- Causes of development of SLE
- Types of SLE
- Pathogenesis of SLE or what happens in the body
- Discovery of scientists
- Lupus abnormalities
- Differential diagnosis
- Treatment of lupus erythematosus
- Prognosis and prevention
Systemic lupus erythematosus or SLE refers to a group of systemic autoimmune diseases with unknownetiological factor. The disease develops due to genetically caused failures of immune regulation, which determine the formation of organonesspecific antibodies to cell-cell antigens and lead to the development of so-called immune inflammation in the tissues of organs.
This disease is also called multisystem inflammatory disease, since almost all organs and systems are affected: joints, skin, kidneys, brain, etc.
The risk of developing SLE includes young women of childbearing age, especially the Negroid race - about 70% of cases of SLE are diagnosed in this group of the population. However, SLE can develop at absolutely any age, even d
Causes of the development of SLE
The reasons for the development of SLE have not been established to date. There are several theories of development of systemic lupus erythematosus, which are controversial and have both confirmatory and refuting factors:
- Genetic theory .According to this theory, the disease is genetically conditioned. However, a specific gene provoking the development of SLE has not yet been detected.
- Viral Theory. It was revealed that in patients with SLE, the Epstein-Barr virus is often found.
- Bacterial theory of .It is proved that the DNA of a number of bacteria can stimulate the synthesis of antinuclear autoantibodies.
- Hormonal theory. Women suffering from SLE often have an increased level of prolactin hormones and estrogen. There is also a frequent primary manifestation of SLE during pregnancy or after childbirth, when the female body undergoes huge hormonal changes.
- The effect of physical factors. It is known that ultraviolet radiation can trigger the synthesis of skin cells by autoantibodies( in people predisposed to SLE).
None of the above theories can explain the cause of the disease with absolute accuracy. As a consequence, SLE is said to be a polyethiologic disease, i. E.having several causes of occurrence.
Types of SCR
The disease is classified according to the stages of the course of the disease:
Acute form of , when there is a lupus erythematosus, the symptoms are characterized by a sudden and abrupt manifestation: a significant temperature rise up to febrile indices, rapid damage to several organs, high immunological activity.
The subacute form of is characterized by the periodicity of exacerbations, however, with a lesser degree of symptomatology than with acute SLE.The defeat of the organs develops within the first 12 months of the disease.
The chronic form of is characterized by a long-term manifestation of one or more symptoms. A combination of SLE with an antiphospholipid syndrome with a chronic form of the disease is especially characteristic.
Pathogenesis of SLE or what happens in the body
Under the influence of a certain causative factor, or when combined in a dysfunction of the immune system, the DNA of different cells is "exposed".These cells are perceived by their own organism as foreign or antigens. The body immediately begins to produce specific antibody proteins, which are specific to these cells and protect against them. As a result of the interaction of antibodies and antigens, immune complexes are formed that are fixed in certain organs.
This process leads to the development of an immune inflammatory reaction and cell damage. The most commonly affected connective tissue cells, therefore, SLE is attributed to diseases of this particular tissue of the body. Connective tissue is widely represented in all organs and systems, therefore practically the whole organism is involved in the pathological lupus process.
Immune complexes with fixation on vascular walls can provoke thrombus formation. Circulating antibodies have a toxic effect and lead to anemia and thrombocytopenia.
The discovery of
scientists One of the last two studies, according to scientists, was a mechanism that controls the aggression of the human body against its own tissues and cells. This opens up new opportunities for developing additional diagnostic techniques and will allow the development of effective ways of treating SLE.
This discovery occurred when the American Agency for Quality Control of Medicines was about to announce a decision about the use of the biological drug "Benlist".This new drug, Benlista( USA), has now been approved for use in the treatment of lupus erythematosus.
The essence of the discovery is as follows.
With SLE, the body produces antibodies against its own DNA, called antinuclear antibodies( ANA).Thus, a blood test for ANA in a patient with suspected SLE will correctly interpret the diagnosis.
The main mystery of SLE was the mechanism of DNA cell penetration outside. In 2004, it was found that the explosive death of neutrophil cells results in the ejection of their contents, including nuclear DNA, outwardly in the form of strands, between which pathogenic viruses, fungi and bacteria are easily entangled. In healthy people, such neutrophil traps easily break up in the intercellular space. In people who suffer from SLE, the antimicrobial proteins LL37 and HNP do not allow the destruction of nuclear DNA residues.
These proteins and DNA residues together are able to activate plasmacytoid dendritic cells, which in turn produce proteins( interferon) that support the immune response. Interferon forces neutrophils to release even more trap threads, supporting an endless pathological process.
Thus, according to scientists, the pathogenesis of lupus erythematosus lies in the cycle of cell death of neutrophils and chronic inflammation of tissues. This discovery is important for both diagnosis and treatment of SLE.If one of these proteins can become a marker of SLE - this will greatly simplify the diagnosis.
Another interesting fact. Among 118 patients participating in another study aimed at detecting vitamin D deficiency in patients with connective tissue diseases. Among 67 patients with autoimmune diseases( rheumatoid arthritis, lupus erythematosus), vitamin D deficiency was found in 52%, among 51 patients with pulmonary fibrosis of another nature - in 20%.What confirms the need and effectiveness of adding to the treatment of autoimmune diseases courses of vitamin D.
Symptoms of the disease depend on the stages of development of the pathological process.
In case of acute primary manifestation of of lupus erythematosus suddenly occurs:
- fever up to 39-39 C
- joint soreness
Often patients can specify the date of onset of clinical manifestations - so severe is the symptomatology. After 1-2 months, a clear defeat of vital organs is formed. If the disease progresses further, then a year or two patients die.
In subacute , the first symptoms are less pronounced, the pathological process develops more slowly - organ damage occurs gradually, within 1-1.5 years.
In the chronic course of the , several or several symptoms persist continuously for several years. The aggravation of the disease is rare, the work of vital organs is not violated.
Basically, the initial manifestations of SLE do not have specificity, they easily pass in the treatment with anti-inflammatory drugs, or independently. Remission is distinguished by the duration of the course. Sooner or later there is an exacerbation of the disease, most often in the autumn-summer periods due to the increase in solar radiation, while the skin condition in patients deteriorates sharply. Over time, there are symptoms of organ damage.
- Skin, nails and scalp
Involvement in the pathological process of the skin - the most common symptoms of lupus erythematosus in women, the occurrence of which is associated with some causal factor: prolonged exposure to sunlight, exposure to cold, psychoemotional shock( see urticaria - symptoms,treatment, allergy to the sun).
Very characteristic of SLE reddening of skin areas near the nose and cheeks, in shape similar to the wings of an insect butterfly. In addition to the face, erythema appears in open areas of the skin - the upper limbs, the decollete zone. Erythema is prone to peripheral proliferation.
In discoid lupus erythema skin erythema is replaced by inflammatory edema. This area gradually becomes denser and after some time atrophies with the formation of the scar. Foci of discoid lupus are found on different parts of the body, which indicates the dissemination of the process.
Another symptom of SLE is a capillary, which is manifested by redness, swelling and numerous hemorrhages in the form of small dots, localized on the pads of the fingers, soles and palms.
The defeat of hair in SLE is manifested by gradual partial or complete alopecia( see the causes of hair loss in women).During the period of exacerbation, a change in the structure of the nails is characteristic, often leading to the atrophy of the peri-oval pubis.
Focal alopecia or generalized, pruritus and urticaria are the most characteristic symptoms of SLE.In addition to cutaneous manifestations of patients, headache, joint pain, changes in kidney and heart function, mood swings from euphoria to aggression.
- Mucous membranes
Mucous membranes of the mouth and nose often suffer: redness appears, erosion( enanthema) on the mucosa and small mouth ulcers( see ointments for stomatitis, aphthous stomatitis in adults, treatment).In the formation of cracks, erosions and ulcers of the red border of the lips, lupus-cheilitis appears. Foci of lesions become dense, cyanotic-red plaques, which are painful when eating, are prone to ulceration, have clear boundaries and sometimes are covered with pity scales.
- Musculoskeletal system
Up to 90% of patients with SLE have joint lesions. Suffer small joints, more often the fingers of the hand( see arthritis of the fingers).The pathological process spreads symmetrically, leading to pain and stiffness in the joints. Often develop necrosis of bone aseptic nature. In addition to the joints of the hand, the femoral and knee joints suffer, which leads to their functional insufficiency. If the ligamentous apparatus is involved in the process, contractures of a non-permanent character develop, and in severe SLE - dislocations and subluxations.
- Respiratory organs
The lungs are most often affected with the development of bilateral pleurisies, acute lupus pneumonitis and pulmonary hemorrhages. The last two pathologies are life threatening.
- Cardiovascular system
In the prevailing majority of cases, the endocarditis of Liebman-Sachs develops with the involvement of the mitral valve in the pathological lupus process. Valvular valves are fused and a stenotic heart defect is formed. In the case of pericarditis, the pericardial sheets become thicker. Myocarditis provokes a painful syndrome in the chest area, an increase in the size of the heart. Often, small and medium vessels( including vital coronary arteries and cerebral vessels) are affected, and as a result, patients often die due to cerebral stroke and ischemic heart disease.
- Nervous system
Neurological symptoms are diverse, ranging from migraine and ending with transient type ischemic attacks and strokes. Possible epileptic seizures, cerebral ataxia, chorea. Peripheral neuropathy develops in a fifth of cases, in which inflammation of the optic nerve, which leads to loss of vision, is considered to be a very unfavorable phenomenon.
- Kidney. The severe course of SLE leads to the formation of lupus nephritis of various types.
When lupus erythematosus is diagnosed in children, there are initially signs of joint damage( arthralgia of the volatile nature, acute and subacute periarthritis) without progression, and typical skin lesions like erythematous rash, anemia appears. Should differentiate SLE with atopic dermatitis in children.
Chronic lupus erythematosus is differentiated from red flat lichen, tuberculous leukoplakia and lupus, early rheumatoid arthritis, Sjogren's syndrome( see dry mouth, dry eye syndrome, photophobia).With the defeat of the red border of the lips, the chronic SLE is differentiated from the abrasive pre-carcinogenic chehalitis Manganotti and actinic cheilitis.
Since the defeat of internal organs is always similar to the course with various infectious processes, SLE is differentiated from Lyme disease, syphilis, mononucleosis( infectious mononucleosis in children: symptoms), HIV infection( see symptoms of HIV infection - myth or reality), etc.
Treatment of lupus erythematosus
Treatment is selected individually for a particular patient. The complex of therapeutic measures is carried out on an outpatient basis.
Indications for hospitalization are:
- persistent hyperthermia for no apparent reason
- life threatening conditions: malignant renal failure, acute pneumonitis or bleeding from the lung
- neurological complications
- severe thrombocytopenia, significant decrease in red blood cells and blood lymphocytes
- lack of efficacy from outpatient treatment
Systemic lupus erythematosusin the acute period are treated with hormonal preparations( prednisolone, corticosteroid ointments see the list of hormonal ointmentsfrom psoriasis) and cytostatics( cyclophosphamide) according to the scheme. Non-steroidal anti-inflammatory drugs( Diclofenac et al., See a list of pricks from pain) are indicated in the presence of hyperthermia and development of defeat of the musculoskeletal system.
When a process is localized in one or another body, a specialist is consulted and appropriate corrective therapy is administered.
People suffering from SLE should avoid being in direct sunlight. Open areas of the skin should be lubricated with a protective cream from UV rays.
Immunosuppressive therapy with own stem cells is very effective, especially in severe cases. In most cases, autoimmune aggression stops and the patient's condition stabilizes.
Of great importance is the observance of a healthy lifestyle, the rejection of bad habits, feasible physical exercise, rational nutrition and psychological comfort.
Prognosis and prevention of
It should be noted that complete recovery from SLE can not be achieved.
Prognosis for life with adequate and timely treatment is favorable. About 90% of patients survive 5 years or more after the onset of the disease. The prognosis is unfavorable at the early onset of the disease, high activity of the process, development of lupus nephritis, infection. The prognosis for life is unfavorable for the development of SLE in males.
Due to the unexplained etiology of primary prevention of SLE there is no. To prevent exacerbations, direct sunlight should be avoided and the skin should be protected as much as possible( clothing, sunscreen, etc.).
Prevention of exacerbations of SLE in children is the organization of home education, prevention of infections and strengthening of immunity. Vaccination can be carried out only in the period of absolute remission. The introduction of gamma globulin is possible only in the presence of absolute indications.