SLE (systemic lupus erythematosus) is a disease currently diagnosed in several million inhabitants of our planet. Patients include the elderly, infants and adults. Doctors have not yet been able to establish the causes of the pathology, although the factors that stimulate the disease have been studied. SLE is not completely curable, but it is not a death sentence either. Measures and methods have been developed to help stabilize the condition of patients and provide them with a long, fulfilling life.
What is SLE: Basics
Some people think the treatment of SLE is futile. The prognosis of this pathology in a patient often causes panic when a person learns that a full recovery cannot be achieved. To make it not so scary, you should understand the essence of the pathological condition. The term is used to denote such an autoimmune disease in which the cells of the body attack other he althy structures, forming aggressive components, lymphocytic clones. This is due to the malfunction of the immune system.systems that, for various reasons, take normal elements as targets.
Currently, among other autoimmune pathologies, SLE is considered one of the most complex. A distinctive feature is the formation of antibodies to the body's DNA. The disease covers almost all tissues and organs, various cells get damaged in the most unpredictable places, which leads to an inflammatory process. The most typical areas of localization of inflammation are the kidneys, heart, blood vessels, connective tissues.
About a hundred years ago, no cure could be offered for symptoms of SLE. The person was considered doomed. Currently, a wide variety of medicines have been developed to increase survival, alleviate manifestations, and reduce internal damage. In total, this helps to improve the quality of life of people with such a diagnosis. Around the beginning of the last century, SLE was the cause of rapid death, by the middle of the century the survival rate reached 50%. Currently, 96% of patients live five years, and 76% live fifteen years. The probability of death is adjusted by gender, ethnicity, place of residence. Black men are the hardest hit by SLE.
Features of terminology
The discrepancy in opinions about the treatment of SLE in Europe, America and Russia is due to some difference in terminology. In particular, in English-language scientific works, not only SLE is called lupus, but also a number of other pathological conditions, that is, there it is a prefabricated term. It most often implies SLE, since this form is the most widelycommon. It must be admitted that about five million people suffer from various types of lupus. In addition to SLE, there are neonatal, medical, and cutaneous varieties.
When skin pathological processes occur only in the skin, so the disease does not go into a systemic form. There are subacute cases, discoid. A drug-induced disease is provoked by drugs, it resembles the course of SLE, but does not require a therapeutic course - it is enough to cancel the drug that provoked the pathology.
The nuances of manifestations
It is possible to suspect that SLE treatment is necessary if the bridge of the nose, cheeks are covered with a rash. The shape of the rash resembles a butterfly, which gave the name of the pathology. However, this manifestation is not observed in 100% of cases. The specific set of symptoms depends on the characteristics of the organism. Even in one patient, the symptoms can gradually change, and the disease itself either weakens or becomes active again. The predominant percentage of symptoms is non-specific, which complicates the diagnosis.
Usually, the need for SLE treatment is identified when a patient goes to the doctor with a non-specific set of symptoms, of which the most pronounced is febrile fever, in which the temperature exceeds 38.5 degrees. On examination, swelling of the joints is visible, this area responds with pain, the body aches. The patient has enlarged lymph nodes, the person quickly gets tired, weakens. Some develop ulcers in the oral cavity, hair falls out, malfunctions of the gastrointestinal tract are observed. Headaches, depressed mental state are possible. All this lowersefficiency, excludes a person from an active social life. Sometimes, against the background of SLE, cognitive failures, psychosis and affective disorders, myasthenia gravis, problems with movement coordination develop.
Disease indexing
Modern methods of treating SLE differ in effectiveness and efficiency, so it was decided to introduce indexing systems to assess the adequacy of the chosen therapy. About a dozen indices have been introduced to monitor the progress of symptoms over a given time period. Each of the violations receives an initial score, and the final summation helps to clearly determine how severe the case is. This evaluation method was first used in the 1980s, and later studies have confirmed its reliability and accuracy.
SLE treatment is practiced in Israel, Russia, America and other countries with sufficient medical capacity. In our country, people with this diagnosis are ready to be admitted to the Moscow State Regional Clinical Center, the Children's Clinical Hospital, and the KNFPZ. Tareeva, RAMS, RCCH, CDKB FMBA. However, such a variety of institutions does not yet indicate an impeccable level of assistance. Unfortunately, the availability of drugs is relatively low, especially those related to the newest and most effective developments. The price of treatment for a year is from 600,000 rubles or more, which is associated with the high cost of medicines. You will have to take medicine for many years.
Past and Present
Currently, SLE is a disease whose treatment is aimed at relieving symptoms. Whereindo not count on a complete recovery. Medications help to control the immune system. A competent set of means is the key to a long-term remission, that is, SLE becomes simply a chronic disease for a person. When the condition changes, the attending physician selects a new course. As a rule, several specialists work with the patient at once - a multidisciplinary team. Attract doctors specializing in diseases of the blood, kidneys, heart, skin, nervous system. Rheumatologists and psychiatrists are involved in the treatment of SLE. In Western countries, family doctors are involved in the process.
Complexity and intricacy of pathogenesis explains the problem of selecting an adequate treatment for SLE. Currently, targeted drugs are being actively developed, but statistics show that you should not count on a miracle. A huge number of seemingly promising developments at the stage of clinical trials have already shown their inefficiency. Currently, the classical course of treatment is formed by a complex of non-specific drugs.
What will help
Drugs for the treatment of SLE are several groups. First, the patient is prescribed compounds that suppress the immune system, thereby correcting the increased activity of cells. Cytostatic agents are popular: "Methotrexate", "Cyclophosphamide". Sometimes Azathioprine is prescribed, in other cases they stop at Mycophenolate mofetil. The same drugs have found active use in antitumor treatment, they are used to control the division of too active cells. Maina feature of their treatment is the abundance of severe negative effects on various systems and organs.
Corticosteroids are used to treat SLE. They are shown during the acute phase. This group includes non-specific agents that suppress foci of inflammation. Their task is to facilitate the autoimmune reaction. Corticosteroids have been used in the treatment of SLE since the middle of the last century. At one time, it was they who became a new step in alleviating the condition of patients. Today it is almost impossible to imagine the treatment of the disease without the use of corticosteroids - in fact, there is no alternative to them. However, one must be aware of the numerous severe negative effects on the body. The most commonly prescribed drugs containing prednisolone, methylprednisolone.
Exacerbation and remission
In 1976, for the first time, pulse therapy was used to treat SLE in the acute phase. Its effectiveness turned out to be quite high, so the approach is relevant at the present time. The bottom line is that the patient receives "Cyclophosphamide", "Methylprednisolone" on impulse. Over the following decades, the regimen was refined, developing the gold standard for the treatment of SLE. Not without drawbacks - side effects are quite severe, and for some groups of patients, pulse therapy is categorically not recommended. It is not suitable, for example, for people with high blood pressure. This indicator is difficult to control. Pulse therapy is not indicated for systemic infection of the body, since there is a high probability of metabolic problems,behavioral disorders.
Treatment of SLE in children and adults in remission involves taking antimalarial drugs. The practice of their use in this pathology is quite long. A lot of evidence has been accumulated confirming the effectiveness of such a program. Antimalarial formulations are good for people suffering from skin lesions localized in the musculoskeletal system. The most well-known substance is hydroxychloroquine, which inhibits the production of alpha-IFN. The use of such a tool in the treatment of SLE allows for a long time to reduce the activity of the pathology, to alleviate the condition of internal systems and organs. In pregnancy, hydroxychloroquine significantly improves outcomes. The use of the drug prevents thrombosis - a fairly common complication of blood vessels. Currently, among other clinical recommendations in the treatment of SLE, antimalarial drugs are one of the basic conditions for the stability of the condition for all patients. However, do not forget about the possible side effects. There is a risk of retinopathy, poisoning of the body, which is especially characteristic of people with insufficient functioning of the liver and kidneys.
Science does not stand still
Described earlier is a classic version of the therapeutic course, but do not neglect the new in the treatment of SLE. Several targeted agents are now available to patients. The most effective interact with B-cells. These are Rituximab, Belimumab.
"Rituximab" contains mouse antibodies that have proven effective in B-cell lymphomas. Substanceselectively fights with mature cells of this type, reacts with the CD20 membrane protein. Studies have been conducted showing that the remedy is effective in SLE, especially in severe form. The drug is resorted to if the symptoms are expressed in the work of the kidneys, the circulatory system, there are manifestations on the skin. However, the two main randomized controlled studies did not confirm the high efficacy of the drug. Rituximab is not currently included in clinical guidelines for the treatment of SLE.
"Belimumab" has established itself as a more effective and reliable tool. Studies have shown that BAFF / BLYS in the blood serum with the disease in question is increased when compared with those of a he althy person. BAFF is an element of the signaling cascade that triggers autoreactive cellular structures. This element determines cell maturation, reproduction, and the generation of immunoglobulin. Belimumab contains antibodies of the same name that bind BAFF and neutralize its effects. As the practice of SLE treatment in Israel, America, Europe and Russia has shown, the substance is safe and well tolerated by patients. Activities dedicated to determining the quality of "Belimumab" lasted seven years. It has been established that among the side effects most often there is a mild, moderate infection that is not life-threatening for patients. Officially, Belimumab has been the primary treatment for SLE since 1956.
Opportunities and Therapy
Presumably effectivethere will be treatment for SLE directed at the first type of interferons. A number of antibodies to them have already shown good results in trials, but final testing has yet to be organized. The effectiveness of abatacept is being actively investigated. This compound is able to inhibit mutual reactions at the cellular level, thereby stabilizing immunity tolerance. Presumably, in the future, SLE therapy will be practiced with the use of anti-cytokine agents, which are currently under development and testing. The drugs "Etanercept", "Infliximab" are of particular interest to the scientific community.
The market is filled with a variety of drugs purporting to be effective in the treatment of SLE. Reviews of "Transfactor", for example, claim that this substance helped to put on its feet, completely cure lupus, despite the officially proven incurability of the disease. Before using any generic drugs, non-specific substances and supplements, you should consult with your doctor. Poor choice of formulations can cause danger to he alth and life.
Folk remedies
Is it possible to practice the treatment of SLE with folk remedies? Of course, certain approaches have been invented, but they should not be expected to be particularly effective. This is due to the peculiarities of the disease, because only the most modern means can cope with disorders at the cellular level, and even those are still not effective enough. Unfortunately, no medicinal herbs and infusions can cure SLE. ByIn consultation with the doctor, some prescriptions can be used to relieve specific symptoms. The choice must be strictly individual. It always depends on the nuances of the course of the disease.
Spa treatment for SLE in remission can improve a patient's quality of life. Full recovery cannot be achieved in this way, but being in comfortable conditions, in an environmentally friendly environment with the practice of specific procedures and taking the drugs recommended by the attending physician is the key to improving a person's well-being. A well-chosen sanatorium course will help to consolidate the remission.
Pathogenesis
For a long time, scientists did not know what the pathogenesis of SLE was. In recent years, it has been established that there are several mechanisms that trigger the disease. The main factor is the work of the immune system, the immune response. When examining patients, approximately 95% of patients can detect autoantibodies that attack the cells of the body due to erroneous recognition of them as foreign structures. Currently, the main cells with which the danger is associated are type B, which produce active autoantibodies. They are indispensable for adaptive immunity, produce signaling cytokines. It is assumed that with increased cell activity, SLE develops, since too many autoantibodies are generated that attack antigens in the blood serum in membranes, cytoplasm, and cell nuclei. This explains the clinical manifestations of SLE. The situation is complicated by the fact that cells generate inflammatory mediators, transmitT-lymphocytes receive data not about foreign structures, but about the elements of their own body.
The pathogenesis of SLE has two aspects: active lymphocytic apoptosis, a decrease in the quality of processing of autophagy by-products. This stimulates an immune response directed at the cells of your body.
Where does the trouble come from
Despite the clarification of the pathogenesis, at present it has not been possible to determine exactly the reasons for the onset of SLE. It is believed that the disease is multifactorial, appears with the complex influence of several aspects.
Special attention of scientists is attracted by heredity as an impetus for the development of SLE. In many ways, the relevance of this aspect is indicated by the variability in ethnicity, gender. It has been established that among women, SLE occurs up to ten times more often than among men, and the peak incidence is in the age group of 15-40 years, that is, the entire reproductive period.
Ethnicity, as can be seen from the statistics, determines the severity of the course, the prevalence of the disease, the likelihood of death. Butterfly rash is a fairly typical manifestation in white-skinned patients. Dark-skinned people are more likely to be diagnosed with a severe course with a tendency to frequent relapses. Afro-Caribbeans, African Americans, are more likely than others to have kidney problems with SLE. The discoid form is more common among black people.
Statistics suggest that heredity, genetic characteristics is an important factor in the etiology of SLE.
Difficultiesin drug development
To confirm the theory of genetic predisposition, a whole genome associative search method was developed and applied, in which thousands of variants of genomes and phenotypes are compared. The information of patients with SLE is being studied. This technology made it possible to identify 60 loci divided into several categories. Some are associated with innate characteristics, others are genetic factors that affect adaptive immunity. It has been established that an impressive percentage of loci is characteristic not only of SLE, but also of other autoimmune diseases.
It is suggested that a person's genetic data can be used to determine the level of risk of developing SLE. Perhaps, in the future, genetic information will simplify the diagnosis of the disease and help to more effectively choose methods of its treatment. The specificity of the disease is such that primary complaints rarely help to establish an accurate diagnosis, so a lot of time is wasted. The choice of a suitable therapeutic course is also rarely successful the first time, because the variability in response to different drugs is too great.
Today, genetic tests have not yet found their way into clinical practice - they have yet to be finalized and made more accessible. Forming a predisposition model, it is necessary to take into account the characteristics of genes, mutual reactions, the number of cytokines, markers, and other indicators. In addition, the model should include an analysis of epigenetic features.
Provoking factors
Supposedly, the development of SLE is influenced by ultraviolet radiation. The light of our luminaryoften provokes rashes, redness. Possibly, infection plays a role. There is a theory explaining autoimmune reactions as a response to viral mimicry. Perhaps provocateurs are not specific viruses, but features of the body's typical method of fighting invasion.
It was not possible to formulate exactly whether smoking, drinking alcohol affects the likelihood of developing SLE. The first may increase the risk, the second, as seen in some studies, reduces it, but there is no confirmed information.
Case refinement
As mentioned above, SLE has no specific features. If the patient's condition is difficult to explain by other reasons, there is a suspicion of lupus. The patient is sent for a laboratory blood test, determination of antinuclear bodies, LE cells. If the tests show the presence of antibodies to DNA, the diagnosis is considered confirmed.
