Lupus erythematosus treatment and drugs. Systemic lupus erythematosus
For citation: Klyukvina N.G., Shekshina S.V., Nasonov E.L. Modern view on the treatment of systemic lupus erythematosus // BC. 2002. No. 6. S. 307
MMA named after I.M. Sechenov Institute of Rheumatology RAMS, Moscow
FROM Systemic lupus erythematosus (SLE) is an autoimmune rheumatic disease, the pathogenesis of which is based on defects in immunoregulation, leading to uncontrolled hyperproduction of autoantibodies to the components of one's own tissues and the development of chronic inflammation affecting many organs and systems.
The keen interest in SLE over the past 50 years has made it possible to improve the methods of diagnosing the disease, but curation of patients continues to be challenging task. The development of treatment methods is hampered primarily by the unknown etiology of the disease, the high variability of clinical manifestations, the possibility of both prolonged spontaneous remissions and a malignant, rapidly progressive, sometimes fulminant course. To control the disease, almost the entire arsenal of anti-inflammatory and cytotoxic drugs existing in medicine is currently used, the use of which has significantly improved the prognosis of patients' lives. If before 1970 only 40% of SLE patients lived longer than 5 years, now the 5-year survival rate is 90% or more.
In this regard, it is difficult to overestimate the importance glucocorticoids (GC), the effect of which occurs almost immediately after the appointment. Long-term observations have shown that GCs have been and remain the drugs of choice in the acute course of the disease with visceral manifestations . However, already at the first stages it became clear that long-term glucocorticoid therapy in patients with SLE raises a number of questions regarding doses, duration of treatment and prevention of complications associated with the use of potent drugs. Glucocorticoid therapy has been improved over the years: modified dosage forms, certain provisions on doses are established in accordance with the degree of disease activity and certain visceral pathology. The opinion has become stronger that HA should be given to SLE patients for many years, mostly for life, moving from high doses in the high activity phase to maintenance and maintaining this dose even during the period of clinical remission.
A new stage in the use of HA in SLE was the introduction method of intravenous administration of ultra-high doses - "pulse therapy" , which proved to be very effective in previously incurable patients. Available data indicate a significant immunosuppressive effect of intravenously administered high doses of HA already in the first day. It is believed that shock doses of HA (primarily methylprednisolone) stop the formation of immune complexes, in more are deposited in inflamed tissues, block the damaging effect of lymphotoxins. The anti-inflammatory and immunoregulatory effect of pulse therapy is largely due to the effect on the cytokine system, tumor necrosis factor and the suppression of transcription of the metalloproteinase and lipocortin genes.
Over the years, the use of pulse therapy has changed and the methodology for its implementation. First, the concept of using this method only when other treatments fail is revised. A certain category of patients has been identified (young age, rapidly progressive lupus nephritis, high immunological activity), in which this method should be used at the onset of the disease or immediately in case of any exacerbation.
Secondly, "classic" pulse therapy is not always enough (daily for 3 consecutive days at the rate of 15-20 mg per kg of the patient's weight). In order to increase efficiency, in some cases, loading doses of methylprednisolone are prescribed at intervals of several weeks for several months. To enhance the impact on immunopathological processes, the program prescription of pulse therapy with methylprednisolone can be enhanced by the addition of cyclophosphamide, and in order to achieve a clinical effect in some cases intravenous administration methylprednisalone and cyclophosphamide continues for 5-10 days. In a special way, a strategy for the treatment of patients with the ineffectiveness of standard pulse therapy programs with methylprednisolone and cyclophosphamide, in cases of resistance to prescribed drugs, as well as in the presence of a combination of several prognostically unfavorable factors, is built. In such situations, the most promising synchronous intensive care , which is based on a combination of pulse therapy and extracorporeal methods of treatment (plasmapheresis).
Therapy with GC alone does not allow suppression of activity in some forms of SLE. Since the 60s, cytostatic immunosuppressants, aminoquinoline derivatives and other drugs have been included in the treatment complex, which, along with GC, have become, as it were, basic therapy.
Most commonly used in SLE cyclophosphamide, azathioprine And chlorbutin , in last years in some forms, they began to successfully prescribe methotrexate . Active lupus nephritis and generalized vasculitis, high overall disease activity and resistance to GC are currently considered indications for the inclusion of cytostatic immunosuppressants in the complex treatment of patients with SLE; the appearance of adverse reactions of these drugs already at the first stages of treatment, the need to reduce the maintenance dose of prednisolone. The introduction of cytostatics into the treatment complex made it possible to suppress the activity of the disease against the background of lower doses of HA, to increase the survival of patients, primarily with lupus nephritis. With regular monitoring and individual selection of the dose and drug, careful monitoring of therapy, it was possible to significantly reduce the number of adverse reactions and complications.
have not lost their meaning aminoquinoline derivatives in SLE patients without severe visceral manifestations and during the period of reducing the doses of GCs and cytostatics to maintain remission. A detailed study of the mechanisms of action of these drugs, which revealed anti-inflammatory, antiplatelet, hypolipidemic, photoprotective, antioxidant, antimicrobial and analgesic effects, made it possible to take a fresh look at the prospects for their inclusion in the SLE treatment regimen.
Nevertheless, despite the progress made, the management of patients with SLE remains one of the most difficult problems of modern rheumatology. According to the combination of efficacy and safety, drugs that affect immune inflammation - HA, cyclophosphamide, azathioprine, chlorbutine, and others - do not always satisfy clinicians. In addition, in many patients, the early administration of adequate doses of GCs and cytotoxic drugs does not avoid irreversible damage to vital organs and systems (primarily the kidneys and central nervous system), and is also often associated with the development of severe, potentially fatal adverse reactions (intercurrent infection). , cytopenia, hemorrhagic cystitis, osteoporotic fractures, increased number of malignant neoplasms etc.). All this determines the need to study new approaches to the pharmacotherapy of SLE. Improving the methods of influencing the immune process occurs in two directions: changing the traditional modes of their use and introducing new drugs into practice.
The point of application of new therapeutic methods is effect on lymphocytes (bone marrow transplantation, use of nucleoside analogues and extracorporeal therapies), prevention of immune complex formation and deposition, and alteration of the immune response by inducing antigen-specific tolerance or interaction with the cytokine system.
Considering new approaches to treatment, it should be noted that human autoimmune diseases, having their own unique features, generally resemble the immunopathological processes that form the basis of the transplantation immunity response. Therefore, it is not surprising that many modern types of immunotherapy for diffuse connective tissue diseases were first successfully applied in organ and tissue transplantation.
Of particular interest in recent years is cyclosporine A , which is considered as one of the most effective medicines with selective immunosuppressive activity . It is increasingly used in clinical practice for the treatment of many immunoinflammatory diseases of the internal organs, including SLE. The specific mechanisms that determine the effectiveness of cyclosporine A (CsA) in SLE are not fully understood. Obviously, CsA is very close to glucocorticoids in terms of the nature of its effect on the synthesis of cytokines. It cannot be ruled out that one of the important mechanisms of action of CsA in SLE is associated with inhibition of interferon-g synthesis. Of interest is the ability of CsA to suppress the expression of the CD40 ligand on the membrane of T-lymphocytes. Monoclonal antibodies to the CD40 ligand are known to be highly effective in slowing disease progression in mouse models of lupus-like disease. CsA probably also has a similar activity.
At present, certain experience has been gained in the use of CsA in SLE. Back in the early 1980s, several open short-term trials of the effectiveness of CsA in SLE were conducted using high doses (more than 5 mg/kg/day) of the drug. In 1981, CsA was first administered to 5 patients with SLE at a dose of 10 mg/kg/day for 7 weeks. However, in all cases, treatment was discontinued due to the development side effects(primarily nephrotoxic) and arterial hypertension. Thus, the effectiveness of CsA was significantly compromised by the development of a large number of complications, which was most likely due to the use of high doses of the drug.
In recent years, a series of long-term (more than 2 years) open-label clinical trials have been conducted to evaluate the effectiveness of low (less than 5 mg/kg/day) doses of CsA in SLE. Most of the reports on the use of CsA in patients with SLE concern the use of this drug in lupus nephritis, and a pronounced antiproteinuric effect occurring during the first 2-3 months of therapy was observed in a significant number of patients. A number of researchers have also shown the effectiveness of low doses of the drug in thrombocytopenia, anemia and leukopenia, skin manifestations of SLE, polyserositis refractory to therapy and arthritis. It should be noted that in the presence of a good effect of therapy, the side effects that developed in some patients were not severe and rarely served as a basis for interrupting treatment. Almost all researchers recorded a steroid-sparing effect of the drug.
The undoubted advantage of CsA in comparison with other drugs used for the treatment of SLE is lower frequency of both immediate and long-term side effects , primarily infectious complications and malignant neoplasms. For example, the risk of developing lymphoproliferative tumors against the background of long-term treatment with CsA is very low - 0.05-0.14%, and drug withdrawal can lead to tumor regression. Unlike other cytotoxic drugs, CsA has minimal teratogenicity.
The existing own experience of prescribing CsA in SLE patients confirms the results of other studies: against the background of patients taking low doses of the drug (on average 2-2.5 mg / kg / day), a decrease in disease activity, a positive dynamics of the clinical manifestations of SLE, primarily a decrease in the daily proteinuria, normalization of temperature, relief of arthralgia, decrease in the brightness of skin rashes. We believe that CsA treatment has a positive effect on disease activity, affecting severe organ pathology, and the use of low doses and careful monitoring of therapy makes it possible to avoid the development of severe side effects. CsA can be considered an alternative second-line drug for intolerance and ineffectiveness of glucocorticoids and cytostatics . In addition, undoubted positive aspects the inclusion of CsA in the treatment regimen for SLE should be considered a lower incidence of concomitant infection and the possibility of prescribing during pregnancy. There are preliminary data on a decrease in the level of anticardiolipin and antiplatelet antibodies during CsA therapy, as well as a preventive effect on the early development of atherosclerosis, which is of great importance for patients with SLE.
Encouraging results have also been obtained with the use of another selective immunosuppressant, mycophenolate mofetil, in SLE patients. Mycophenolate mofetil (CellCept) is a synthetic morpholinoethyl ester of mycophenolic acid and is its precursor. After ingestion of mycophenolate mofetil, hepatic esterases completely convert it into the active compound - mycophenolic acid - which is a non-competitive inhibitor of inosine monophosphate dehydrogenase, an enzyme that limits the rate of synthesis of guanosine nucleotides. Since the functional activity of lymphocytes, to a greater extent than other rapidly dividing cells, depends on the synthesis of purines, the drug gives a more pronounced antiproliferative effect on lymphocytes and exhibits cytostatic rather than cytotoxic activity . In nanomolar concentrations, mycophenolic acid inhibits the proliferation of stimulated T- and B-lymphocytes in human peripheral blood in vitro, has an antiproliferative effect on mesangial cells of the human and animal kidneys, and inhibits the formation of antibodies in cultures of human lymphocytes and spleen cells. In addition, the depletion of gaunosine nucleotides under the influence of mycophenolic acid adversely affects not only DNA synthesis, but also the process of glycosylation of adhesion molecules. Such suppression of the humoral response may prove useful in the treatment of antibody-mediated diseases. As an anti-metabolic type immunosuppressant, mycophenolate mofetil was initially studied as a treatment and prophylaxis for acute allogeneic transplant rejection of various solid organs. As a rule, this drug is used as part of combination therapy after transplantation, appointing it instead of azathioprine, in combination with glucocorticoids and cyclosporine. In large randomized controlled trials in patients undergoing kidney or heart transplantation, mycophenolate mofetil was significantly more effective than azathioprine in reducing rejection rates.
To date, data have been published in the literature regarding the use of mycophenolate mofetil in about 100 patients with refractory lupus nephritis. Therapy with mycophenolate mofetil at doses of 1.5-2 g/day led to a decrease in proteinuria, stabilization of serum creatinine levels, normalization of the levels of the C3 component of complement and antibodies to DNA, and a decrease in SLE activity. In 2000, a group of researchers from Hong Kong published data on a comparative analysis of the effectiveness of two drug regimens - HA in combination with mycophenolate mofetil and HA in combination with traditional cytostatics in SLE patients with diffuse proliferative lupus nephritis. As it turned out, the effectiveness of two various schemes therapy, as measured by a decrease in proteinuria, creatinine and an increase in albumin, was the same in both groups after 12 months of therapy. At the same time, the frequency of concomitant infectious complications that developed was 2 times higher in the group receiving cyclophosphamide and azathioprine, and side effects such as amenorrhea, hair loss, leukopenia were not recorded at all in patients taking mycophenolate mofetil. Literature data, as well as our own experience with mycophenolate mofetil in patients with SLE, suggest that in patients with kidney damage, the drug may be a worthy therapeutic alternative to cyclophosphamide and azathioprine, with better tolerability.Another direction of SLE therapy in recent years is the use of certain immunomodulators such as thalidomide, bindarit, nucleoside analogs (fludarabine, mizoribine, leflunomide). The use of these drugs in experimental models of lupus-like disease led to a decrease in proteinuria and an increase in the survival rate of mice. At present, some experience has been gained in the use of these drugs in patients with SLE. Clinical Trials thalidomide were mainly carried out in patients with severe skin lesions resistant to antimalarial drugs and GCs. In the vast majority of patients, it was possible to achieve a good effect and reduce the dose of GC, while drug withdrawal did not lead to an exacerbation of symptoms. The main limitation in the use of thalidomide is its teratogenicity. In addition, the development of irreversible peripheral neuropathy, depending on the dose and duration of treatment, has been described.
There are separate reports of use in SLE lobenzarite , which has a pronounced immunomodulatory activity and is able to suppress the synthesis of antibodies to DNA and inhibit the synthesis of IgM-rheumatoid factor.
The results of the study in vitro indicate a synergistic effect of the combination of nucleoside analogs and cyclophosphamide; Similar treatment regimens have been successfully used in oncology for a long time. As for SLE patients, there are isolated reports of the effectiveness of these drugs in patients with lupus nephritis, their steroid-sparing effect has been proven. At the same time, one cannot fail to note the high incidence of concomitant infectious complications, which raises the question of the safety of the use of these drugs and the risk/benefit ratio in patients with SLE.
Prospects for the treatment of patients with SLE, undoubtedly - for biological methods of exposure, using the so-called "biological" agents . These drugs are being developed with the aim of influencing specific immunological processes, which include T-cell activation, T-B cell interaction, the production of antibodies to double-stranded DNA, the activation of cytokines, and others. In this respect, there is great potential for the use anti-idiotypic monoclonal antibodies, intravenous immunoglobulin . Few data are available on the efficacy of recombinant DNase, a DNA-cleaving enzyme, in experimental lupus models. The administration of the drug to NZB/W mice with an active renal process resulted in a decrease in proteinuria and serum creatinine, however, the use in several patients with SLE has not yet given convincing positive results, despite the good tolerability of the drug. Identification of elevated serum levels of IL-10 in SLE patients and relatives, as well as their correlation with disease activity, served as the basis for using monoclonal antibodies to IL-10 . Preliminary results from several studies indicate a positive effect of antibodies against skin lesions, kidney disease, arthritis and serositis refractory to glucocorticoid therapy.
Thus, the undoubted prospect for the treatment of SLE patients will be the use of "biological" agents, especially since most of them do not have a generalized immunosuppressive effect.
Currently, the most aggressive treatment for SLE is autologous stem cell transplant (ATSC). By the year 2000, a little more than 30 patients with SLE had gained experience in using ATSC. Preliminary positive results certainly need further confirmation. Long-term monitoring of patients is necessary, bearing in mind the possibility of inducing the development of malignant tumors. Despite the impression that this type of therapy is effective in cases of refractory and severe SLE, due to the high mortality that accompanies it, ATSC can be recommended only in the most severe, hopeless cases.
Have not lost their relevance and attempts to treat SLE with modulation of sex hormones , the imbalance of which is unconditionally recognized as a pathogenetic factor of the disease. as new medicines currently used are dehydroepiandrosterone and other androgens, estrogen antagonists (including tamoxifen and selective estrogen receptor modulators), and the prolactin inhibitor, bromocriptine.
Experimental models have shown that the introduction estrogen antagonists or male sex hormones can reduce disease activity. The first attempts to prescribe hormonal drugs concerned the synthetic androgen, 19-nortestosterone, and gave conflicting results: after the clinical improvement in women after discontinuation of the drug, a relapse of symptoms was observed, and in men, a negative trend was noted during the treatment. It has been suggested that the lack of effect in males can be explained by the weak effect of 19-nortestosterone, which does not affect endogenous testosterone production. The study of the effectiveness of dehydroepiandrosterone, a natural androgen with a moderate effect, made it possible to discuss the feasibility of prescribing it in non-severe forms of SLE. In one placebo-controlled study, the drug was shown to be twice as effective as placebo, with generally good tolerability. The effectiveness of dehydroepiandrosterone is primarily attributed to the change in the level of adrenal sex hormones, mainly to increase the concentration of testosterone, although the mediated release of mineralocorticoid and glucocorticoid hormones may also be important.
The use of oral contraceptives and estrogen replacement therapy in SLE patients has long been disputed. However, in Lately The effectiveness of selective estrogen receptor modulators has been demonstrated in experimental models, which has made it possible to take a fresh look at the prospects for their use in humans. It must be borne in mind that such therapy should be carried out with caution due to the potential risk of thrombotic complications, especially in patients with secondary antiphospholipid syndrome.
A series of studies is devoted to the evaluation of therapeutic activity tamoxifen - a synthetic non-steroidal antiestrogen that specifically binds to estrogen receptors, which is used to treat breast cancer in women. According to early clinical studies performed on a small clinical material, tamoxifen did not significantly affect the course of the disease in women with SLE. However, it has recently been shown that tamoxifen slows down the development of the pathological process in mice with experimental lupus-like disease induced by immunization with anti-DNA monoclonal antibodies. It is assumed that the favorable effect of the drug on the course of SLE may be associated with a change in the profile of the synthesis of Th-1 and Th-2 cytokines, an increase in cortisol production and a decrease in prolactin levels.
The association of hyperprolactinemia with SLE activity has been convincingly proven. Purpose bromocriptine , a dopamine receptor agonist, leads to a decrease in prolactin levels. When conducting a randomized, placebo-controlled study in 66 patients with SLE, even in the absence of high levels of prolactin, their statistically significant decrease was revealed compared with the control, accompanied by a decrease in disease activity.
Thus, preliminary results in animal models or pilot studies in humans suggest the effectiveness of a number of pharmacological agents in SLE, which include both "non-specific" immunoregulators and drugs that affect the sex hormone profile.
Another aspect of the treatment of SLE should also be mentioned. Despite the undesirability of polypharmacy in SLE, the accumulated experience indicates the need for a number of auxiliary medicines . This applies to the use of drugs that improve cerebral circulation and metabolism of brain cells (vinpocetine, pentoxifylline, cerebrolysin, piracetam, etc.), antiplatelet agents, anticonvulsants, tranquilizers.
The presence of secondary antiphospholipid syndrome in patients with SLE also requires a correction in the treatment regimens. (AFS). A difficult problem is the prevention of recurrent thrombosis in APS. This is due to the heterogeneity of the pathogenetic mechanisms underlying APS, the polymorphism of clinical manifestations, and the lack of reliable clinical and laboratory parameters to predict the recurrence of thrombotic disorders. APS patients are prescribed indirect anticoagulants And antiplatelet agents (low doses acetylsalicylic acid), which are widely used for the prevention of thrombosis. However, the use of high doses of indirect anticoagulants is associated with an increased risk of bleeding, so this therapy needs to be carefully monitored. Patients with APS often have mild thrombocytopenia, which is usually corrected with small doses of glucocorticoids. Sometimes with GC-resistant forms of thrombocytopenia, low doses of acetylsalicylic acid, danazol, warfarin are effective. High hopes are placed on the use low molecular weight heparin , as well as the introduction of new methods of anticoagulant therapy based on the use of arginals, hyuidins, anticoagulant peptides and antiplatelet agents (monoclonal antibodies to platelets) .
An important direction in the pharmacotherapy of SLE is the prevention or treatment of concomitant (often drug-induced) pathologies, primarily "early" atherosclerosis, osteoporosis, and infectious complications, which have no less negative impact on life prognosis than the disease itself. This determines the need for a wider introduction of modern antihypertensive, lipid-lowering, anti-osteoporotic and antimicrobial drugs. Since some of them, such as statins, antibiotics and possibly bisphosphonates, have anti-inflammatory and immunomodulatory activity, their use has the potential to increase the effectiveness of the treatment of inflammatory rheumatic diseases.
Again the question of dietary regimen with SLE, as there is evidence of the influence of certain nutrients on the mechanism of inflammation. A reduced content of linoleic acid in food leads to a decrease in the synthesis of prostaglandins and leukotrienes, which have a pro-inflammatory effect. With an increase in the content of unsaturated fatty acids in food, the intensity of the processes of inflammation and fibrosis decreases. In this regard, it seems appropriate to study the effect of diets with a certain content of fatty acids on various manifestations of the disease, which will allow us to evaluate the effect of dietary regimens on the development of the pathological process.
Thus, today it is possible to effectively influence the pathological process in SLE. With the skillful use of potent drugs, one can not only significantly improve the prognosis in SLE patients, but also avoid serious complications of the therapy. Despite the fact that the presented material cannot fully cover the whole variety of approaches to the treatment of SLE, in general, it still allows us to trace the main modern trends, primarily regarding drugs that have a high specificity influencing the mechanisms of the development of the disease and are characterized by a fairly high efficacy and safety. . At the same time, one should not forget about the development of new treatment methods that are not symptomatic, but pathogenetically directed.
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In recent years, a real sensation has been the FDA registration of a new biological drug for treatment - belimumab.
Belimumab is the first genetically engineered biological drug (GEBD) specifically designed for the treatment of lupus, which was one of the biggest achievements in rheumatology over the past 50 years!!!
The mechanism of action of Belimumub is very complex. In short and simplified, B-lymphocyte stimulator (BLyS) was of particular interest to researchers. Clinical studies have shown that in patients with systemic lupus erythematosus, an increase in serum BLyS concentration is observed, which correlates with the concentration of antibodies to double-stranded DNA and the dynamics of disease activity. These data served as a theoretical basis for the development of anti-BLyS drugs, primarily belimumab, for the treatment of SLE.
Official indications for prescribing belimumab for systemic lupus erythematosus:
- moderate or high disease activity,
- the presence of severe immunological disorders ( positive results detection of antinuclear factor and/or antibodies to DNA),
- insufficient effectiveness of standard therapy.
Belimumab is generally well tolerated, with infections, joint pain, headache, diarrhea, and nausea being the most common adverse events. Severe infections and depression are very rare.
Based on a large-scale study of the efficacy of belimumab in real clinical practice, the results of which were reported at the X International Conference on Systemic Lupus Erythematosus (Buenos Aires, Argentina, April 18–19, 2013), high efficacy of belimumab was shown in 501 patients with SLE. The use of belimumab for 6 months led to improvement in 57-65% of patients with high and moderate disease activity. In 77% of patients treated with belimumab, it was possible to significantly reduce the dose of hormones, and in 9% to completely cancel them. Therapy with belimumab led to a decrease in the frequency of exacerbations, hospitalizations, and a decrease in the activity of systemic lupus erythematosus. The first signs of improvement, as a rule, are observed on the 8th week of therapy: a decrease in the feeling of fatigue, a decrease in the activity indices of systemic lupus erythematosus. A more pronounced improvement is observed by the 12-16th week.
Indications for prescribing belimumab for SLE in clinical practice:
- Insufficient effectiveness of previous therapy for at least 3 months.
- High or moderate SLE activity.
- The need to prescribe hormonal drugs or their combination with cytostatics to maintain remission.
- Contraindications for the appointment of one of the cytostatics or the need to prescribe cytostatics in a dose exceeding the recommended one.
- High levels of anti-DNA antibodies, low complement levels.
- Damage to the skin, joints, muscles, mucous membranes, thrombocytopenia, progressing against the background of standard therapy.
- Jade with a low degree of clinical and morphological activity.
The main goals of therapy:
- decrease in disease activity;
- prevention of recurrence of the appearance of activity;
- reduction in the daily dose of hormonal drugs;
- reducing the risk of developing irreversible organ damage;
- improving the quality of life.
Here are the news today. And you can read the real diary of a patient on Belimumab. For your information, the development of new and effective drugs continues. We'll be watching closely 🙂
- a group of severe autoimmune diseases of the connective tissue, affecting mainly the skin and internal organs of a person. This disease got its name because of the characteristic rashes on the skin of the face, in their appearance resembling wolf bites. Young women get sick more often, men and children suffer from lupus erythematosus much less often.
In total, the number of people suffering from lupus erythematosus is 0.004-0.25% of their total number.
Among the reasons are: hereditary predisposition to this disease, other causes of its appearance are still unexplored. It is believed that acute infectious diseases, severe psychotrauma, prolonged exposure to stress, or intolerance to certain pharmacological drugs can provoke the appearance of lupus erythematosus.
A characteristic feature of lupus erythematosus is a wide range of its manifestations, because this disease affects almost all organs and systems of the human body. Nevertheless, there is a list of symptoms, the presence of which is mandatory for lupus erythematosus:
- constitutional symptoms (malaise, weight loss)
- skin lesions (increased sensitivity to light, alopecia, characteristic butterfly-shaped erythema on the skin of the nose and cheeks)
- erosive lesions of the mucous membranes
- arthritis-type joint damage
- damage to the lungs and heart
- kidney damage (in 50% of patients) up to renal failure
- disorders of the nervous system (acute psychosis, organic brain syndrome)
- changes in general blood and urine tests
- antiphospholipid syndrome in 20-30% of patients
- high antinuclear antibody titer
The specific type of lupus erythematosus and the degree of disease activity at one time or another is determined by a rheumatologist after a comprehensive examination. Cutaneous lupus is most often treated by a dermatologist.
Treatment methods for lupus erythematosus
The disease cannot be completely cured, so treatment continues for life. How to treat lupus erythematosus is decided by the attending physician individually for each patient, depending on the specific symptoms, the severity of the disease and its activity.
With a mild course of the disease or in remission, treatment is mainly symptomatic. The following drugs are prescribed:
- analgesics for severe pain syndrome
- aspirin (80-320 mg per day) with a tendency to thrombosis
- antimalarial drugs: hydroxychloroquine (200 mg orally per day) or a combination of chloroquine (250 mg) and quinacrine (50-100 mg) - for severe skin and joint damage
- vitamins A, B6, B12, C
In severe cases of the disease with damage to internal organs, apply:
- glucocorticoids (in severe condition daily 40-60 mg of prednisolone, 20-40 mg - with moderate disease activity) are taken for a month with a gradual decrease to a therapeutic dose (up to 10 mg per day)
- immunosuppressants (mycofenalata mofetil 500–1000 mg, azathioprine 1–2.5 mg/kg, or cyclophosphamide 1–4 mg/kg once daily orally)
- heparin in combination with aspirin subcutaneously, heparin or vafarin orally for thrombosis and vascular embolism
- in severe cases, with low efficacy of treatment with glucocorticoids, pulse therapy with methylprednisolone and cyclophosphamide is indicated, which are administered in large doses (1 g per day) intravenously by drip for 3 days in a row
- hemosorption and plasmapheresis - to remove toxic immune complexes from the body
- stem cell transplantation - an expensive procedure inaccessible to most patients
- calcium preparations and vitamin D3 - to minimize the negative effects of the use of glucocorticoids
Patients are observed dispensary. Indications for their immediate hospitalization are:
- infectious complications
- chest pain
- severe symptoms of pathologies of the nervous system
- kidney failure
- thrombosis
Traditional medicine against lupus erythematosus
Prescription treatment for cutaneous and systemic lupus erythematosus traditional medicine is supportive and can be used during a mild course of the disease or at the stage of remission. In this case, you can not use drugs that stimulate the immune system - this can aggravate the course of the disease.
Popular effective recipes:
- Decoction of dried mistletoe leaves collected from a birch in the cold season. 2 tsp leaves are poured with a glass of boiling water, simmered in a water bath for 1-2 minutes and insisted for half an hour. The resulting infusion is drunk in three doses during the day. Take 1 month.
- Decoction of licorice. Dried licorice roots (1 tablespoon) pour boiling water (500 ml), simmer for 15 minutes, cool at room temperature. Strained broth to drink during the day between meals. Do this for a month.
- Ointment from birch buds or tarragon for the treatment of lupus erythema. A glass of ground birch buds (tarragon) is mixed with a half-liter can of pork fat. This mixture is simmered for 5-7 days for several hours in the oven with the door open. The resulting ointment is applied to erythema and taken orally before meals, 1 tsp.
There are many other alternative medicine recipes that are used to treat lupus erythematosus. However, with a severe form of the disease and at the stage of its exacerbation, traditional medicine should give way to traditional drug treatment.
How to live with a diagnosis of lupus erythematosus?
In this case, you must follow the basic recommendations:
- avoid stress and stressful situations
- Avoid prolonged exposure to the sun and solarium
- monitor your health: prevent exacerbation of chronic diseases, treat colds immediately after their first symptoms
- do not take oral contraceptives and do not smoke - this significantly increases the risk of thrombosis
- use high-quality cosmetics, do not carry out hardware and chemical cleaning of the face
- take vitamin complexes
- eat a balanced diet and exercise
Variants of the course of the disease and prognosis
The prognosis is unfavorable. Mortality among patients with lupus erythematosus is 3 times higher than usual. Most often, the cause of death is infectious complications and the consequences of deep damage to internal organs. But with timely detection of the disease and competent drug therapy, it is possible to keep this disease under control and not allow it to destroy life and health.
Systemic lupus erythematosus (SLE)- a chronic autoimmune disease caused by a malfunction of immune mechanisms with the formation of damaging antibodies to one's own cells and tissues. SLE is characterized by damage to the joints, skin, blood vessels and various organs (kidneys, heart, etc.).
The cause and mechanisms of the development of the disease
The cause of the disease has not been elucidated. It is assumed that the trigger mechanism for the development of the disease are viruses (RNA and retroviruses). In addition, people have a genetic predisposition to SLE. Women get sick 10 times more often, which is associated with the peculiarities of their hormonal system ( high concentration estrogen in the blood). The protective effect of male sex hormones (androgens) with respect to SLE has been proven. Factors that can cause the development of the disease can be a viral, bacterial infection, medications.The basis of the mechanisms of the disease is a violation of the functions of immune cells (T and B - lymphocytes), which is accompanied by excessive formation of antibodies to the body's own cells. As a result of excessive and uncontrolled production of antibodies, specific complexes are formed that circulate throughout the body. Circulating immune complexes (CIC) settle in the skin, kidneys, on the serous membranes of internal organs (heart, lungs, etc.) causing inflammatory reactions.
Symptoms of the disease
SLE is characterized by a wide range of symptoms. The disease proceeds with exacerbations and remissions. The onset of the disease can be both lightning fast and gradual. General symptoms
- Fatigue
- Weight loss
- Temperature
- Decreased performance
- Fast fatiguability
Damage to the musculoskeletal system
- Arthritis - inflammation of the joints
- Occurs in 90% of cases, non-erosive, non-deforming, joints of the fingers, wrists, knee joints are more often affected.
- Osteoporosis - decreased bone density
- As a result of inflammation or treatment with hormonal drugs (corticosteroids).
- Muscle pain (15-64% of cases), muscle inflammation (5-11%), muscle weakness (5-10%)
Mucosal and skin lesions
- Skin lesions at the onset of the disease appear only in 20-25% of patients, in 60-70% of patients they occur later, in 10-15% of the skin manifestations of the disease do not occur at all. Skin changes appear on areas of the body exposed to the sun: face, neck, shoulders. Lesions have the appearance of erythema (reddish plaques with peeling), dilated capillaries along the edges, areas with excess or lack of pigment. On the face, such changes resemble the appearance of a butterfly, as the back of the nose and cheeks are affected.
- Hair loss (alopecia) is rare, usually affecting the temporal region. Hair falls out in a limited area.
- Increased skin sensitivity to sunlight (photosensitivity) occurs in 30-60% of patients.
- Mucosal involvement occurs in 25% of cases.
- Redness, decreased pigmentation, malnutrition of the tissues of the lips (cheilitis)
- Small punctate hemorrhages, ulcerative lesions of the oral mucosa
Respiratory damage
Respiratory system lesions in SLE are diagnosed in 65% of cases. Pulmonary pathology can develop both acutely and gradually with various complications. The most common manifestation of damage to the pulmonary system is inflammation of the membrane covering the lungs (pleurisy). It is characterized by pain in the chest, shortness of breath. SLE can also cause the development of lupus pneumonia (lupus pneumonitis), characterized by: shortness of breath, cough with bloody sputum. SLE often affects the vessels of the lungs, leading to pulmonary hypertension. Against the background of SLE, infectious processes in the lungs often develop, and it is also possible to develop a serious condition such as blockage pulmonary artery thrombus (pulmonary embolism).Damage to the cardiovascular system
SLE can affect all structures of the heart, the outer shell (pericardium), the inner layer (endocardium), directly the heart muscle (myocardium), valves and coronary vessels. The most common is the pericardium (pericarditis).- Pericarditis is an inflammation of the serous membranes that cover the heart muscle.
- Myocarditis is inflammation of the heart muscle.
- The defeat of the valves of the heart, the mitral and aortic valves are more often affected.
- Damage to the coronary vessels can lead to myocardial infarction, which can also develop in young patients with SLE.
- Damage to the inner lining of blood vessels (endothelium) increases the risk of atherosclerosis. Peripheral vascular disease is manifested by:
- Livedo reticularis (blue spots on the skin creating a grid pattern)
- Lupus panniculitis (subcutaneous nodules, often painful, may ulcerate)
- Thrombosis of the vessels of the extremities and internal organs
Kidney damage
Most often in SLE, the kidneys are affected, in 50% of patients lesions of the renal apparatus are determined. A frequent symptom is the presence of protein in the urine (proteinuria), erythrocytes and cylinders are usually not detected at the onset of the disease. The main manifestations of kidney damage in SLE are: proliferative glomerulonephritis and mebran nephritis, which is manifested by nephrotic syndrome (proteins in the urine more than 3.5 g/day, decreased protein in the blood, edema).Damage to the central nervous system
It is assumed that CNS disorders are caused by damage to the cerebral vessels, as well as the formation of antibodies to neurons, to cells responsible for protecting and nourishing neurons (glial cells), and to immune cells (lymphocytes).The main manifestations of damage to the nervous structures and blood vessels of the brain:
- Headache and migraine, the most common symptoms in SLE
- Irritability, depression - rare
- Psychoses: paranoia or hallucinations
- brain stroke
- Chorea, parkinsonism - rare
- Myelopathy, neuropathy and other disorders of the formation of nerve sheaths (myelin)
- Mononeuritis, polyneuritis, aseptic meningitis
Digestive tract injury
Clinical lesions of the digestive tract are diagnosed in 20% of patients with SLE.- Damage to the esophagus, violation of the act of swallowing, expansion of the esophagus occurs in 5% of cases
- Ulcers of the stomach and 12th intestine are caused both by the disease itself and by the side effects of treatment.
- Abdominal pain as a manifestation of SLE, and can also be caused by pancreatitis, inflammation of the intestinal vessels, intestinal infarction
- Nausea, abdominal discomfort, indigestion
- Hypochromic normocytic anemia occurs in 50% of patients, the severity depends on the activity of SLE. Hemolytic anemia is rare in SLE.
- Leukopenia is a decrease in white blood cells. It is caused by a decrease in lymphocytes and granulocytes (neutrophils, eosinophils, basophils).
- Thrombocytopenia is a decrease in platelets in the blood. It occurs in 25% of cases, caused by the formation of antibodies against platelets, as well as antibodies to phospholipids (fats that make up cell membranes).
Diagnosis of SLE
Diagnosis of SLE is based on data from the clinical manifestations of the disease, as well as data from laboratory and instrumental studies. The American College of Rheumatology has developed special criteria by which it is possible to make a diagnosis - systemic lupus erythematosus.Criteria for the diagnosis of systemic lupus erythematosus
The diagnosis of SLE is made if at least 4 out of 11 criteria are present.
| Characteristic: without erosion, peripheral, manifested by pain, swelling, accumulation of insignificant fluid in the joint cavity |
| Red in color, oval, round or annular in shape, plaques with uneven contours on their surface are scales, dilated capillaries nearby, scales are difficult to separate. Untreated lesions leave scars. |
| The oral mucosa or nasopharyngeal mucosa is affected in the form of ulcerations. Usually painless. |
| Increased sensitivity to sunlight. As a result of exposure to sunlight, a rash appears on the skin. |
| Specific rash in the form of a butterfly |
| Permanent loss of protein in the urine 0.5 g/day, excretion of cellular casts |
| Pleurisy is an inflammation of the membranes of the lungs. It is manifested by pain in the chest, aggravated by inhalation. Pericarditis - inflammation of the lining of the heart |
| Convulsions, Psychosis - in the absence of drugs that can provoke them or metabolic disorders (uremia, etc.) |
|
|
|
|
| Elevated anti-nuclear antibodies (ANA) |
The degree of disease activity is determined by special SLEDAI indices ( Systemic lupus erythematosus disease activity index). The disease activity index includes 24 parameters and reflects the state of 9 systems and organs, expressed in points that are summarized. Maximum 105 points, which corresponds to very high disease activity.
Disease activity indices bySLEDAI
| Manifestations | Description | Punctuation |
| Pseudo-epileptic seizure(development of convulsions without loss of consciousness) | It is necessary to exclude metabolic disorders, infections, medications that could provoke it. | 8 |
| psychoses | Violation of the ability to perform actions in the usual mode, impaired perception of reality, hallucinations, decreased associative thinking, disorganized behavior. | 8 |
| Organic changes in the brain | Changes in logical thinking, orientation in space is disturbed, memory, intelligence, concentration, incoherent speech, insomnia or drowsiness are reduced. | 8 |
| Eye disorders | Inflammation of the optic nerve, excluding arterial hypertension. | 8 |
| Damage to the cranial nerves | Damage to the cranial nerves revealed for the first time. | |
| Headache | Severe, persistent, may be migraineous, not responding to narcotic analgesics | 8 |
| Cerebral circulatory disorders | First detected, excluding the consequences of atherosclerosis | 8 |
| Vasculitis-(vascular damage) | Ulcers, gangrene of the extremities, painful knots on the fingers | 8 |
| Arthritis- (inflammation of the joints) | Damage to more than 2 joints with signs of inflammation and swelling. | 4 |
| Myositis- (inflammation of skeletal muscles) | Muscle pain, weakness with confirmation of instrumental studies | 4 |
| Cylinders in the urine | Hyaline, granular, erythrocyte | 4 |
| erythrocytes in urine | More than 5 red blood cells in the field of view, exclude other pathologies | 4 |
| Protein in the urine | More than 150 mg per day | 4 |
| Leukocytes in urine | More than 5 white blood cells in the field of view, excluding infections | 4 |
| Skin lesions | Inflammatory damage | 2 |
| Hair loss | Enlargement of lesions or complete hair loss | 2 |
| Mucosal ulcers | Ulcers on the mucous membranes and on the nose | 2 |
| Pleurisy- (inflammation of the membranes of the lungs) | Chest pain, pleural thickening | 2 |
| Pericarditis-( inflammation of the lining of the heart) | Detected on ECG, echocardiography | 2 |
| Decreased compliment | Decreased C3 or C4 | 2 |
| AntiDNA | Positively | 2 |
| Temperature | More than 38 degrees C, excluding infections | 1 |
| Decrease in blood platelets | Less than 150 10 9 /l, excluding medicines | 1 |
| Decrease in white blood cells | Less than 4.0 10 9 /l, excluding medicines | 1 |
- Light activity: 1-5 points
- Moderate activity: 6-10 points
- High activity: 11-20 points
- Very high activity: more than 20 points
Diagnostic tests used to detect SLE
- ANA- screening test, specific antibodies to cell nuclei are determined, is determined in 95% of patients, does not confirm the diagnosis in the absence of clinical manifestations of systemic lupus erythematosus
- Anti DNA– antibodies to DNA, determined in 50% of patients, the level of these antibodies reflects the activity of the disease
- Anti-sm- specific antibodies to the Smith antigen, which is part of short RNA, are detected in 30-40% of cases
- Anti-SSA or Anti-SSB, antibodies to specific proteins located in the cell nucleus, are present in 55% of patients with systemic lupus erythematosus, are not specific for SLE, and are also detected in other connective tissue diseases
- Anticardiolipin - antibodies to mitochondrial membranes (energy station of cells)
- Antihistones- antibodies against proteins necessary for packaging DNA into chromosomes, characteristic of drug-induced SLE.
- Markers of inflammation
- ESR - increased
- C - reactive protein, elevated
- Compliment level lowered
- C3 and C4 are reduced as a result of excessive formation of immune complexes
- Some people are born with reduced compliment levels, a predisposing factor for developing SLE.
- General blood analysis
- Possible decrease in red blood cells, white blood cells, lymphocytes, platelets
- Analysis of urine
- Protein in the urine (proteinuria)
- Red blood cells in the urine (hematuria)
- Casts in the urine (cylindruria)
- White blood cells in urine (pyuria)
- Blood chemistry
- Creatinine - an increase indicates kidney damage
- ALAT, ASAT - an increase indicates liver damage
- Creatine kinase - increases with damage to the muscular apparatus
- X-ray of the joints
- X-ray and computed tomography of the chest
- Nuclear magnetic resonance and angiography
- echocardiography
Specific Procedures
- A lumbar puncture can help rule out infectious causes of neurological symptoms.
- A biopsy (analysis of organ tissue) of the kidneys allows you to determine the type of glomerulonephritis and facilitate the choice of treatment tactics.
- A skin biopsy allows you to clarify the diagnosis and exclude similar dermatological diseases.
Treatment of systemic lupus
Despite significant advances in the modern treatment of systemic lupus erythematosus, this task remains very difficult. Treatment aimed at eliminating the main cause of the disease has not been found, just as the cause itself has not been found. Thus, the principle of treatment is aimed at eliminating the mechanisms of the development of the disease, reducing provoking factors and preventing complications. - Eliminate physical and mental stress conditions
- Reduce sun exposure, use sunscreen
- Glucocorticosteroids the most effective drugs in the treatment of SLE.
Dosing regimens:
- Inside:
- Initial dose of prednisolone 0.5 - 1 mg / kg
- Maintenance dose 5-10 mg
- Prednisolone should be taken in the morning, the dose is reduced by 5 mg every 2-3 weeks
- High-dose intravenous methylprednisolone (pulse therapy)
- Dose 500-1000 mg/day, for 3-5 days
- Or 15-20 mg/kg body weight
Indications for pulse therapy: young age, fulminant lupus nephritis, high immunological activity, damage to the nervous system.
- 1000 mg methylprednisolone and 1000 mg cyclophosphamide on the first day
- Cytostatics: cyclophosphamide (cyclophosphamide), azathioprine, methotrexate, are used in the complex treatment of SLE.
- Acute lupus nephritis
- Vasculitis
- Forms resistant to treatment with corticosteroids
- The need to reduce doses of corticosteroids
- High SLE activity
- Progressive or fulminant course of SLE
- Cyclophosphamide with pulse therapy 1000 mg, then every day 200 mg until a total dose of 5000 mg is reached.
- Azathioprine 2-2.5 mg/kg/day
- Methotrexate 7.5-10 mg/week, by mouth
- Anti-inflammatory drugs
- Naklofen, nimesil, aertal, catafast, etc.
- Aminoquinoline drugs
- delagil, plaquenil, etc.
- Biologicals are a promising treatment for SLE
- Anti CD 20 - Rituximab
- Tumor necrosis factor alpha - Remicade, Gumira, Embrel
- Other drugs
- Anticoagulants (heparin, warfarin, etc.)
- Antiplatelet agents (aspirin, clopidogrel, etc.)
- Diuretics (furosemide, hydrochlorothiazide, etc.)
- Calcium and potassium preparations
- Methods of extracorporeal treatment
- Plasmapheresis is a method of blood purification outside the body, in which part of the blood plasma is removed, and with it the antibodies that cause SLE disease.
- Hemosorption is a method of purifying blood outside the body using specific sorbents (ion exchange resins, Activated carbon and etc.).
What are the complications and prognosis for life with systemic lupus erythematosus?
The risk of developing complications of systemic lupus erythematosus directly depends on the course of the disease.Variants of the course of systemic lupus erythematosus:
1.
Acute course- is characterized by a lightning-fast onset, a rapid course and the rapid simultaneous development of symptoms of damage to many internal organs (lungs, heart, central nervous system, and so on). The acute course of systemic lupus erythematosus, fortunately, is rare, since this option quickly and almost always leads to complications and can cause the death of the patient.
2.
Subacute course- characterized by a gradual onset, a change in periods of exacerbations and remissions, a predominance of general symptoms (weakness, weight loss, subfebrile temperature (up to 38 0
C) and others), damage to internal organs and complications occur gradually, not earlier than 2-4 years after the onset of the disease.
3.
chronic course- the most favorable course of SLE, there is a gradual onset, damage mainly to the skin and joints, longer periods of remission, damage to internal organs and complications occur after decades.
Damage to organs such as the heart, kidneys, lungs, central nervous system, and blood, which are described as symptoms of the disease, in fact, are complications of systemic lupus erythematosus.
But it is possible to distinguish complications that lead to irreversible consequences and can lead to the death of the patient:
1. Systemic lupus erythematosus- affects the connective tissue of the skin, joints, kidneys, blood vessels and other body structures.
2. medicinal lupus erythematosus- unlike the systemic form of lupus erythematosus, a completely reversible process. Drug-induced lupus develops as a result of exposure to certain drugs:
- Medicinal products for the treatment of cardiovascular diseases: phenothiazine groups (Apressin, Aminazine), Hydralazine, Inderal, Metoprolol, Bisoprolol, Propranolol and some others;
- antiarrhythmic drug Novocainamide;
- sulfonamides: Biseptol and others;
- anti-tuberculosis drug Isoniazid;
- oral contraceptives;
- drugs plant origin for the treatment of vein diseases (thrombophlebitis, varicose veins of the lower extremities, and so on): horse chestnut, venotonic Doppelhertz, Detralex and some others.
3. Discoid (or cutaneous) lupus erythematosus may precede the development of systemic lupus erythematosus. With this type of disease, the skin of the face is affected to a greater extent. Changes on the face are similar to those in systemic lupus erythematosus, but blood tests (biochemical and immunological) do not have changes characteristic of SLE, and this will be the main criterion for differential diagnosis with other types of lupus erythematosus. To clarify the diagnosis, it is necessary to conduct a histological examination of the skin, which will help to differentiate from diseases similar in appearance (eczema, psoriasis, skin form of sarcoidosis, and others).
4. neonatal lupus erythematosus occurs in newborn babies whose mothers suffer from systemic lupus erythematosus or other systemic autoimmune diseases. At the same time, the mother may not have symptoms of SLE, but autoimmune antibodies are detected during their examination.
Symptoms of neonatal lupus erythematosus the child usually manifests itself before the age of 3 months:
- changes on the skin of the face (often look like a butterfly);
- congenital arrhythmia, which is often determined by ultrasound of the fetus in the II-III trimesters of pregnancy;
- lack of blood cells in the general blood test (decrease in the level of erythrocytes, hemoglobin, leukocytes, platelets);
- detection of autoimmune antibodies specific for SLE.
5. Also, the term "lupus" is used for tuberculosis of the skin of the face - tuberculous lupus. Tuberculosis of the skin is very similar in appearance to the systemic lupus erythematosus butterfly. The diagnosis will help to establish a histological examination of the skin and microscopic and bacteriological examination of the scraping - Mycobacterium tuberculosis (acid-fast bacteria) is detected.
A photo:
this is what tuberculosis of the skin of the face or tuberculous lupus looks like.
Systemic lupus erythematosus and other systemic connective tissue diseases, how to differentiate?
Group systemic diseases connective tissue:- Systemic lupus erythematosus.
- Idiopathic dermatomyositis (polymyositis, Wagner's disease)- defeat by autoimmune antibodies of smooth and skeletal muscles.
- Systemic scleroderma is a disease in which normal tissue is replaced by connective tissue (which does not carry functional properties), including blood vessels.
- Diffuse fasciitis (eosinophilic)- damage to the fascia - structures that are cases for skeletal muscles, while in the blood of most patients there is an increased number of eosinophils (blood cells responsible for allergies).
- Sjögren's syndrome- damage to various glands (lacrimal, salivary, sweat, and so on), for which this syndrome is also called dry.
- Other systemic diseases.
Differential diagnosis of systemic connective tissue diseases.
| Diagnostic criteria | Systemic lupus erythematosus | Systemic scleroderma | Idiopathic dermatomyositis |
| The onset of the disease |
|
 A photo: Raynaud's syndrome |
|
| Temperature | Prolonged fever, body temperature above 38-39 0 C. | Prolonged subfebrile condition (up to 38 0 С). | Moderate prolonged fever (up to 39 0 C). |
| Appearance of the patient (at the beginning of the disease and in some of its forms appearance patient may not be changed in all these diseases) | Skin lesions, mostly of the face, "butterfly" (redness, scales, scars). Rashes can be all over the body and on the mucous membranes. Dry skin, loss of hair, nails. Nails are deformed, striated nail plates. Also, throughout the body there may be hemorrhagic rashes (bruises and petechiae).  | The face can acquire a “mask-like” expression without facial expressions, stretched, the skin is shiny, deep folds appear around the mouth, the skin is motionless, tightly soldered to deep-lying tissues. Often there is a violation of the glands (dry mucous membranes, as in Sjögren's syndrome). Hair and nails fall out. Dark spots on the skin of the extremities and neck against the background of "bronze skin".  | A specific symptom is swelling of the eyelids, their color may be red or purple, on the face and in the décolleté area there is a varied rash with reddening of the skin, scales, hemorrhages, scars. With the progression of the disease, the face acquires a “mask-like appearance”, without facial expressions, stretched, may be skewed, and drooping of the upper eyelid (ptosis) is often detected.  |
| The main symptoms during the period of disease activity |
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| Forecast | Chronic course, over time, more and more organs are affected. Without treatment, complications develop that threaten the life of the patient. With adequate and regular treatment, it is possible to achieve a long-term, stable remission. | ||
| Laboratory indicators |
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| Principles of treatment | Long-term hormonal therapy (Prednisolone) + cytostatics + symptomatic therapy and other drugs (see article section "Treatment of systemic lupus"). | ||
As you can see, there is not a single analysis that would completely differentiate systemic lupus erythematosus from other systemic diseases, and the symptoms are very similar, especially on early stages. It is often enough for experienced rheumatologists to assess the skin manifestations of the disease to diagnose systemic lupus erythematosus (if any).
Systemic lupus erythematosus in children, what are the features of symptoms and treatment?
Systemic lupus erythematosus is less common in children than in adults. In childhood, rheumatoid arthritis is more often detected from autoimmune diseases. SLE predominantly (in 90% of cases) affects girls. Systemic lupus erythematosus can occur in infants and young children, though rarely, the largest number cases of this disease occur during puberty, namely at the age of 11-15 years. Given the peculiarity of immunity, hormonal levels, growth intensity, systemic lupus erythematosus in children proceeds with its own characteristics.
Features of the course of systemic lupus erythematosus in childhood:
- more severe disease , high activity of the autoimmune process;
- chronic course disease in children occurs only in a third of cases;
- more common acute or subacute course diseases with rapid damage to internal organs;
- also isolated only in children acute or fulminant course SLE - almost simultaneous damage to all organs, including the central nervous system, which can lead to the death of a small patient in the first six months from the onset of the disease;
- frequent development of complications and high mortality;
- the most common complication is bleeding disorder in the form of internal bleeding, hemorrhagic eruptions (bruises, hemorrhages on the skin), as a result - the development of a shock state of DIC - disseminated intravascular coagulation;
- systemic lupus erythematosus in children often occurs in the form of vasculitis - inflammation of the blood vessels, which determines the severity of the process;
- children with SLE are usually malnourished , have a pronounced deficiency of body weight, up to cachexia (extreme degree of dystrophy).
1.
The onset of the disease acute, with an increase in body temperature to high numbers (over 38-39 0 C), with pain in the joints and severe weakness, a sharp loss of body weight.
2.
Skin changes in the form of a "butterfly" in children are relatively rare. But, given the development of a lack of blood platelets, a hemorrhagic rash is more common throughout the body (bruises for no reason, petechiae or pinpoint hemorrhages). Also, one of the characteristic signs of systemic diseases is hair loss, eyelashes, eyebrows, up to complete baldness. The skin becomes marbled, very sensitive to sunlight. There may be various rashes on the skin that are characteristic of allergic dermatitis. In some cases, Raynaud's syndrome develops - a violation of the circulation of the hands. In the oral cavity there may be long-term non-healing sores - stomatitis.
3.
Joint pain- a typical syndrome of active systemic lupus erythematosus, the pain is periodic. Arthritis is accompanied by the accumulation of fluid in the joint cavity. Pain in the joints over time is combined with pain in the muscles and stiffness of movement, starting with the small joints of the fingers.
4.
For children characterized by the formation of exudative pleurisy(fluid in the pleural cavity), pericarditis (fluid in the pericardium, membrane of the heart), ascites and other exudative reactions (dropsy).
5.
Heart failure in children, it usually manifests as myocarditis (inflammation of the heart muscle).
6.
Kidney damage or nephritis much more often develops in childhood than in adults. Such nephritis relatively quickly leads to the development of acute renal failure (requiring intensive care and hemodialysis).
7.
Lung injury is rare in children.
8.
In the early period of the disease in adolescents, in most cases, there is defeat gastrointestinal tract
(hepatitis, peritonitis, etc.).
9.
Damage to the central nervous system in children it is characterized by capriciousness, irritability, in severe cases, convulsions may develop.
That is, in children, systemic lupus erythematosus is also characterized by a variety of symptoms. And many of these symptoms are masked under the guise of other pathologies, the diagnosis of systemic lupus erythematosus is not immediately assumed. Unfortunately, after all, timely treatment is the key to success in the transition of an active process into a period of stable remission.
Diagnostic principles systemic lupus erythematosus are the same as in adults, based mainly on immunological studies (detection of autoimmune antibodies).
In the general blood test, in all cases and from the very beginning of the disease, a decrease in the number of all blood cells (erythrocytes, leukocytes, platelets) is determined, blood clotting is impaired.
Treatment of systemic lupus erythematosus in children, as in adults, involves long-term use of glucocorticoids, namely Prednisolone, cytostatics and anti-inflammatory drugs. Systemic lupus erythematosus is a diagnosis that requires urgent hospitalization of the child in a hospital (rheumatology department, with the development of severe complications - in the intensive care unit or intensive care unit).
In a hospital, a complete examination of the patient is carried out and the necessary therapy is selected. Depending on the presence of complications, symptomatic and intensive therapy. Given the presence of bleeding disorders in such patients, injections of Heparin are often prescribed.
In the case of timely started and regular treatment, it is possible to achieve stable remission, while children grow and develop according to age, including normal puberty. In girls, a normal menstrual cycle is established and pregnancy is possible in the future. In this case forecast favorable for life.
Systemic lupus erythematosus and pregnancy, what are the risks and features of treatment?
As already mentioned, young women often suffer from systemic lupus erythematosus, and for any woman, the issue of motherhood is very important. But SLE and pregnancy is always a big risk for both the mother and the unborn baby. Pregnancy risks for a woman with systemic lupus erythematosus:
1. Systemic lupus erythematosus
In most cases does not affect the ability to get pregnant
,
as well as long-term use of prednisolone.
2. When taking cytostatics (Methotrexate, Cyclophosphamide and others), it is absolutely impossible to become pregnant
,
since these drugs will affect germ cells and embryonic cells; pregnancy is possible only not earlier than six months after the abolition of these drugs.
3. Half
cases of pregnancy with SLE ends with the birth of healthy, full-term baby
. At 25%
cases such children are born premature
, but in a quarter of cases
observed miscarriage
.
4. Possible Complications pregnancy with systemic lupus erythematosus,
in most cases associated with damage to the vessels of the placenta:
- fetal death;
- . So, in a third of cases, an aggravation of the course of the disease develops. The risk of such deterioration is maximum in the first weeks of I, or in the III trimester of pregnancy. And in other cases, a temporary retreat of the disease is observed, but for the most part one should expect a strong exacerbation of systemic lupus erythematosus 1-3 months after birth. No one knows which path the autoimmune process will take.
6. Pregnancy can be a trigger in the development of the onset of systemic lupus erythematosus. Also, pregnancy can provoke the transition of discoid (cutaneous) lupus erythematosus to SLE.
7. Mother with systemic lupus erythematosus can pass genes to her baby that predispose him to develop a systemic autoimmune disease during his lifetime.
8. The child may develop neonatal lupus erythematosus associated with the circulation of maternal autoimmune antibodies in the blood of the baby; this condition is temporary and reversible.- It is necessary to plan a pregnancy under the supervision of qualified doctors , namely a rheumatologist and a gynecologist.
- It is advisable to plan a pregnancy during a period of persistent remission chronic course of SLE.
- In case of acute systemic lupus erythematosus with the development of complications, pregnancy can adversely affect not only health, but also lead to the death of a woman.
- And if, nevertheless, pregnancy occurred during an exacerbation, then the question of its possible preservation is decided by the doctors, together with the patient. After all, exacerbation of SLE requires long-term use of drugs, some of which are absolutely contraindicated during pregnancy.
- Pregnancy is recommended no earlier than 6 months after discontinuation of cytotoxic drugs (Methotrexate and others).
- With lupus lesion of the kidneys and heart there can be no talk of pregnancy, this can lead to a woman's death from kidney and / or heart failure, because it is these organs that are under a huge load when carrying a baby.
1. Essential throughout pregnancy observed by a rheumatologist and an obstetrician-gynecologist , the approach to each patient is only individual.
2. Be sure to follow the rules: do not overwork, do not be nervous, eat normally.
3. Pay close attention to any changes in your health.
4. Delivery outside the maternity hospital is unacceptable , as there is a risk of developing severe complications during and after childbirth.
7. Even at the very beginning of pregnancy, a rheumatologist prescribes or corrects therapy. Prednisolone is the main drug for the treatment of SLE and is not contraindicated during pregnancy. The dose of the drug is selected individually.
8. Also recommended for pregnant women with SLE taking vitamins, potassium supplements, aspirin (up to the 35th week of pregnancy) and other symptomatic and anti-inflammatory drugs.
9. Mandatory treatment of late toxicosis and other pathological conditions of pregnancy in a maternity hospital.
10. After childbirth the rheumatologist increases the dose of hormones; in some cases, it is recommended to stop breastfeeding, as well as the appointment of cytostatics and other drugs for the treatment of SLE - pulse therapy, since it is the postpartum period that is dangerous for the development of severe exacerbations of the disease.Previously, all women with systemic lupus erythematosus were advised not to become pregnant, and in the event of conception, all were recommended artificial termination of pregnancy (medical abortion). Now, doctors have changed their opinion on this matter, you can’t deprive a woman of motherhood, especially since there are considerable chances to give birth to a normal healthy baby. But everything must be done in order to minimize the risk to mother and baby.
Is lupus erythematosus contagious?
Of course, any person who sees strange rashes on the face thinks: “Maybe it’s contagious?”. Moreover, people with these rashes walk for so long, feel unwell and constantly take some kind of medication. Moreover, earlier doctors also assumed that systemic lupus erythematosus is transmitted sexually, by contact, or even by airborne droplets. But having studied the mechanism of the disease in more detail, scientists completely dispelled these myths, because this is an autoimmune process.The exact cause of the development of systemic lupus erythematosus has not yet been established, there are only theories and assumptions. It all boils down to one thing, that the underlying cause is the presence of certain genes. But still, not all carriers of these genes suffer from systemic autoimmune diseases.
The trigger mechanism for the development of systemic lupus erythematosus can be:
- various viral infections;
- bacterial infections (especially beta-hemolytic streptococcus);
- stress factors;
- hormonal changes (pregnancy, adolescence);
- factors environment (for example, ultraviolet radiation).
Only tuberculous lupus can be contagious (tuberculosis of the skin of the face), since a large number of tuberculosis sticks are detected on the skin, while the contact route of transmission of the pathogen is isolated.
Lupus erythematosus, what diet is recommended and are there any methods of treatment with folk remedies?
As with any disease, nutrition plays an important role in lupus erythematosus. Moreover, with this disease, there is almost always a deficiency, or against the background of hormonal therapy - excess body weight, lack of vitamins, trace elements and biologically active substances.The main characteristic of the SLE diet is a balanced and proper diet.
1. foods containing unsaturated fatty acids (Omega-3):
- sea fish;
- many nuts and seeds;
- vegetable oil in in large numbers;
3. juices, fruit drinks;
4. lean poultry meat: chicken, turkey fillet;
5. low-fat dairy , especially dairy products (low-fat cheese, cottage cheese, yogurt);
6. cereals and vegetable fiber (grain bread, buckwheat, oatmeal, wheat germ and many others).1. Foods with saturated fatty acids have a bad effect on blood vessels, which can aggravate the course of SLE:
- animal fats;
- fried food;
- fatty meats (red meat);
- dairy products with high fat content and so on.
Photo: alfalfa grass.
3. Garlic - powerfully stimulates the immune system.
4. Salty, spicy, smoked dishes holding fluid in the body.If diseases of the gastrointestinal tract occur against the background of SLE or taking medications, then the patient is recommended frequent fractional meals according to therapeutic diet- table number 1. All anti-inflammatory drugs are best taken with or immediately after meals.
Treatment of systemic lupus erythematosus at home is possible only after the selection of an individual treatment regimen in a hospital setting and the correction of conditions that threaten the life of the patient. Heavy drugs used in the treatment of SLE cannot be prescribed on their own, self-medication will not lead to anything good. Hormones, cytostatics, non-steroidal anti-inflammatory drugs and other drugs have their own characteristics and a bunch of adverse reactions, and the dose of these drugs is very individual. The therapy selected by doctors is taken at home, strictly adhering to the recommendations. Omissions and irregularity in taking medications are unacceptable.
Concerning traditional medicine recipes, then systemic lupus erythematosus does not tolerate experiments. None of these remedies will prevent the autoimmune process, you can just lose precious time. Folk remedies can give their effectiveness if they are used in combination with traditional methods of treatment, but only after consultation with a rheumatologist.
Some traditional medicines for the treatment of systemic lupus erythematosus:
Precautionary measures! All folk remedies containing poisonous herbs or substances should be out of the reach of children. One must be careful with such remedies, any poison is a medicine as long as it is used in small doses.Photo, what do the symptoms of lupus erythematosus look like?
A photo: changes on the skin of the face in the form of a butterfly in SLE.
Photo: skin lesions of the palms with systemic lupus erythematosus. In addition to skin changes, this patient shows thickening of the joints of the phalanges of the fingers - signs of arthritis.
Dystrophic changes in nails with systemic lupus erythematosus: fragility, discoloration, longitudinal striation of the nail plate.
Lupus lesions of the oral mucosa . According to the clinical picture, they are very similar to infectious stomatitis, which do not heal for a long time.
And this is what they might look like early symptoms of discoid or cutaneous lupus erythematosus.
And this is what it might look like neonatal lupus erythematosus, these changes, fortunately, are reversible and in the future the baby will be absolutely healthy.
Skin changes in systemic lupus erythematosus characteristic of childhood. The rash is hemorrhagic in nature, reminiscent of measles rashes, leaves pigment spots that do not go away for a long time.
The Yauza Clinical Hospital provides diagnostics (examination by a specialist, blood and urine tests) and comprehensive treatment of systemic lupus erythematosus. We use the most effective modern methods of treatment: using biological genetically engineered drugs, extracorporeal hemocorrection.
- 40–50 cases per 100,000 population - the incidence of SLE. 70–90% of patients with lupus are women of childbearing age
- Up to 85% of cases, stable remission lasting up to 30 years is possible with complex treatment of SLE
- 65% of patients with severe lupus improve with the use of genetically engineered drugs
Systemic lupus erythematosus (SLE)- a chronic inflammatory systemic, i.e., potentially affecting various organs and systems of the human body, an autoimmune disease. In this case, the immune system "attacks" the body's own structures (skin, joints, kidneys, etc.), which leads to inflammation in these organs.
Individual treatment plan
Our specialists determine the tactics of treatment, taking into account the variety of forms and clinical manifestations, based on the activity, severity of the disease and the number of affected organs and systems.
Steroid drugs
Cutaneous forms of the disease respond well to treatment with topical steroid-containing agents.
Non-steroidal anti-inflammatory drugs
For joint pain, non-steroidal anti-inflammatory drugs (NSAIDs) (ibuprofen, diclofenac, meloxicam, naproxen, nimesulide, celecoxib, etoricoxib) are most often used. These drugs are recommended to be prescribed in short courses, with caution in diseases of the gastrointestinal tract, kidneys, liver, and in persons with an increased risk of cardiovascular complications.
Antimalarial drugs
In mild SLE with lesions of the skin and joints, it may be sufficient to prescribe antimalarial drugs (hydroxychloroquine), which have proven themselves in this disease.
Glucocorticosteroid hormones and immunosuppressive drugs
In severe cases with damage to internal organs, glucocorticosteroid hormones (prednisolone, methylprednisolone) and immunosuppressive drugs (azathioprine, methotrexate, cyclophosphamide, mycophenolate mofetil) are prescribed.
New! Biological genetically engineered drugs
We use the most modern treatment methods. Recently, the first biological (genetically engineered) drug, belimumab, has been developed and officially registered to better control the activity of the disease.
New! Methods of extracorporeal hemocorrection
High efficiency in the treatment of systemic lupus erythematosus is shown by methods of extracorporeal hemocorrection (cryoapheresis, cell mass incubation, immunosorption, plasma cascade filtration, lymphocytapheresis, etc.). They allow you to remove pathological components from the blood that cause and maintain autoimmune inflammation, thereby accelerating the onset of remission, as well as reduce the doses of medications while increasing their effectiveness.
Non-pharmacological methods of treatment and prevention
An important aspect of treatment is the timely correction of cardiovascular risk factors (smoking, hypertension, high cholesterol), as well as the prevention of osteoporosis (calcium and vitamin D preparations).
Observation
The rheumatologist monitors the patient's condition throughout the treatment of SLE. To monitor the effectiveness of the therapy, the doctor performs regular diagnostics and, if necessary, adjusts the treatment.
Forecast
Despite the fact that this is a chronic inflammatory autoimmune disease that affects various vital organs, the prognosis of SLE has improved significantly in recent years, primarily due to continuous improvement in diagnostic and therapeutic capabilities.
Important! Fundamental is the fact that treatment must always be matched to the activity of the disease in order to avoid adverse effects of both the disease and the therapy.
Pregnancy with SLE
Separate difficult problem is the planning and management of pregnancy in women with lupus. It is especially relevant due to the fact that the disease is most common in young women of childbearing age. Pregnancy planning is possible only with a complete stable remission of the renal process, stable kidney function and blood pressure. Recent studies have shown that the outcome of pregnancy directly depends on the degree and duration of remission of the disease before conception. During pregnancy, systematic monitoring of both a gynecologist and a rheumatologist is mandatory. Regular monitoring of disease activity, blood pressure, and fetal development is especially important.
Why are we
- Doctors. The appointment is conducted by a highly qualified rheumatologist with extensive experience in Russia and abroad.
- Complexity. The treatment of systemic lupus erythematosus is carried out in close cooperation between different specialists of our medical center (rheumatologists, transfusiologists, gynecologists).
- Individual approach Specialists draw up an individual treatment plan for each patient in accordance with the form and activity of the disease.
- Innovation Possibility effective treatment severe forms of SLE with the latest genetically engineered biological preparations, methods of extracorporeal hemocorrection.
- Efficiency Rapid achievement of remission of the disease and an increase in its duration, reduction of the intensity of possible subsequent exacerbations, improvement of the patient's well-being, prevention of disability.
Service cost
Service prices You can look at or check by phone listed on the site.