Systemic lupus erythematosus is a chronic autoimmune disease characterized by damage to the connective tissue and blood vessels and, as a result, involvement of almost all organs and body systems in the pathological process.
In the development of systemic lupus erythematosus a certain role is played by hormonal disorders, in particular, an increase in the amount of estrogen. This explains the fact that the disease is more often recorded in young women and adolescent girls. According to some data, viral infections and chemical intoxication play an important role in the occurrence of pathology.
This disease relates to autoimmune diseases. Its essence lies in the fact that the immune system on some irritant begins to produce antibodies. They adversely affect healthy cells, as they affect their DNA structure. Thus, due to antibodies, there is a negative change in connective tissue and blood vessels.
What causes the development of systemic lupus erythematosus, and what kind of disease? The etiology of the disease is unknown. In the development of it suggest the role of viral infection, as well as genetic, endocrine and metabolic factors.
Lymphocytotoxic antibodies and antibodies to double-stranded RNA, which are markers of persistent viral infection, are found in patients and their relatives. In the endothelium of the capillaries of damaged tissues (kidneys, skin), virus-like inclusions are detected; on experimental models the virus is identified.
SLE occurs predominantly in young (20–30 years) women, but cases of the disease are not uncommon in adolescents and older people (more than 40–50 years). Among the cases, only 10% of men are noted, but the disease is more severe in them than in women. Insolation, drug intolerance, stress are often provoking factors; for women, childbirth or abortion.
The disease is classified according to the stages of the disease:
- Acute systemic lupus erythematosus. The most malignant form of the disease, differs continuously-progressive course, a sharp increase and a multiplicity of symptoms, resistance to therapy. According to this type of systemic lupus erythematosus often occurs in children.
- The subacute form is characterized by the frequency of exacerbations, however, with a lesser degree of severity of symptoms, rather than with the acute course of SLE. Organ damage develops during the first 12 months of the disease.
- The chronic form is characterized by a long-term manifestation of one or several symptoms. The combination of SLE with the antiphospholipid syndrome in the chronic form of the disease is especially characteristic.
Also during the disease there are three main stages:
- The minimum. There are minor headaches and joint pains, occasional fever, malaise, as well as initial skin symptoms of the disease.
- Moderate. Significant damage to the face and body, involvement of vessels, joints, internal organs into the pathological process.
- Pronounced. Observed complications from the internal organs, brain, circulatory system, musculoskeletal system.
Systemic lupus erythematosus is characterized by lupus crises, in which the disease activity is maximal. The duration of a crisis can be from one day to two weeks.
Symptoms of lupus erythematosus
In adults, systemic lupus erythematosus manifests itself with a large number of symptoms, which is caused by the damage of tissues of almost all organs and systems. In some cases, the manifestations of the disease are limited exclusively to skin symptoms, and then the disease is called discoid lupus erythematosus, but in most cases there are multiple lesions of the internal organs, and then they talk about the systemic nature of the disease.
In the initial stages of the disease, lupus erythematosus is characterized by a continuous course with periodic remissions, but almost always goes into a systemic form. Often marked erythematous dermatitis on the face like a butterfly - erythema on the cheeks, cheekbones and always on the back of the nose. Hypersensitivity to solar radiation appears - photodermatoses are usually rounded in shape, are multiple.
Joint damage occurs in 90% of patients with SLE. Small joints, as a rule, of fingers, are involved in the pathological process. The lesion is symmetrical, patients are worried about pain and stiffness. Deformity of the joints is rare. Aseptic (without inflammatory component) bone necrosis is common. The femoral head and knee joint are affected. The clinic is dominated by symptoms of functional insufficiency of the lower extremity. When the ligamentous apparatus is involved in the pathological process, non-permanent contractures develop, in severe cases, sprains and subluxations.
Common symptoms of SLE:
- Soreness and swelling of the joints, muscle pain;
- Unexplained fever;
- Chronic fatigue syndrome;
- A red rash on the face or a change in the color of the skin;
- Chest pain with deep breathing;
- Increased hair loss;
- Whitening or blueing of the skin of the fingers of the hands or feet in the cold or under stress (Raynaud's syndrome);
- Hypersensitivity to the sun;
- Swelling of the legs and / or around the eyes;
- Swollen lymph nodes.
Dermatological signs of the disease include:
- Classic rash on the nose and cheeks;
- Spots on the limbs, body;
- Brittle nails;
- Trophic ulcers.
- Redness and ulceration (ulceration) of the red border of the lips.
- Erosion (surface defects - "corrosive" mucous membrane) and ulcers on the mucous membrane of the oral cavity.
- Lupus cheilitis is a pronounced, dense swelling of the lips, with grayish scales tightly adjacent to each other.
The defeat of the cardiovascular system:
- Lupus myocarditis.
- Endocarditis Liebman-Sachs.
- Coronary artery disease and myocardial infarction.
With lesions of the nervous system, the most frequent manifestation is asthenic syndrome:
- Weakness, insomnia, irritability, depression, headaches.
With further progression may develop epileptic seizures, impaired memory and intelligence, psychosis. Some patients develop serous meningitis, optic neuritis, intracranial hypertension.
Nephrological manifestations of SLE:
- Lupus nephritis is an inflammatory disease of the kidneys, in which the glomerular membrane thickens, fibrin is deposited, and hyaline blood clots are formed. In the absence of adequate treatment, the patient may develop a persistent reduction in renal function.
- Hematuria or proteinuria, which is not accompanied by pain and does not bother the person. Often this is the only manifestation of lupus from the urinary system. Since currently SLE is diagnosed in a timely manner and effective treatment begins, acute renal failure develops only in 5% of cases.
- Erosive and ulcerative lesion - patients are worried about lack of appetite, nausea, vomiting, heartburn, pain in various parts of the abdomen.
- Intestinal infarction due to inflammation of the blood vessels supplying the intestines - a picture of "acute abdomen" develops with high-intensity pain, often localized around the navel and in the lower abdomen.
- Lupus hepatitis - jaundice, an increase in the size of the liver.
- Acute lupus pneumonitis.
- The defeat of the connective tissue of the lungs with the formation of multiple foci of necrosis.
- Pulmonary hypertension.
- Pulmonary embolism.
- Bronchitis and pneumonia.
Assuming lupus before visiting a doctor is almost impossible. Seek advice if you have an unusual rash, fever, joint pain, fatigue.
Systemic lupus erythematosus: photo in adults
What does systemic lupus erythematosus look like? We offer for viewing detailed photos.
If you suspect a systemic lupus erythematosus, the patient is referred for consultation by a rheumatologist and a dermatologist. To diagnose systemic lupus erythematosus, several systems of diagnostic signs have been developed.
Currently, the system developed by the American Rheumatic Association is preferred as more modern.
The system includes the following criteria:
- butterfly symptom:
- discoid rash;
- ulceration of mucous membranes;
- kidney damage - protein in the urine, cylinders in the urine;
- brain damage, convulsions, psychosis;
- increased skin sensitivity to light - the appearance of a rash after exposure to the sun;
- arthritis - lesion of two or more joints;
- reducing the number of red blood cells, white blood cells and platelets in the clinical blood test;
- detection of antinuclear antibodies in the blood (ANA).
- the appearance of specific antibodies in the blood: anti-DNA antibodies, anti-CM antibodies, false positive Wasserman, anticardiolipin antibodies, lupus anticoagulant, positive test for LE cells.
The main goal of treating systemic lupus erythematosus is the suppression of the autoimmune reaction of the body, which underlies all the symptoms. Patients are assigned different types of drugs.
Treatment of systemic lupus erythematosus
Unfortunately, a complete cure for lupus is impossible. Therefore, therapy is chosen so as to reduce the symptoms, to stop the inflammatory, as well as autoimmune processes.
Tactics of treatment of SLE is strictly individual and may change with the time of illness. Diagnosis and treatment of lupus are often the combined efforts of the patient and doctors, specialists of various specialties.
Current drugs for the treatment of lupus:
- Glucocorticosteroids (prednisone or others) are potent drugs that fight inflammation in lupus erythematosus.
- Cytostatic immunosuppressants (azathioprine, cyclophosphamide, etc.) - drugs that suppress the immune system can be very useful in lupus erythematosus and other autoimmune diseases.
- TNF-α blockers (Infliximab, Adalimumab, Etanercept).
- Extracorporeal detoxification (plasmapheresis, hemosorption, cryoplasma sorption).
- Pulse therapy with high doses of glucocorticosteroids and / or cytotoxic drugs.
- Nonsteroidal anti-inflammatory drugs - can be used to treat inflammation, swelling and pain caused by lupus.
- Symptomatic treatment.
If you have lupus, you can take a few steps to help yourself. Simple measures can make exacerbations less frequent, and also improve the quality of your life:
- Give up smoking.
- Practice regularly.
- Eat a healthy diet.
- Watch out for the sun.
- Adequate rest.
The prognosis for life with systemic lupus is unfavorable, but recent advances in medicine and the use of modern drugs give a chance for a prolongation of life. Already more than 70% of patients live more than 20 years after the initial manifestations of the disease.
At the same time, doctors warn that the course of the disease is individual, and if in one part of patients SLE develops slowly, in other cases, rapid development of the disease is possible. Another feature of systemic lupus erythematosus is the unpredictability of exacerbations that can occur suddenly and spontaneously, which threatens with grave consequences.