Systemic lupus erythematosus: Symptoms, Causes, photos of women and men

Systemic lupus erythematosus - autoimmune pathology, at which the loss of blood vessels and connective tissue, and as a result - of the human skin. The disease is systemic in nature, ie, violation occurs in several systems of the body, exerting a negative influence on him as a whole and in the individual organs in particular, including the immune system.

Exposure of women's disease is several times higher than that of men, due to the peculiarities of the female body structure. The most critical age for the development of systemic lupus erythematosus (SLE) is the period of sexual maturation, during pregnancy and after an interval, until the body passes a stage recovery.

In addition, a separate category for the occurrence of disease is considered to be the children's age in the period of 8 years, but this is not determining a parameter because it is not excluded a congenital form of the disease and its manifestation in the early stages of life.

What is this disease?

Systemic lupus erythematosus (SLE, Libman-Sacks disease) (lat. lupus erythematodes, Eng. systemic lupus erythematosus) - a diffuse connective tissue disease characterized by systemic Immune lesions of connective tissue and its derivatives, with the defeat of the microcirculatory blood vessels channel.

Systemic autoimmune disease in which damage DNA human antibody produced by the immune system healthy cells, mainly damaged connective tissue with a vascular component mandatory presence. Disease was named due to its characteristic features - a rash on the nose and cheeks (the affected portion is shaped like a butterfly) which, as considered in the Middle Ages, resembles space wolf bites.

Story

The name of the lupus received from the Latin word "lupus" - wolf, "eritematozus" - red. This name was given because of the similarity of the signs of skin with injuries after being bitten by a hungry wolf.

The history of the disease lupus got its start in 1828. It happened after the French dermatologist Biett first described skin symptoms. Much later, after 45 years dermatologist Kaposhi noticed that some diseased skin, along with the signs of the disease are the internal organs.

In 1890. It was discovered by the English physician Osler, that systemic lupus erythematosus can occur without cutaneous manifestations. Description of the phenomenon LE- (LE) cells - a discovery of fragments of blood cells, in 1948. It allowed the identification of patients.

In 1954. They were detected in the blood of sick certain proteins - antibodies acting against its own cells. This finding has been used in the development of sensitive tests for the diagnosis of systemic lupus erythematosus.

Causes of

The causes of the disease not completely elucidated. Established only putative factors that contribute to the pathological changes.

Genetic mutations - identified a group of genes associated with specific disorders of immunity and susceptibility to systemic lupus erythematosus. They are responsible for the apoptotic process (the body get rid of the dangerous cells). In case of delay of potential pest damage occurs to healthy cells and tissues. Another way - the disorganization of the management process of the immune defense. The reaction of phagocytes becomes excessively strong, it does not end with the destruction of foreign agents for "foreign" to make their own cells.

1. Age - the maximum systemic lupus erythematosus sick people from 15 to 45 years, but there are cases occurred in children and the elderly.
2. Heredity - there are cases of family illness, probably transferred from the older generations. However, the risk of the patient's birth remains low.
3. Race - American studies have shown that black people sick 3 times more often white, and this reason is more pronounced in the indigenous Indians, natives of Mexico, Asian women, Spanish women.
4. Paul - known among female patients is 10 times more than men, so scientists are trying to establish a connection with sexual hormones.

Among the external pathogenic factors is most intense solar radiation. Hobby tan can provoke genetic changes. There is an opinion that is more common in systemic lupus people professionally dependent on activity sun, cold, harsh environmental temperature fluctuations (sailors, fishermen, agricultural workers, builders).

A significant portion of the patients clinical signs of systemic lupus appear during hormonal changes in the background of pregnancy, menopause, hormonal contraceptives, in a period of intense sexual maturation.

Also, the disease is associated with past infection, but to prove the role and impact of any pathogen (are purposeful work on the role of viruses) is not yet possible. Attempts to identify the relationship with immunodeficiency syndrome or set of communicable diseases until unsuccessful.

Pathogenesis

How is systemic lupus erythematosus in a healthy person at first sight? Under the influence of certain factors and decreased function of the immune system in the body fails at which begins to produce antibodies against 'native' cells. Ie, tissues and organs begin to be perceived by the body as foreign objects and runs a self-destruct program.

Such a reaction of the organism is pathogenic in nature, causing the development of inflammation and inhibition of normal cells in many ways. Most often, changes are subject to the blood vessels and connective tissue. The pathological process leads to disruption of skin integrity, changes in its appearance and reduction of blood flow in the lesion. With the progression of the disease affects the internal organs and systems of the whole organism.

Classification

Depending on the nature of the injury and course of the disease is classified into several types:

1. Lupus erythematosus, caused by taking certain medications. It leads to the appearance of symptoms of SLE, which may spontaneously disappear after cessation of treatment. Drug capable lead to the development of SLE, are preparations for treating hypotension (arteriolar vasodilators), antiarrhythmics, anticonvulsants facilities.
2. Systemic lupus erythematosus. The disease tends to rapid progression with a lesion of any organ or system of the body. It occurs with fever, malaise, headaches, a rash on the face and body, as well as the different nature pain anywhere on the body. The most characteristic of migraine, arthralgia, pain in the kidneys.
3. Neonatal lupus. It occurs in newborns, often combined with heart defects, severe impairment of the immune and circulatory systems, abnormal liver development. The disease is extremely rare; conservative treatment measures can effectively reduce the symptoms of neonatal lupus.
4. Discoid lupus. The most common form of the disease - the centrifugal erythema Biett, the main manifestation of which are the skin symptoms: a red rash, thickening of the epidermis, inflamed plaque in transforming scarring. In some cases, the disease results in damage to the mucous membranes of the mouth and nose. A variation is deep discoid lupus sarcoma-Irganga, characterized relapsing course and deep lesions of the skin. A special feature of the current forms of the disease are symptoms of arthritis, as well as reducing the human health.

Symptoms of Lupus

As a systemic disease, systemic lupus erythematosus is characterized by the following symptoms:

• chronic fatigue syndrome;
• swelling and tenderness of the joints and muscle pain;
• unexplained fever;
• chest pain when breathing deeply;
• increased hair loss;
• red skin rash on the face or discoloration of the skin;
• sensitivity to the sun;
• edema, swelling of the feet, the eyes;
• lymphadenopathy;
• bluish or whitening of fingers, legs, while staying in the cold or at the time of stress (Raynaud syndrome).

Some people experience headaches, seizures, dizziness, depression.

New symptoms can appear years later and after diagnosis. In some patients suffer from one system of the body (joints and skin, organs of hematopoiesis), in other cases symptoms can affect many organs and carry multiorgan character. The very severity and depth of the lesion body systems all different. Often the affected muscles and joints, thus causing arthritis and myalgia (muscle pain). Skin rashes are similarities between patients.

If the patient has multiple organ manifestations, the following pathological changes:

• kidney inflammation (lupus nephritis);
• inflammation of the blood vessels (vasculitis);
• pneumonia: pleurisy, pneumonia;
• Heart disease: coronary vasculitis, myocarditis or endocarditis, pericarditis;
• blood disorders: leukopenia, anemia, thrombocytopenia, the risk of blood clots;
• brain damage or central nervous system, and it provokes: psychosis (change in behavior), a headache, dizziness, paralysis, impaired memory, problems with vision, convulsions.

What is lupus, photos

The photo below shows how the disease manifests itself in humans.

The manifestation of symptoms of the autoimmune disease may vary considerably in different patients. However, common sites of localization of the lesions tend to become skin, joints (especially hands and fingers), heart, lungs and bronchi and digestive organs, hair and nails, which become more brittle and prone to hair loss, as well as the brain and nervous system.

Stage of the disease

Depending on the severity of the disease symptoms of systemic lupus erythematosus has several current steps:

1. The acute stage - at this stage of development of lupus is progressing rapidly, the general condition of the patient deteriorating, he complains about the constant fatigue, fever up to 39-40 degrees, fever, pain and aching muscles. The clinical picture is developing rapidly, already 1 month disease covers all the organs and tissues of the body. The prognosis of the acute form of lupus is not comforting, and often the patient's life expectancy is less than 2 years;
2. Subacute stage - rate of disease progression and severity of clinical symptoms not the same as in the acute stage of the disease and by the time until onset of symptoms may take more than 1 of the year. At this stage, the disease is often replaced by periods of relapse and remission resistant, generally favorable prognosis of the patient and depends upon the adequacy of the prescribed treatment;
3. The chronic form - the form of the disease has a sluggish flow, clinical symptoms are mild, the internal organs almost overwhelmed and the body as a whole is functioning normally. Despite the relatively mild course of lupus, the disease can not be cured at this stage, the only thing you can do - is to alleviate the severity of symptoms with medication at the time of exacerbation.

complications of SLE

The main complications that triggers the SCR:

1) Heart Disease:

• pericarditis - inflammation of the heart bag;
• hardening of the coronary arteries that feed the heart due to the accumulation of thrombotic clots (atherosclerosis);
• endocarditis (infection of the damaged heart valve) due to compaction of the heart valves, accumulation of blood clots. Often carried transplant valves;
• myocarditis (inflammation of the heart muscle), causing severe arrhythmia, heart muscle disease.

2) The renal disease (nephritis, nephrosis) developed in 25% of patients with SLE. The first symptoms - swelling in the legs, the presence of protein in the urine, and blood. Extremely dangerous for the life of kidney failure to operate normally. Treatment involves the use of powerful drugs against SLE, dialysis, kidney transplant.

3) Diseases of the blood, life-threatening.

• reduction in red blood cells (oxygen feeding cells), leukocytes (suppressing infection and inflammation), platelets (promote clotting);
• Hemolytic anemia caused by lack of red blood cells or platelets;
• pathological changes of hematopoiesis.

4) Diseases of the lung (30%), pleurisy, an inflammation of the chest muscles, joints and ligaments. The development of acute lupus (inflammation of lung tissue). Pulmonary Embolism - arterial occlusion emboli (blood clots) due to the increased blood viscosity.

Diagnostics

The assumption of the presence of lupus can be made on the basis of red foci of inflammation on the skin. External signs erythematosus may change over time, so for them it is difficult to make an accurate diagnosis. Requires the use of complex additional tests:

• common blood and urine tests;
• determining the level of liver enzymes;
• Analysis for Antinuclear body (ANA);
• chest X-ray;
• echocardiography;
• biopsy.

differential diagnosis

Chronic lupus erythematosus differentiate from lichen planus, leukoplakia and tuberculosis lupus, early rheumatoid arthritis, Sjogren's syndrome (see. dry mouth, dry eye syndrome, photophobia). In lesions vermilion border SLE chronic differentiate from abrasive prekantseroznogo Manganotti cheilitis and actinic cheilitis.

Since the defeat of the internal organs are always similar to the stream with a variety of infectious processes, SLE differentiate from Lyme disease, syphilis, mononucleosis (glandular fever in children: symptoms) HIV infection.

Treatment of systemic lupus erythematosus

Treatment should be as suitable for a particular patient.

Hospitalization is required in the following cases:

• with persistent fever with no apparent cause;
• in the event of life-threatening conditions: rapidly progressive renal failure, acute pneumonitis or pulmonary hemorrhage.
• the appearance of neurological complications.
• with a significant reduction in the number of platelets, red blood cells or blood lymphocytes.
• when the exacerbation of SLE can not be treated on an outpatient basis.

For the treatment of systemic lupus erythematosus in exacerbation widely used hormonal drug (prednisolone) and cytostatics (cyclophosphamide) in a specific pattern. nonsteroidal antiinflammatory drugs (diclofenac) assigned when lesion bodies musculoskeletal system as well as with increasing temperature.

For the adequate treatment of the disease of an organ need expert advice in this area.

food rules

Hazardous and harmful products in lupus:

• large amounts of sugar;
• all fried, greasy, salted, smoked, canned;
• products for which there is an allergic reaction;
• sweet soda, energy and alcoholic drinks;
• in the presence of kidney problems contraindicated food containing potassium;
• canned sausages factory preparation;
• shoplifter mayonnaise, ketchup, sauces, dressings;
• pastry cream, condensed milk, with artificial fillers (factory jam, jam);
• fast food and foods with unnatural fillers, dyes, rippers, amplifiers taste and smell;
• products containing cholesterol (buns, bread, red meat, dairy products with a high fat content, sauces, dressings and soups based preparations are taken cream);
• products having a long shelf life (meaning those foods that spoil quickly, but thanks to various chemical additives in the composition, can be stored for a long time - here, as an example, include dairy products per annum Shelf).

In using these products can accelerate the progression of the disease, which can lead to death. These are the maximum impact. And, as a minimum, the sleeping stage of lupus go into active, which is why all the symptoms worsen and feel much worse.

life expectancy

Survival at 10 years after diagnosis of systemic lupus erythematosus - 80% in 20 years - 60%. Major causes of death: lupus nephritis, neuro-lupus, intercurrent infection. There are cases of survival of 25-30 years.

In general, the quality and duration of life in systemic lupus erythematosus is dependent on several factors:

1. The patient's age: the younger the patient - the higher the activity of the autoimmune process and aggressive disease occurs, due to the greater reactivity of immunity at a young age (more autoimmune antibodies destroy its own tissue).
2. Timeliness, regularity and adequacy of treatment: chronic administration of glucocorticoid hormones and other drugs can be achieve a long remission period, reducing the risk of complications, and as a result - improved quality of life and its duration. Moreover, it is important to begin treatment before the onset of complications.
3. Variant of the disease: acute course runs extremely adverse and severe, life-threatening complications can occur in a couple of years. And with a chronic course, and this is 90% of the SCR, it is possible to live a full life until old age (if you follow all the advice of a doctor and a rheumatologist physician).
4. Adherence greatly improves the prognosis of the disease. For this must be constantly observed by a doctor, to adhere to its recommendations in a timely manner to address to doctors with the appearance of any symptoms exacerbation of disease, avoid contact with sunlight, limit water treatments, a healthy lifestyle and to comply with other rules of prevention exacerbations.

If you put the diagnosis of lupus, it does not mean that life is over. Try to defeat the disease, maybe not literally. Yes, probably, you will be somewhat limited. But millions of people and more serious diseases live a bright, full of life experiences! So can you.

prevention

The aim of prevention - to prevent the development of relapse, long-term to maintain the patient in a state of stable remission. Prevention of lupus is based on an integrated approach:

1. Regular dispensary examination and consultation with a rheumatologist.
2. Supplementation strictly prescribed dose and at specified time intervals.
3. Adherence to work and leisure.
4. Restful sleep, for at least 8 hours a day.
5. Diet restriction of salt and a sufficient amount of protein.
6. Hardening, walking, gymnastics.
7. Application gormonosoderzhaschih ointments (e.g. Advantan) in skin lesions.
8. Use sunscreens (creams).