucous membranes, oral cavity;
• scaly rash on the face, head, neck;
• increased sensitivity to sunlight;
• arthritis, polyarthritis;
• blood changes - the appearance of antibodies, a decrease in the number of cells;
• non-healing wounds in the mouth and on the lips;
• serositis;
• convulsions, psychosis, depression;
• discoloration of fingertips, ears;
• Rein's syndrome - numbness of the extremities.

How does lupus progress?

There are two main forms of the disease, the development and diagnosis of which differ. Discoid is expressed only in skin disease of varying severity. How does systemic lupus proceed? The disease affects the internal organs, the cardiovascular system, joints, and the central nervous system. According to research results, life expectancy from the moment of the first diagnosis is about 20-30 years, women are more often ill.

Lupus treatment

Lupus - what is this disease? To clarify and make a diagnosis, an extended examination of the patient is carried out. A rheumatologist is engaged in treatment, which determines the presence of SLE, the severity of damage to the body, its systems, and complications. How to treat lupus erythematosus? Patients undergo treatment throughout their lives:

1. Immunosuppressive therapy - oppression and suppression of one's own immunity.
2. Hormone therapy - maintaining hormone levels with the help of drugs for the normal functioning of the body.
3. Taking anti-inflammatory drugs.
4. Treatment of symptoms, external manifestations.
5. Detoxification.

Is lupus erythematosus contagious?

The appearance of a bright red rash causes dislike among others, fear of infection, repels from the sick: lupus, is it contagious? There is only one answer - it is not contagious. The disease is not transmitted by airborne droplets, the mechanisms of its occurrence are not fully understood, doctors say that heredity is the main factor in its occurrence.

Video: lupus disease - what is it

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 are well understood. 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 cycle of autoimmune diseases - diseases in which the body begins to fight with 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”. Some of the most common diseases will be covered in separate publications. To maintain objectivity, we invited Doctor of Biological Sciences, Corr. RAS, Professor of the Department of Immunology, Moscow State University Dmitry Vladimirovich Kuprash. In addition, each article has its own reviewer, delving into all the nuances in more detail.

The reviewer of this article was Olga Anatolyevna Georginova, Candidate of Medical Sciences, therapist-rheumatologist, assistant of 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 a febrile fever (a temperature above 38.5 ° C), and it is this symptom that serves as the reason for him to go to the doctor. His joints swell and hurt, his whole body “aches”, the lymph nodes enlarge and cause discomfort. The patient complains of rapid fatigue and increasing weakness. Other symptoms reported at the appointment include mouth ulcers, alopecia, and gastrointestinal disturbances. Often the patient suffers from excruciating headache, depression, severe fatigue. His condition negatively affects his working capacity and social life. Some patients may even present with affective disorders, cognitive impairment, psychoses, movement disorders, and myasthenia gravis.

Not surprisingly, Josef Smolen of the Vienna City General Hospital (Wiener Allgemeine Krankenhaus, AKH) called systemic lupus erythematosus “the most complex disease in the world” at the 2015 congress dedicated to this disease.

In order to assess the activity of the disease and the success of treatment, about 10 different indices are used in clinical practice. With their help, you can track changes in the severity of symptoms over a period of time. Each violation is assigned a certain 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 (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 the classification of V.A. Nasonova.

Main targets of the disease

Some tissues are more affected by autoreactive antibody attacks than others. In SLE, the kidneys and the cardiovascular system are particularly affected.

Autoimmune processes also disrupt the functioning of blood vessels and the heart. According to conservative estimates, every tenth death from SLE is caused by circulatory disorders that have developed as a result of systemic inflammation. The risk of ischemic stroke in patients with this disease is doubled, the likelihood of intracerebral hemorrhage - three times, and subarachnoid - almost four times. Survival after stroke is also much worse than in the general population.

The set 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 temperature and cognitive impairment. Thrombosis and severe organ damage, such as end-stage renal disease, can be added to this. Because of these different manifestations, SLE is called a disease with a thousand faces.

Family planning

One of the most important risks imposed by SLE is the numerous complications during pregnancy. The vast majority of patients are young women of childbearing age, so family planning, pregnancy management and fetal monitoring are now of great importance.

Before the development of modern methods of diagnosis and therapy, a mother's disease often negatively affected the course of pregnancy: conditions that threatened the woman's life arose, pregnancy often ended in intrauterine fetal death, premature birth, and preeclampsia. Because of this, for a long time, doctors strongly discouraged women with SLE from having children. In the 1960s, women lost a fetus in 40% of cases. 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 the remission of the disease, since the survival of the mother, the success of pregnancy and childbirth depends on the activity of the disease in the 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 as a necessary step,.

In rare cases now, a woman finds out that she has SLE while 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.

Approximately one in 20,000 children develops neonatal lupus- passively acquired autoimmune disease, known for more than 60 years (frequency of cases is given for the USA). It is mediated by maternal antinuclear autoantibodies to Ro/SSA, La/SSB or U1-ribonucleoprotein antigens. The presence of SLE in the mother is not at all necessary: ​​only 4 out of 10 women who give 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 body of mothers.

The exact mechanism of damage to the tissues of the child is still unknown, and most likely it is more complicated than just the penetration of maternal antibodies through the placental barrier. The prognosis for the health of the newborn 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 already noticeable at birth, in others they develop within a few 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. Much of the disease burden is social, and it can create a vicious circle of increased symptoms.

So, regardless of gender and ethnicity, poverty, low level of education, lack of health insurance, insufficient social support and treatment contribute to the aggravation of the patient's condition. This, in turn, leads to disability, loss of working capacity and a further decrease in social status. All this significantly worsens the prognosis of the disease.

It should not be discounted that the treatment of SLE is extremely expensive, and the costs directly depend on the severity of the disease. To direct costs include, for example, the cost of inpatient treatment (time spent in hospitals and rehabilitation centers and related procedures), outpatient treatment (treatment with prescribed mandatory and additional medicines, doctor visits, laboratory tests and other tests, ambulance calls), surgical operations, transportation to medical facilities and additional medical services. According to 2015 estimates, in the United States, a patient spends an average of $33,000 per year on all of the above items. If he developed lupus nephritis, then the amount more than doubles - up to $ 71 thousand.

indirect costs may even be higher than the direct ones, since they include loss of working capacity and disability due to illness. Researchers estimate the amount of such losses at $ 20,000.

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 deal with the treatment of this disease. One of the largest institutions where patients can seek help is the Research Institute of Rheumatology. 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 Evgeny Lvovich Nasonov recalls, at first his mother, Valentina Alexandrovna Nasonova, who worked in the department of rheumatology, almost every day came home in tears, since four out of five patients died on her hands. Fortunately, this tragic trend has been overcome.

Assistance to patients with SLE is also provided in the department of rheumatology of the Clinic of Nephrology, Internal and Occupational Diseases named after E.M. Tareev, Moscow city rheumatological center, DGKB im. PER. Bashlyaeva DZM (Tushino Children's City Hospital), the Scientific Center for Children's Health of the Russian Academy of Medical Sciences, the Russian Children's Clinical Hospital and the Central Children's Clinical Hospital of the FMBA.

However, even now it is very difficult to get sick with SLE in Russia: the availability of the latest biological preparations for the population leaves much to be desired. The cost of such therapy is about 500-700 thousand rubles a year, and the medication is long-term, by no means limited to one year. At the same time, such treatment does not fall into 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.

Now therapy with biological preparations is used in the Research Institute of Rheumatology. At first, the patient receives them for 2-3 weeks while he is in the hospital - the CHI covers these costs. After discharge, he needs to submit an application at the place of residence for additional drug provision to the regional department of the Ministry of Health, and the final decision is made by the local official. Often his answer is negative: in a number of regions, patients with SLE are not interested in the local health department.

At least 95% of patients have autoantibodies, recognizing fragments of the body's own cells as foreign (!) And therefore dangerous. Not surprisingly, the central figure in the pathogenesis of SLE is considered B cells producing autoantibodies. These cells are the most important part of adaptive immunity, which have 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 their own cells in the body. As a result, they generate many autoantibodies that are directed to nuclear, cytoplasmic and membrane antigens contained in blood plasma. As a result of the binding of autoantibodies and nuclear material, immune complexes, which are deposited in the tissues and are not effectively removed. Many of the clinical manifestations of lupus are the result of this process and subsequent organ damage. The inflammatory response is exacerbated by B cells secreting about inflammatory cytokines and present to T-lymphocytes not foreign antigens, but self-antigens.

The pathogenesis of the disease is also associated with two other simultaneous events: with an increased level apoptosis(programmed cell death) of lymphocytes and with deterioration in the processing of garbage material that occurs during autophagy. Such “littering” of the body leads to inciting an immune response in relation to its own cells.

Recent studies show that autophagy is important for the normal course of many immune responses: for example, for the maturation and operation of immune system cells, pathogen recognition, processing and antigen presentation. There is now more and more evidence that autophagic processes are associated with the onset, course, and severity of SLE.

It was shown that in vitro macrophages of SLE patients take up less cellular debris compared to macrophages of healthy controls. Thus, with unsuccessful utilization, 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 features listed above, 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 number of type I interferons affects the activity of immune cells, which leads to a malfunction of the immune system.

Figure 3. Current understanding of the pathogenesis of SLE. One of the main causes of the clinical symptoms of SLE is the deposition in tissues of immune complexes formed by antibodies that have bound fragments of the nuclear material of cells (DNA, RNA, histones). This process provokes a strong inflammatory reaction. In addition, with an increase in apoptosis, netosis, and a decrease in the efficiency of autophagy, unutilized cell fragments become targets for cells of the immune system. Immune complexes via receptors FcγRIIa enter the plasmacytoid dendritic cells ( pDC), where the nucleic acids of the complexes activate Toll-like receptors ( TLR-7/9) , . Activated in this way, pDC begin a powerful production of type I interferons (incl. IFN-α). These cytokines, in turn, stimulate the maturation of monocytes ( Mo) 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 cytokines BAFF (stimulator of B-cells, promoting their maturation, survival and production of antibodies) and APRIL (inducer of cell proliferation). All this leads to an increase in the number of immune complexes and an even more powerful activation of pDC - the circle closes. Abnormal oxygen metabolism is also involved in the pathogenesis of SLE, which increases inflammation, cell death, and the influx of self-antigens. In many ways, this is the fault of mitochondria: disruption of their work leads to increased formation of reactive oxygen species ( ROS) and nitrogen ( RNI), deterioration of the protective functions of 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 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 the surrounding tissues, contribute to the constant influx of autoantigens and the specific suicide of neutrophils - netose(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 reticular structures are composed predominantly of the main lupus autoantigens. Interaction with the latter antibodies makes it difficult for the body to clear these traps and increases the production of autoantibodies. This is how a vicious circle is formed: increasing tissue damage during the progression of the disease entails an increase in the amount of ROS, which destroys tissues 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 usually do not encounter most of the body's own antigens because the potential self-antigens reside within cells and are not presented to lymphocytes. After autophagic death, the remnants of dead cells are quickly utilized. However, in some cases, for example, with an excess of reactive oxygen and nitrogen species ( ROS and RNI), the immune system encounters self-antigens "nose to nose", which provokes the development of SLE. For example, under the influence of ROS, polymorphonuclear neutrophils ( PMN) are subjected to netose, and a "network" is formed from the remnants of the cell (eng. 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 trigger 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 is guilty?

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 the hereditary predisposition to the disease. SLE has not escaped this either - which is not surprising, because the incidence varies greatly by gender and ethnicity. Women suffer from this disease about 6-10 times more often than men. Their peak incidence occurs at 15–40 years, that is, at childbearing age. Ethnicity is associated with prevalence, disease course, and mortality. For example, a "butterfly" rash is typical of 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 in them. Discoid lupus is also more common in dark-skinned people.

These facts indicate that genetic predisposition may play an important role in the etiology of SLE.

To clarify this, the researchers used the method genome-wide association search, or GWAS, which allows you to correlate thousands of genetic variants with phenotypes - in this case with the manifestations of the disease. Thanks to this technology, more than 60 loci of predisposition to systemic lupus erythematosus have been identified. They can be conditionally 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 cell residues. 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 link 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 shared by SLE and other autoimmune diseases (Figure 5).

Genetic data could be used to determine the risk of developing SLE, its diagnosis or treatment. This would be extremely useful in practice, because due to the specific nature of the disease, it is not always possible to identify it by the patient's first complaints and clinical manifestations. The selection of treatment also takes some time, because patients respond differently to therapy - 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 certain 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, they, according to research, make a huge contribution to the development of SLE.

Unlike the genome epi the genome is relatively easy to modify under the influence of external factors. Some believe that without them, SLE may not develop. The most obvious of these is ultraviolet radiation, since patients often develop redness and rashes on their skin after exposure to sunlight.

The development of the disease, apparently, can provoke and viral infection. It is possible that in this case, autoimmune reactions occur 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 is in the focus of research. However, in most cases, scientists find it difficult to name the "names" of specific culprits. It is assumed that autoimmune reactions are not provoked by specific viruses, but by the general mechanisms of combating this type of pathogens. For example, the activation pathway for type I interferons is common in response to viral invasion and in the pathogenesis of SLE.

Factors such as smoking and drinking, but their influence is ambiguous. It is likely that smoking can increase the risk of developing the disease, exacerbate it and increase organ damage. Alcohol, on the other hand, according to some reports, reduces the risk of developing SLE, but the evidence is quite contradictory, and this method of protection against the disease is best not to use.

There is not always a clear answer regarding the influence occupational risk factors. While exposure to silica has been shown to trigger the development of SLE, according to a number of studies, exposure to metals, industrial chemicals, solvents, pesticides, and hair dyes has yet to be definitively answered. Finally, as mentioned above, lupus can be provoked drug use: The most common triggers are chlorpromazine, hydralazine, isoniazid, and procainamide.

Treatment: past, present and future

As already mentioned, there is still no cure for “the most complex disease in the world”. The development of a drug is hampered by the multifaceted pathogenesis of the disease, involving different parts of the immune system. However, with a competent individual selection of maintenance therapy, deep remission can be achieved, and the patient can live with lupus erythematosus just like with a chronic disease.

Treatment for various changes in the patient's condition can be adjusted by a doctor, more precisely, 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, a hematologist, a dermatologist, and a neurologist. In Russia, a patient with SLE first of all goes to a rheumatologist, and depending on the damage to the systems and organs, he may need 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 now in development, while others have shown their failure at the trial stage. Therefore, non-specific drugs are most widely used in clinical practice.

Standard treatment includes several types of medications. First of all, write out immunosuppressants- in order to suppress the excessive activity of the immune system. The most commonly used of these are cytotoxic 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, clones of activated lymphocytes). It is clear that such therapy has many dangerous side effects.

In the acute phase of the disease, patients usually take corticosteroids- non-specific anti-inflammatory drugs that help calm the most violent flurries of autoimmune reactions. They have been used in the treatment of SLE since the 1950s. Then they transferred 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.

With exacerbation of SLE since 1976, it is also used pulse therapy: the patient receives impulsively high doses of methylprednisolone and cyclophosphamide. Of course, over 40 years of use, the scheme of such therapy has changed a lot, but it 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 hypertension and those suffering from systemic infections. In particular, the patient may develop metabolic disorders and change behavior.

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 a long-term reduction in disease activity, reduces organ and tissue damage, and improves pregnancy outcome. In addition, the drug reduces the risk of thrombosis - and this is extremely important, given the complications that occur 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 a barrel of honey. In rare cases, retinopathy develops in response to this therapy, and patients with severe renal or hepatic insufficiency are at risk of toxic effects associated with hydroxychloroquine.

Used in the treatment of lupus and newer, targeted drugs(Fig. 5). The most advanced developments targeting B cells are 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 infection by viruses and exposure to ultraviolet light. 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 DC. This is how an autoimmune reaction begins, the synthesis of autoantibodies starts. Now many biological preparations are being studied - drugs that affect the regulation of the body's immune components. Targeting the innate immune system anifrolumab(antibody to IFN-α receptor), sifalimumab and rontalizumab(antibodies to IFN-α), infliximab and etanercept(antibodies to tumor necrosis factor, TNF-α), sirucumab(anti-IL-6) and tocilizumab(anti-IL-6 receptor). Abatacept (cm. text), belatacept, AMG-557 and IDEC-131 block co-stimulatory molecules of T-cells. Fostamatinib and R333- inhibitors of splenic tyrosine kinase ( 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 antilupus therapy is type I interferons, which have already been discussed above. Several antibodies to IFN-α have already shown promising results in SLE patients. Now the next, third, phase of their testing is planned.

Also, of 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, for example, etanercept and infliximab- specific antibodies to tumor necrosis factor, TNF-α.

Conclusion

Systemic lupus erythematosus remains the most difficult test for the patient, a difficult task for the doctor and an underexplored area for the scientist. However, the medical side of the issue 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 the methods of providing information, specialized mobile applications, platforms with accessible information significantly improve the quality of life of people with SLE.

A lot of help in this matter and patient organizations- public associations of people suffering from some kind of 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, research, education, support and assistance. Its primary goals include reducing the time to diagnosis, providing patients with safe and effective treatment, and increasing access to treatment and care. In addition, the organization stresses the importance of educating medical staff, bringing concerns to the authorities and raising social awareness about systemic lupus erythematosus.

The global burden of SLE: prevalence, health disparities and socioeconomic impact. Nat Rev Rheumatol. 12 , 605-620;

Systemic lupus erythematosus (SLE) is a severe autoimmune disease in which the human immune system produces antibodies that destroy the DNA of its own healthy cells that form the basis of connective tissue. Connective tissue is everywhere in the body. Therefore, the inflammatory process caused by lupus affects almost all systems and internal organs of a person (skin, blood vessels, joints, brain, lungs, kidneys, heart). The symptoms of lupus are very varied. It can masquerade as other diseases, so in many cases it is difficult to make a correct diagnosis.

A characteristic symptom of lupus is a butterfly-shaped rash on the cheeks and bridge of the nose. In the Middle Ages, it was believed that these manifestations resemble the places of wolf bites, hence the name of the disease. The systemic disease lupus erythematosus is considered incurable, but modern medicine is able to control its symptoms and, with appropriate treatment, support patients and prolong their lives. The disease proceeds with difficult to predict periods of exacerbations and remissions and affects mainly women aged 15 to 45 years.

In the frequency of occurrence of lupus, not only sex, age, but also racial characteristics can be traced. So, men get lupus 10 times less often than the fairer sex, the main peak of incidence occurs at the age of 15 to 25 years, and according to American researchers, SLE more often affects blacks and Asians.

Children are also susceptible to this disease, at an early age SLE is much more severe than in adults and leads to serious damage to the kidneys and heart. We will tell you more about the causes that contribute to the development of the disease, its symptoms and methods of treating the disease.

Systemic lupus erythematosus is a complex multifactorial disease, the exact cause of which is still unknown. Currently, most scientists are inclined to the viral theory of the origin of the disease, according to which the body begins to produce a large number of antibodies to certain groups of viruses.

At the same time, it is noted that not all patients with chronic viral infection develop lupus, but only those who have a genetic predisposition to this disease. Researchers believe that heredity plays an important role, and there are specific genes that increase the body's susceptibility to this pathology.

It is also noted that lupus is not a consequence of immunodeficiency, a type of oncological lesions or a contagious infectious disease that can be transmitted from person to person. In addition to the main cause, there are many concomitant factors that can provoke the development of the disease:

• The onset of the disease or the exacerbation of already existing symptoms causes excessive ultraviolet radiation.
• Hormonal changes in the body (especially in women in the reproductive or menopausal period).
• Infectious and cold diseases.
• Bad habits. Smoking can not only provoke the onset of the disease, but also complicate its course due to damage to blood vessels.
• The use of drugs. The development of lupus symptoms can provoke the use of drugs such as antibiotics, hormonal drugs, anti-inflammatory and antifungal drugs, antihypertensive, anticonvulsant and antiarrhythmic drugs.

Sometimes lupus erythematosus develops as a result of adverse environmental factors and interactions with certain metals and insecticides.

The course of the disease can be different:

1. acute form. It is characterized by a sudden onset and the most severe course. It is characterized by rapid progression, a sharp increase in symptoms and damage to vital organs within 1-2 months. This form of lupus is difficult to treat and can be fatal within 1-2 years.
2. Subacute form. Most often, the disease occurs in this form, which is characterized by a calmer course and is accompanied by a slow increase in symptoms. From the first manifestations to a detailed picture of the disease with damage to internal organs, an average of 1.5 to 3 years passes.
3. Chronic form. It is considered the most favorable option in terms of therapy. It is characterized by an undulating nature of the course, periods of remission are replaced by exacerbations that can be treated with drugs. This form of lupus can last for a long time, which improves the prognosis and increases the life expectancy of the patient.

Symptoms

The clinical picture of the disease can be different - from minimal symptoms to severe, associated with damage to vital organs. Symptoms of the disease can occur suddenly or develop gradually.

Common symptoms of lupus erythematosus include:

• Fatigue (chronic fatigue syndrome)
• unexplained fever
• Painful joint swelling, muscle pain
• Various skin rashes
• Pain in chest when breathing deeply
• Lack of appetite, weight loss

A characteristic butterfly-shaped rash appears on the cheeks and bridge of the nose. In addition, red rashes can appear on any part of the body: on the chest, arms, shoulders. Other symptoms of lupus include increased sensitivity to sunlight, development of anemia, chest pain, hair loss, and reduced blood flow to the fingers and toes from exposure to cold.

Patients have general weakness, irritability, headaches, insomnia, depression. In some patients, only the joints and skin may be affected, while in others, the disease affects many organs, manifesting itself with severe symptoms. Depending on which organs and systems of the body have been affected, the manifestations of the disease depend.

With the appearance of a characteristic rash, fever, joint pain and severe weakness, it is urgent to seek medical advice, undergo an examination and establish the cause of the ailment. In many patients, the initial symptoms of lupus erythematosus are mild, but it should be understood that this disease is chronic and over time, as the disease progresses, the symptoms will become very serious, which threatens with serious consequences and death.

In most cases, modern medicine is able to control the course of the disease and prevent serious complications caused by damage to internal organs. Adequate drug treatment significantly improves the prognosis and allows you to maintain normal health for a long time. The prognosis for life in systemic lupus is unfavorable, but recent advances in medicine and the use of modern drugs give a chance to prolong life. Already more than 70% of patients live more than 20 years after the initial manifestations of the disease.

At the same time, doctors warn that the course of the disease is individual, and if some patients develop SLE slowly, then in other cases, the disease can develop rapidly. Another feature of systemic lupus erythematosus is the unpredictability of exacerbations, which can occur suddenly and spontaneously, which threatens with serious consequences.

Most patients maintain daily physical activity, but in some cases it is necessary to stop active work due to severe weakness, fatigue, joint pain, mental disorders and other symptoms. Life expectancy will largely depend on the degree of damage to vital organs (lungs, heart, kidneys). In the recent past, patients with systemic lupus died at a young age, now the use of modern effective drugs makes it possible to deal with severe manifestations of the disease and predict a normal life expectancy.

Diagnostics

Systemic lupus erythematosus is characterized by multiple manifestations that are similar to those of other diseases. Therefore, the recognition of the disease is extremely difficult and it may take a long time to make an accurate diagnosis. Scientists have developed 11 main criteria, the presence of which will indicate the development of the disease. Experienced doctors are able to make the correct diagnosis based on only 4 characteristic signs.

Characteristic criteria:

1. Rash on the face in the shape of a butterfly.
2. Discoid manifestations - a scaly, coin-like rash on the face, chest, arms, neck, after the disappearance of which scars remain on the skin.
3. Rashes on the skin that appear under the influence of sunlight (photosensitivity).
4. The appearance of painless sores on the mucous membranes of the mouth or nose.
5. Articular pain, swelling and impaired mobility of 2 or more peripheral joints.
6. Deviations in the analysis of urine, expressed in an increase in protein, kidney cells and red blood cells.
7. Inflammatory process affecting the mucous membrane surrounding the heart (pericarditis) or lungs (pleurisy).
8. Disorders of the nervous system, in which causeless convulsive seizures or psychoses are possible.
9. A change in the composition of the blood associated with an increase in the number of leukocytes, platelets and erythrocytes.
10. Immunological disorders that contribute to high autoimmune activity and increase the risk of secondary infection.
11. The appearance of specific antibodies to the cell nucleus (such autoantibodies act against the nuclei of their own cells, mistaking them for foreign ones).

If clinical symptoms of SLE are present and the test for the presence of antibodies is positive, this will definitely indicate the presence of the disease and further examination is usually not required. Additionally, examinations can be prescribed to detect damage to the kidneys (biopsy), heart and lungs (CT, MRI).

Therapy of the disease is long and complicated, the main efforts of doctors are aimed at alleviating the symptoms and stopping autoimmune and inflammatory processes. To date, it is impossible to completely recover from lupus, with intensive treatment, remission can be achieved, but it is usually short-lived and is soon replaced by an exacerbation. Of great importance is the moral support of patients and explaining to them the features of therapy and the course of the disease. The patient receives recommendations on diet, reduction of psycho-emotional stress and treatment of concomitant infections.

Drug therapy for systemic lupus erythematosus will depend on the activity of the process and the severity of symptoms, the treatment process should be constantly monitored by a doctor. When the manifestations subside, it is necessary to adjust the treatment regimen, reducing the dose of drugs, with the development of an exacerbation, on the contrary, increase the dosage.

With a mild form of the disease, non-steroidal anti-inflammatory drugs (NSAIDs) and salicylates are used to eliminate the symptoms of myalgia, arthritis. It should be borne in mind that long-term use of NVPS can cause damage to the kidneys, stomach and contribute to the development of serous meningitis.

With severe weakness, fatigue, skin lesions, antimalarial drugs (hydroxychloroquine, chloroquine) are used. The side effect of taking these drugs is associated with eye damage (retinopathy, myopathy), so the patient should be examined by an ophthalmologist at least once a year.

The main treatment for lupus remains corticosteroid therapy, which is used even at the initial stage of the disease. Corticosteroids are potent drugs, they have a pronounced anti-inflammatory effect, but their long-term use in high doses provokes serious side effects.

With a small activity of the disease, glucocorticosteroids are prescribed for oral administration in moderate doses, with the gradual withdrawal of the drug to the minimum maintenance dosage. With the activity of the process and severe life-threatening complications, high doses are prescribed to take drugs such as Prednisolone, Dexamethasone, Methylprednisolone. They are taken until the process subsides, usually for 4-10 weeks. In case of serious exacerbations of the disease, pulse therapy with Methylprednisolone administered intravenously gives a good effect.

On the part of the doctor, control over the development of side effects and their prevention is necessary, since with long-term treatment and the use of large doses of glucocorticoids, osteoporosis, bone necrosis, diabetes mellitus, arterial hypertension, and infectious complications may develop.

In combination with hormonal therapy, cytostatic drugs are used, which can reduce the frequency and severity of exacerbations and reduce the dose of glucocorticoids. Cytostatic drugs (Cyclophosphamide, Azathioprine, Methotrexate) are prescribed in case of severe lesions of the central nervous system and renal failure.

With the development of complications, antibacterial, antifungal (with candidiasis) drugs are used. With the development of diabetes, a diet is prescribed, insulin preparations are administered. When a tuberculosis infection is attached, they are treated with anti-tuberculosis drugs, with damage to the gastrointestinal tract, a course of anti-ulcer therapy is carried out. Patients with severe complications, accompanied by damage to vital organs, undergo plasmapheresis procedures and use program hemodialysis.

Systemic disease lupus erythematosus It is a serious illness leading to disability and threatening death. But still, modern medicine successfully fights the manifestations of the disease and is able to achieve periods of remission, during which the patient can live a normal life. SLE patients should strictly adhere to all the recommendations of the attending physician, inform him of all changes in their condition and avoid negative factors that can aggravate the course of the disease.

Folk remedies

Nature is rich in natural substances that can support and restore the body by suppressing the inflammatory process.

Remember that before starting treatment with the use of folk recipes, you need to consult a doctor, this will save you from unwanted complications.

Systemic lupus erythematosus is a disease that has one of the most complex pathogenesis and still has not been clarified etymology, defined in the group of autoimmune diseases. One of the variants of the course of lupus erythematosus is Liebman-Sachs disease, in which the heart is damaged, but in general the clinical manifestations of the disease are the same. The disease has gender differences, which are explained by the distinctive features of the structure of the female body. Women are the main risk group. To protect yourself from pathology, you should know the main factors that contribute to the onset of the disease.

It is difficult for specialists to establish a specific cause that explains the development of lupus. Theoretically, it is possible to determine the genetic predisposition and hormonal disorders in the body as one of the main root causes of systemic lupus. However, a combination of certain factors can also affect the formation of the disease.

Possible factors provoking lupus erythematosus

Causes of lupus erythematosus in women

It is impossible to reliably identify the reasons that explain the frequent defeat of women by this pathology, since scientists have not fully studied the etymology of the disease. Despite this, a number of factors have been established that predispose to the development of lupus:

1. A visit to the solarium with increased regularity, exposure to open sunlight.
2. The period of pregnancy and postpartum.
3. Stressful situations that appear with a certain regularity (lead to hormonal disorders).

Attention! Additionally, the manifestation of lupus in women can be affected by an allergic reaction of the body to certain foods, unfavorable ecology and genetic predisposition.

Causes of lupus erythematosus in men

There are even fewer root causes explaining the development of lupus in men, but their nature is similar to the provocative factors of the disease in women - this is hormonal instability in the body, frequent stressful situations. So, it has been established that the male body is vulnerable to lupus erythematosus when testosterone levels are reduced, while prolactin is overestimated. In addition to these reasons, all of the above general factors should be added, which predispose to the disease, regardless of gender differences.

It is important! The course of the disease in men may differ from the symptoms in women, since different systems of the body are affected. According to statistics, the joints are damaged. It is remarkable that in men against the background of pathology, additional diseases develop, such as nephritis, vasculitis, and hematological disorders.

At-risk groups

1. The presence of an infectious chronic disease.
2. immunodeficiency syndrome.
3. Damage to the skin with dermatitis of various nature.
4. Frequent SARS.
5. The presence of bad habits.
6. Interruptions in the hormonal background.
7. Too much ultraviolet rays.
8. Pathology of the endocrine system.
9. Pregnancy period, postpartum period.

How the disease develops

When the protective functions of immunity in a healthy body are reduced, the risk of activation of antibodies directed against their cells increases. Based on this, the internal organs and all tissue structures of the body begin to be perceived by the immune system as foreign bodies, therefore, the body's self-destruction program is activated, leading to typical symptoms.

The pathogenic nature of such a reaction of the body leads to the development of various inflammatory processes that begin to destroy healthy cells.

Reference! Basically, with pathological lupus, blood vessels and connective tissue structures suffer.

The pathological process that occurs under the influence of lupus erythematosus leads to a violation of the integrity of the skin in the first place. In places of localization of the lesion, blood circulation is reduced. The progression of the disease leads to the fact that not only the skin, but also the internal organs suffer.

Symptomatic signs

Symptoms of the disease directly depend on the location of the lesion and the severity of the disease. Experts identify common signs confirming the diagnosis:

• constant feeling of malaise and weakness;
• deviations from normal temperature indicators, sometimes fever;
• if there are chronic ailments, then their course is aggravated;
• the skin is affected by scaly red spots.

The initial stages of the pathology do not differ in pronounced symptoms, however, there may be periods of exacerbations followed by remissions. Such manifestations of the disease are very dangerous, the patient is mistaken, considering the absence of symptoms as a recovery, therefore he does not seek competent help from a doctor. As a result, all systems in the body are gradually affected. Under the influence of irritating factors, the disease progresses intensively, manifesting itself with more severe symptoms. The course of the disease in this case is complicated.

Late symptoms

After years of development of the pathology, other symptoms may be observed. For example, hematopoietic organs may be affected. Multiple organ manifestations are not excluded, which include the following changes:

1. Inflammatory process that affects the kidneys.
2. Violations in the activity of the brain and central nervous system (resulting in psychosis, frequent headaches, memory problems, dizziness, convulsions).
3. Inflammatory processes of blood vessels (diagnosed as vasculitis).
4. Illnesses associated with the blood (signs of anemia, blood clots).
5. Heart disease (signs of myocarditis or pericarditis).
6. Inflammatory processes that affect the lungs (cause pneumonia).

Carefully! If some of these symptoms appear, then it is urgent to go to a specialist. Lupus erythematosus is a rather dangerous disease, therefore it requires urgent therapy. Self-medication is strictly prohibited.

How is the treatment process

After a detailed examination through immunomorphological examination, luminescent diagnostics, an accurate diagnosis is established. For a complete understanding of the clinical picture, it is necessary to examine all internal organs. Then the specialist directs all actions to eliminate the chronic infection.

An approximate treatment regimen includes the following manipulations:

1. The introduction of quinoline drugs (for example, Plaquenol).
2. The use of corticosteroid drugs in low doses (Dexamethasone).
3. Reception of vitamin and mineral complexes (in particular, vitamins of group B).
4. Reception of nicotinic acid.
5. The use of immunocorrective drugs (Taktivin).
6. External treatment, which involves percutaneous chipping. To do this, you can use Hingamine.
7. Additionally, external ointments of a corticosteroid nature (Sinalar) should be used.
8. Ulcerative skin manifestations require the use of ointments based on antibiotics and various antiseptic agents (Oxycort).

It should be noted that lupus erythematosus must be treated in a hospital setting. In this case, the course of therapy will be very long and continuous. Treatment will consist of two directions: the first is aimed at eliminating the acute form of manifestation and severe symptoms, the second is the suppression of the disease as a whole.

You can learn more about the disease in the video.

Video - Information about the disease lupus erythematosus

Video - Lupus erythematosus: ways of infection, prognosis, consequences, life expectancy

Lupus (systemic lupus erythematosus, SLE) is an autoimmune disease in which the human immune system attacks the host's connective tissue cells as foreign.

Connective tissue is almost everywhere, and most importantly - in the ubiquitous vessels.

The inflammation caused by lupus can affect a wide variety of organs and systems, including the skin, kidneys, blood, brain, heart, and lungs. Lupus is not transmitted from person to person.

Science does not know the exact cause of lupus, like many other autoimmune diseases. These diseases are most likely caused by genetic disorders in the immune system that make it possible for it to produce antibodies against its own host.

Lupus is difficult to diagnose because its symptoms are so varied and it can masquerade as other diseases. The most distinguishing feature of lupus is facial erythema that resembles butterfly wings spread across both cheeks (butterfly erythema). But this symptom is not found in all cases of lupus.

There is no cure for lupus, but its symptoms can be controlled with medication.

Causes and risk factors for lupus

There are many possible causes of lupus:

Exposure to ultraviolet rays (sunlight) can cause lupus or worsen its symptoms.
. Female sex hormones do not cause lupus, but they influence its course. Among them may be high-dose preparations of female sex hormones for the treatment of gynecological diseases. But this does not apply to taking low-dose oral contraceptives (OCs).
. Smoking is considered one of the risk factors for lupus, which can cause the disease and exacerbate its course (especially vascular damage).
. Some medications can aggravate lupus (in each case, you need to read the instructions for the drug).
. Infections such as cytomegalovirus (CMV), parvovirus (erythema infectiosum), and hepatitis C can also cause lupus. Epstein-Barr virus is associated with lupus in children.
. Chemicals can cause lupus. Among these substances in the first place is trichlorethylene (a narcotic substance used in the chemical industry). Hair dyes and fixatives, previously considered the cause of lupus, are now fully justified.

The following groups of people are more likely to develop lupus:

Women get lupus more often than men.
. Africans are more likely to get lupus than whites.
. People between the ages of 15 and 45 are most commonly affected.
. Heavy smokers (according to some studies).
. People with a burdened family history.
. People on a chronic basis with medications associated with a risk of lupus (sulfonamides, some antibiotics, hydralazine).

Drugs that cause lupus

Drugs most commonly associated with lupus include calcium channel blockers, ACE inhibitors, TNF-alpha antagonists, thiazide diuretics, and terbinafine (an antifungal drug).

The following groups of drugs are commonly associated with drug-induced SLE:

Antibiotics: minocycline and isoniazid.
. Antipsychotic drugs: chlorpromazine.
. Biological agents: interleukins, interferons.
. Antihypertensive drugs: methyldopa, hydralazine, captopril.
. Hormonal preparations: leuprolide.
. Inhalation drugs for COPD: tiotropium bromide.
. Antiarrhythmic drugs: procainamide and quinidine.
. Anti-inflammatory: sulfasalazine and penicillamine.
. Antifungals: terbinafine, griseofulvin and voriconazole.
. Hypocholesterolemic: lovastatin, simvastatin, atorvastatin, gemfibrozil.
. Anticonvulsants: valproic acid, ethosuximide, carbamazepine, hydantoin.
. Other drugs: timolol eye drops, TNF-alpha inhibitors, sulfa drugs, high-dose female sex hormone preparations.

Additional list of drugs that cause lupus:

Amiodarone.
. Atenolol.
. Acebutolol.
. Bupropion.
. Hydroxychloroquine.
. Hydrochlorothiazide.
. Glyburide.
. Diltiazem.
. Doxycycline.
. Doxorubicin.
. Docetaxel.
. Gold and its salts.
. Imiquimod.
. Lamotrigine.
. Lansoprazole.
. Lithium and its salts.
. Mephenytoin.
. Nitrofurantoin.
. Olanzapine.
. Omeprazole.
. Praktolol.
. Propylthiouracil.
. Reserpine.
. Rifampicin.
. Sertalin.
. Tetracycline.
. Ticlopidin.
. Trimethadione.
. Phenylbutazone.
. Phenytoin.
. Fluorouracil.
. Cefepime.
. Cimetidine.
. Esomeprazole.

Sometimes systemic lupus erythematosus is caused by chemicals that enter the body from the environment. This happens only in some people, for some unknown reason.

These chemicals include:

Some insecticides.
. Some metal compounds.
. Eosin (fluorescent liquid found in lipsticks).
. Para-aminobenzoic acid (PABA).

Lupus Symptoms

No two cases of lupus are exactly alike. The symptoms of lupus may occur suddenly or develop gradually, may be temporary or may bother the patient for life. In most patients, lupus is relatively mild, with periodic exacerbations, when the symptoms of the disease become worse, and then subside or disappear altogether.

Symptoms of lupus may include:

Fatigue and weakness.
. Temperature rise.
. Pain, swelling and stiffness of the joints.
. Erythema on the face in the form of a butterfly.
. Skin lesions worse from the sun.
. Raynaud's phenomenon (impaired blood flow in the fingers).
. Breathing problems.
. Chest pain.
. Dry eyes.
. Memory loss.
. Violation of consciousness.
. Headache.

It is almost impossible to assume that you have lupus before a visit to the doctor. Seek advice if you develop an unusual rash, fever, joint pain, fatigue.

Lupus Diagnosis

1. Complete blood count.

In this analysis, the content of erythrocytes, leukocytes, platelets, hemoglobin is determined. Lupus may present with anemia. A low white blood cell and platelet count can also indicate lupus.

2. Determination of the ESR indicator.

The erythrocyte sedimentation rate is determined by how quickly your blood erythrocytes settle in the prepared blood sample to the bottom of the tube. ESR is measured in millimeters per hour (mm/h). A rapid erythrocyte sedimentation rate may indicate inflammation, including autoimmune inflammation, as in lupus. But ESR also rises with cancer, other inflammatory diseases, even with a common cold.

3. Assessment of the functions of the liver and kidneys.

Blood tests can show how well your kidneys and liver are working. This is determined by the amount of liver enzymes in the blood and the level of toxic substances that the kidneys must cope with. Lupus can affect both the liver and kidneys.

4. Urinalysis.

Your urine sample may show an increase in protein or red blood cells. This indicates damage to the kidneys, which can be observed in lupus.

5. Analysis for ANA.

Antinuclear antibodies (ANA) are special proteins produced by the immune system. A positive ANA test may indicate lupus, although it may also be the case with other diseases. If your ANA test is positive, your doctor may order other tests.

6. Chest x-ray.

Obtaining a chest image helps to detect inflammation or fluid in the lungs. This may be a sign of lupus or other diseases that affect the lungs.

7. Echocardiography.

Echocardiography (EchoCG) is a technique that uses sound waves to take a real-time picture of a beating heart. An echocardiogram can reveal heart valve problems and more.

8. Biopsy.

Biopsy, taking a sample of an organ for analysis, is widely used in the diagnosis of various diseases. Lupus often affects the kidneys, so your doctor may order a biopsy of your kidneys. This procedure is performed with a long needle after preliminary anesthesia, so there is nothing to worry about. The resulting piece of tissue will help to identify the cause of your illness.

Lupus treatment

Modern drugs for the treatment of lupus:

1. Non-steroidal anti-inflammatory drugs (NSAIDs).

OTC NSAIDs such as naproxen (Anaprox, Nalgesin, Floginas) and ibuprofen (Nurofen, Ibuprom) may be used to treat inflammation, swelling, and pain caused by lupus. Stronger NSAIDs such as diclofenac (Olfen) are available on prescription. Side effects of NSAIDs include abdominal pain, stomach bleeding, kidney problems, and an increased risk of cardiovascular complications. The latter is especially true for celecoxib and rofecoxib, which are not recommended for the elderly.

2. Antimalarial drugs.

Medicines commonly prescribed to treat malaria, such as hydroxychloroquine (Plaquenil), help control lupus symptoms. Side effects: stomach discomfort and retinal damage (very rare).

3. Corticosteroid hormones.

Corticosteroid hormones are powerful drugs that fight inflammation in lupus. Among them are methylprednisolone, prednisolone, dexamethasone. These drugs are prescribed only by a doctor. They are characterized by long-term side effects: weight gain, osteoporosis, high blood pressure, risk of diabetes and susceptibility to infections. The risk of side effects is higher the higher the doses you use and the longer the course of treatment.

4. Immunosuppressors.

Drugs that suppress the immune system can be very helpful for lupus and other autoimmune diseases. Among them are cyclophosphamide (Cytoxan), azathioprine (Imuran), mycophenolate, leflunomide, methotrexate and others. Possible side effects: susceptibility to infections, liver damage, decreased fertility, risk of many types of cancer. A newer drug, belimumab (Benlysta), also reduces inflammation in lupus. Its side effects include fever, nausea, and diarrhea.

Tips for lupus patients.

If you suffer from lupus, there are several steps you can take to help yourself. Simple measures can make flare-ups less frequent and improve your quality of life.

Try the following:

People with lupus experience constant fatigue, which is different from fatigue in healthy people and does not go away with rest. For this reason, it can be difficult for you to judge when to stop and rest. Develop a gentle daily routine for yourself and follow it.

2. Watch out for the sun.

Ultraviolet rays can cause lupus to flare up, so you should wear cover-ups and avoid walking in hot rays. Choose darker sunglasses and a cream with an SPF of at least 55 (for especially sensitive skin).

3. Eat a healthy diet.

A healthy diet should include fruits, vegetables, and whole grains. Sometimes you will have to endure dietary restrictions, especially if you have high blood pressure, kidney or gastrointestinal problems. Take it seriously.

4. Exercise regularly.

Physical exercise approved by your doctor will help you improve your fitness and recover faster from flare-ups. In the long term, fitness is a reduction in the risk of heart attack, obesity and diabetes.

5. Quit smoking.

Among other things, smoking can worsen the damage to the heart and blood vessels caused by lupus.

Alternative medicine and lupus

Well-known non-traditional treatments for lupus in the West:

1. Dehydroepiandrosterone (DHEA).

Dietary supplements containing this hormone may reduce the dose of steroids that the patient receives. DHEA relieves the symptoms of the disease in some patients.

2. Flax seed.

Flaxseed contains a fatty acid called alpha-linolenic, which can reduce inflammation. Some studies have shown the ability of flax seeds to improve kidney function in lupus patients. Side effects include bloating and abdominal pain.

3. Fish oil.

Fish oil supplements contain omega-3 fatty acids, which may be helpful for lupus. Preliminary studies have shown promising results. Side effects of fish oil include nausea, vomiting, belching, and a fishy taste in the mouth.

4. Vitamin D

There is some evidence that this vitamin improves symptoms in people with lupus. However, scientific data on this issue is very limited.

Complications of lupus

This leads to numerous complications:

1. Kidneys.

Renal failure is one of the main causes of death in lupus patients. Signs of kidney problems include itching all over the body, pain, nausea, vomiting, and swelling.

2. Brain.

If the brain is affected by lupus, the patient may experience headaches, dizziness, behavioral changes, and hallucinations. Sometimes there are seizures, and even a stroke. Many people with lupus have trouble remembering and expressing their thoughts.

3. Blood.

Lupus can cause blood disorders such as anemia and thrombocytopenia. The latter is manifested by a tendency to bleeding.

4. Blood vessels.

With lupus, blood vessels in various organs can become inflamed. This is called vasculitis. The risk of vascular inflammation increases if the patient smokes.

5. Lungs.

Lupus increases the chance of inflammation of the pleura, called pleurisy, which can make breathing painful and difficult.

6. Heart.

Antibodies can attack the heart muscle (myocarditis), the sac around the heart (pericarditis), and large arteries. This leads to an increased risk of heart attack and other serious complications.

7. Infections.

People with lupus become vulnerable to infection, especially as a result of treatment with steroids and immunosuppressants. Most often there are infections of the genitourinary system, respiratory infections. Common pathogens: yeast, salmonella, herpes virus.

8. Avascular necrosis of bones.

This condition is also known as aseptic or non-infectious necrosis. Occurs when the blood supply to the bones decreases, leading to fragility and easy destruction of the bone tissue. Often there are problems with the hip joint, which experiences heavy loads.

9. Complications of pregnancy.

Women with lupus have a high risk of miscarriage. Lupus increases the chance of preeclampsia and preterm birth. To reduce your risk, your doctor may recommend that you not conceive until at least 6 months have passed since your last outbreak.

Lupus is associated with an increased risk of many types of cancer. What's more, some lupus drugs (immunosuppressants) themselves increase this risk.

Konstantin Mokanov