Systemic lupus erythematosus - Causes, symptoms and treatment. MF.

Systemic lupus erythematosus( SLE) is a disease in which inflammatory reactions in various organs and tissues develop due to a malfunction of the immune system.

The disease occurs with periods of exacerbation and remission, the occurrence of which is difficult to predict. In the final, systemic lupus erythematosus leads to the formation of a deficiency of one or another organ, or several organs.

Women suffer from systemic lupus erythematosus 10 times more often than men. The disease is most common at the age of 15-25 years. Most often, the disease manifests during puberty, during pregnancy and in the postpartum period.

Causes of systemic lupus erythematosus

The cause of systemic lupus erythematosus is not known. The indirect influence of a number of factors of the external and internal environment, such as heredity, viral and bacterial infection, hormonal changes, environmental factors, is discussed.

• genetic predisposition plays a role in the onset of the disease. It is proved that if one of the twins is diagnosed with lupus, the risk that the second can get sick increases by 2 times. Opponents of this theory indicate that the gene responsible for the development of the disease has not yet been found. In addition, in children, one of whose parents has systemic lupus erythematosus, only 5% develop the disease.

• The frequent detection of Epstein-Barr virus in patients with systemic lupus erythematosus in favor of the viral and bacterial theory. In addition, it has been proven that the DNA of some bacteria can stimulate the synthesis of antinuclear autoantibodies.

• Women with SLE in the blood often have an increase in hormones such as estrogen and prolactin. Often, the disease manifests during pregnancy or after childbirth. All this speaks in favor of the hormonal theory of the development of the disease.

• It is known that ultraviolet rays in a number of predisposed individuals can trigger the production of autoantibodies by the skin cells, which can lead to the onset or aggravation of an already existing disease.

Unfortunately, none of the theories does not reliably explain the cause of the disease. Therefore, currently systemic lupus erythematosus is considered a polyethiologic disease.

Symptoms of systemic lupus erythematosus

Under the influence of one or more of the above factors, under the conditions of malfunctioning of the immune system, the DNA of various cells is "exposed".Such cells are perceived by the body as foreign( antigens), and to protect them from the production of special antibody proteins, specific to these cells. When antibodies and antigens interact, immune complexes are formed, which are fixed in various organs. These complexes lead to the development of immune inflammation and cell damage. Cells of connective tissue are especially affected. Given the wide spread of connective tissue in the body, with systemic lupus erythematosus, almost all organs and tissues of the body are involved in the pathological process. Immune complexes, fixing on the wall of blood vessels, can provoke thrombosis. Circulating antibodies due to their toxic effects lead to the development of anemia and thrombocytopenia.

Systemic lupus erythematosus refers to chronic diseases that occur with periods of exacerbation and remission. Depending on the initial manifestations, the following variants of the course of the disease are distinguished:

• acute course of - manifested by fever, weakness, fatigue, joint pain. Very often patients indicate the date of onset of the disease. Within 1-2 months a detailed clinical picture of the defeat of vital organs is formed. With rapidly progressing course after 1-2 years, patients usually die.
• subacute SCR flow - the first symptoms of the disease are not so pronounced. From the manifestation to the defeat of organs takes an average of 1-1.5 years.
• chronic course of . For many years, one or more symptoms have occurred. In chronic course, periods of exacerbation are rare, without disrupting the work of vital organs. Often, minimal doses of drugs are required to treat the disease.

As a rule, the initial manifestations of the disease are nonspecific, when taking anti-inflammatory drugs or spontaneously they leave without a trace. Often the first sign of the disease is the appearance on the face of reddening in the form of butterfly wings, which also eventually disappears. The period of remission, depending on the type of flow, can be quite long. Then, under the influence of some predisposing factor( prolonged exposure to the sun, pregnancy) there is an exacerbation of the disease, which is also followed by a phase of remission. Over time, symptoms of organ damage are added to nonspecific manifestations. For a detailed clinical picture, the following organs are damaged.

1. Skin, nails and hair .Skin infection is one of the most common symptoms of the disease. Often the symptoms appear or intensify after prolonged exposure to the sun, frost, with psychoemotional shock. A characteristic sign of SLE is the appearance in the cheek and nose area of ​​reddening of the skin in the form of butterfly wings.

Erythema type butterfly

As in the open areas of the skin( face, upper limbs, "decollete" area), there are different shapes and sizes of reddening of the skin, prone to peripheral growth-centrifugal erythema Bietta. Discoid lupus erythematosus is characterized by the appearance of redness on the skin, which is then replaced by an inflammatory edema, then the skin in this area becomes denser, and in the fin is formed the sites of atrophy with scarring.

Discoid lupus erythematosus

Foci of discoid lupus erythematosus can occur in various areas, in this case they speak of dissemination of the process. Another bright manifestation of skin lesions is capillaritis-reddening and edema and numerous small-point hemorrhages on the pads of the fingers, palms, soles. The defeat of hair in systemic lupus erythematosus is manifested by baldness. The changes in the structure of the nails, up to the atrophy of the peri-oral cusp, occur during the period of exacerbation of the disease.

2. Mucous membranes of the .Usually affects the mucous membrane of the mouth and nose. The pathological process is characterized by the appearance of redness, the formation of erosions of the mucous membrane( enanthema), as well as small ulcers of the oral cavity( aphthous stomatitis).

Aphthous stomatitis

When cracks, erosions and ulcers of the red border of the lips appear, lupus-cheilitis is diagnosed.

3. Musculoskeletal system .The defeat of joints occurs in 90% of patients with SLE.

Arthritis of the joint of the second finger in the SLE

The pathological process involves small joints, usually the fingers of the hand. The defeat is symmetrical, patients are concerned about pain and stiffness. Deformation of the joints is rare. Aseptic( without an inflammatory component) necrosis of bones are common. The head of the femur and the knee joint are affected. In the clinic symptoms of functional insufficiency of the lower limb predominate. When involving the pathological process of the ligamentous apparatus, unstable contractures develop, in severe cases dislocations and subluxations.

4. Respiratory system .The most common infection of the lungs. Pleurisy( accumulation of fluid in the pleural cavity), as a rule, bilateral, accompanied by pain in the chest and shortness of breath. Acute lupus pneumonitis and pulmonary hemorrhages refer to life-threatening conditions and lead to the development of respiratory distress syndrome without treatment.

5. Cardiovascular system .The most common is Libman-Sachs endocarditis with frequent mitral valve involvement. In this case, as a result of inflammation, the fissure of valve flaps and the formation of heart disease by the type of stenosis occur. With pericardial sheets of the pericardium thicken, and between them may appear fluid. Myocarditis is manifested by pain in the chest area, an increase in the heart. With SLE, small and medium-sized vessels are often affected, including coronary arteries and cerebral arteries. Therefore, stroke, ischemic heart disease is the main cause of death in patients with SLE.

6. Kidneys .In patients with SLE at high activity of the process, lupus nephritis is formed.

7. The nervous system .Depending on the affected area, patients with SLE show a wide range of neurological symptoms, ranging from migraine headaches to transient ischemic attacks and strokes. In the period of high activity of the process, epileptic seizures, chorea, cerebral ataxia may occur. Peripheral neuropathy occurs in 20% of cases. The most dramatic manifestation of it is optic neuritis with loss of vision.

Diagnosis of systemic lupus erythematosus

Diagnosis of SLE is considered established with 4 or more of 11 criteria( American Rheumatology Association, 1982).

Erythema type butterfly	Fixed erythema( flat or sublime) on the cheekbones, with a tendency to spread to the nasolabial folds.
Discoid rash	Elevated erythematous foci with tightly seated scales, over time, skin atrophy and cicatricial changes.
Photosensitivity	Appears or strengthens the rash after exposure to the sun.
Ulcers of the oral mucosa and / or nasopharynx	Usually painless.
Arthritis	Appearance of edema and soreness of at least two joints without deformity.
Serositis	Pleurisy or pericarditis.
Kidney damage	One of the following manifestations: a periodic increase in the protein in the urine to 0.5 g / day or the determination of the cylinders in the urine.
CNS lesion	One of the following manifestations: convulsive seizures or psychoses unrelated to other causes.
Hematologic disorders	One of the following manifestations: hemolytic anemia, lymphopenia, or thrombocytopenia, not related to other causes.
Immune disorders	Detection of LE cells, or antibodies to serum cDNA, or antibodies to Smith-antigen, or a false positive Wasserman reaction, persisting for 6 months without detection of pale treponema.
Antinuclear antibodies	Increase in the titer of antinuclear antibodies, not related to other causes.

Immunological tests play an important role in the diagnosis of SLE.Absence of an antinuclear factor in the serum of blood makes the diagnosis of SLE in doubt. Based on laboratory data, the degree of activity of the disease is determined.

As the degree of activity increases, the risk of damage to new organs and systems increases, as well as the aggravation of existing diseases.

Classification of hard currency according to the degree of activity.

Treatment of systemic lupus erythematosus

Treatment should be as appropriate for the patient as possible. Hospitalization is necessary in the following cases:

• with a persistent rise in temperature for no apparent reason;
• in the event of life-threatening conditions: rapid-progressive renal failure, acute pneumonitis, or pulmonary hemorrhage.
• with the appearance of neurological complications.
• with a significant decrease in the number of platelets, red blood cells or blood lymphocytes.
• in the case when exacerbation of SLE can not be cured in outpatient settings.

For the treatment of systemic lupus erythematosus during the exacerbation, hormonal drugs( prednisolone) and cytostatics( cyclophosphamide) are widely used according to a certain scheme. When lesions of the organs of the musculoskeletal system, as well as with increasing temperature, non-steroidal anti-inflammatory drugs( diclofenac) are prescribed.

For an adequate treatment of a disease of an organ, a specialist consultation in this area is necessary.

Prognosis for life in SLE with timely and correct treatment is favorable. The five-year survival rate of such patients is about 90%.But, nevertheless, the mortality of patients with SLE exceeds the general population three times. The factors of unfavorable prognosis are early onset of the disease, male sex, development of lupus nephritis, high activity of the process, attachment of infection.

Doctor therapist Sirotkina EV