Systemic lupus erythematosus
- Causes of systemic lupus erythematosus
- Pathogenesis of the disease
- Symptoms of systemic lupus erythematosus
- Diagnosis of systemic lupus erythematosus
- Treatment of systemic lupus erythematosus
Systemic lupus erythematosus (SLE) is a serious disease in which its own immune system perceives the cells of the human body as foreign. As a result, it deploys an immune response against them and provokes organ damage. Since elements of the immune system are present in any part of the body, almost all organs can be affected by lupus. According to statistics, in about 90% of cases, the disease develops in women. In most cases, the first symptoms of the disease appear between the ages of 15 and 25 years.
Many are interested in the origin of the name of the disease, and how do wolves have to do with it? The story goes back to the distant Middle Ages, when in Europe it was still possible to encounter a wild wolf and seriously suffer at such a meeting. This was especially true for the driver, who often had to go on duty at any time of day and in any weather. When attacking them, the wolf tried to bite into the unprotected part of the body, which turned out to be the face (nose and cheeks, to be more specific). Later, the main symptoms of the disease will be listed, and one of them is lupus "butterfly" - skin lesions in the cheekbones and nose.
In addition to systemic lupus erythematosus, there are two more processes: discoid and drug lupus erythematosus. These concepts should not be identified, since pathologies differ from each other both in their clinical manifestations and in the likelihood of serious complications.
Causes of systemic lupus erythematosus
Until now, doctors have not been able to establish the exact cause of the development of such a complex disease. Around the world, dozens of laboratories are looking for an answer to this question, but cannot decisively move forward.
- Based on the data obtained during the collection of anamnesis, doctors managed to establish that people who spend much time in cold or heat get sick more often than others from lupus erythematosus. In most cases, this is due to their professional activities. In favor of ultraviolet radiation (it contains sunlight) says the fact that very often the disease in patients is exacerbated in the summer.
- Hereditary predisposition, in fact, is not the cause of the disease. This is just a substrate, on which other factors subsequently accumulate. Nevertheless, it has been proven that relatives of people who have experienced this problem have a higher risk of developing the disease.
- Some authors claim that lupus is a kind of systemic response of the body's immune system to frequent irritations. Microorganisms, parasites of the gastrointestinal tract, fungal infections, viruses act as irritants. If a person’s immunity is weakened and is constantly exposed to a negative effect, then the probability of a perverted reaction to his cells and tissues increases.
- Exposure to some aggressive chemical compounds (almost always the patient also encounters them in his workplace).
Some factors can cause a deterioration of a person with systemic lupus erythematosus or provoke another exacerbation of it:
- Smoking - a very bad effect on the state of the blood vessels of the microcirculatory bed, which already suffer from SLE.
- Reception by the woman of the preparations containing sex hormones in high doses.
- A drug can not only cause the development of drug lupus, but also cause an aggravation of SLE (remember, these are different diseases).
Pathogenesis of the disease
Many patients cannot understand the doctors who tell them that their own immune system suddenly began to attack their host. After all, even far from medicine people know from school that the immune system is the protector of the body.
Initially, in the human body, a mechanism that performs a regulatory function fails. As mentioned above, the causes and mechanism of this particular link have not been established, and the doctors can only ascertain this fact. As a result, some fractions of lymphocytes (this is a blood cell) show excessive activity and provoke the formation of large protein molecules - immune complexes.
Further, these same complexes spread throughout the body and are deposited in various tissues, mainly the walls of small vessels. Already at the site of attachment, protein molecules provoke the release of enzymes from cells, which normally are enclosed in special microscopic capsules. Aggressive enzymes exert their action and damage the normal tissue of the body, causing many symptoms with which the patient seeks help from a doctor.
Symptoms of systemic lupus erythematosus
As you can see from the previous section, provocateurs of the disease are spread throughout the body with blood flow. This suggests that you can expect damage to virtually any organ.
At first, people do not even suspect that they have developed systemic lupus due to the fact that it manifests itself as follows:
- a rise in body temperature that a person cannot relate to for any reason;
- muscle pain and fatigue when doing work;
- headache and general weakness.
It is clear that such manifestations are characteristic of almost any disease and that, in isolation, they do not represent anything of value to doctors. However, the disease still has many symptoms, which, for convenience, are divided into groups depending on which organ or organ system is affected.
Manifestations of the skin
- The classic symptom of the disease is the characteristic "lupus butterfly". It is a reddening of the skin and the appearance of a rash on it in the area of the cheeks, nose, nose. Despite the fact that the skin is affected in 65% of patients with SLE, this well-known symptom is observed in only 30-50% of patients. Often, women claim that this redness is associated with excitement or overheating in the sun.
- Rashes can also be found on the body and arms.
- Somewhat less often a person notes hair loss and nesting alopecia.
- The appearance of ulcers in the mouth, vagina, nose.
- On the legs and arms in the most severe cases, the skin suffers so much that trophic ulcers appear.
- Derivatives of the skin - hair and nails can also be damaged as a result of the progression of lupus. Nails become brittle, and hair falls out.
Manifestations of the musculoskeletal system
In lupus, connective tissue suffers most, which is present in relatively large numbers in the area of the joints.
- Most patients with SLE report pain in the joints. In this case, the small joints on the hands and wrists are more often and more strongly affected.
- The inflammation of symmetrical joints - polyarthritis - develops somewhat less frequently.
- Unlike rheumatoid arthritis, which is very similar to joint damage in systemic lupus erythematosus, bone destruction does not occur.
- Every fifth patient with joint damage develops its deformity. The bad thing is that this change in shape is permanent and can only be eliminated with the help of surgical intervention.
- In men, very often the disease is manifested by inflammation of the sacroiliac joint. At the same time there is pain in the sacrum and coccyx (slightly above the buttocks). A symptom can bother a man constantly, and can manifest itself after physical exertion in the form of discomfort and a feeling of heaviness.
Manifestations of SLE from the hematopoietic system
- One of the most specific and characteristic manifestations of lupus is the appearance of LE cells in the blood (they are sometimes called lupus). These are leukocytes, inside of which laboratory workers at high magnification detect the nuclei of other blood cells. This phenomenon is a consequence of the false recognition of their own cells as dangerous and alien. Leukocytes receive a signal to destroy them, destroy to pieces and absorb into themselves.
- In a half of the patients, clinical examination of the blood reveals anemia, thrombocytopenia, and leukopenia. This is not always a consequence of the progression of the disease - often this effect is observed as a result of the medical therapy of the disease.
Manifestations of the heart and large blood vessels
- At some part of the patients during the examination, doctors find pericarditis, endocarditis and myocarditis.
- During the examination, doctors do not detect an infectious agent that could provoke the development of inflammatory heart disease.
- With the progression of the disease, in most cases, mitral or tricuspid valves are affected.
- Systemic lupus erythematosus, like many systemic diseases, increases the likelihood of developing atherosclerosis.
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. The probability of kidney damage is higher in acute and subacute course of the disease. In the case of chronic SLE, lupus nephritis develops much less frequently.
- 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.
Neurological and Mental Abnormalities
The progression of the disease can provoke such formidable disorders of the central nervous system, such as encephalopathy, convulsions , impaired sensitivity, cerebrovascular disease. The bad thing is that all changes are quite persistent and difficult to correct.
Diagnosis of systemic lupus erythematosus
Based on all available data, doctors identified the most common symptoms of lupus erythematosus. Some of them are more common, others are less common. If 4 or more criteria are identified in a person, regardless of how long they appeared, a diagnosis of systemic lupus erythematosus is made. These criteria are as follows:
- "Lupus butterfly" - a rash on the cheekbones.
- Discoid eruption.
- Increased sensitivity of the skin to sunlight - photosensitization. A person may have a rash or just a pronounced redness.
- Ulcers on the epithelium of the mouth.
- Inflammatory lesion of two or more peripheral joints, without involvement of bone tissue in the process.
- Inflammation of the serous membranes - pleurisy or pericarditis.
- Excretion with urine per day more than 0.5 g of protein or cylindruria.
- Spasms and psychosis, other neurological disorders.
- Hemolytic anemia, a decrease in blood levels of leukocytes and platelets.
- Detection of antibodies to its own DNA and other immunological abnormalities.
- Increasing the titer of ANF.
Treatment of systemic lupus erythematosus
The patient must initially tune in to the fact that the disease cannot be cured in a few days, or simply to have an operation. So far, the diagnosis is set for life, but you also cannot despair. Properly chosen treatment allows you to avoid exacerbation and lead a fairly full life.
- Drugs with glucocorticoids - it is their patients take regularly throughout their lives. Initially, the dose is set relatively large - in order to eliminate aggravation and all manifestations. After the doctor monitors the patient's condition and slowly reduces the amount of the drug taken. The minimum dose significantly reduces the likelihood of the patient developing side effects from hormone treatment, which are quite numerous.
- Cytotoxic drugs.
- TNF-α inhibitors - a group of drugs that block the action of proinflammatory enzymes in the body and can relieve symptoms of the disease.
- Extracorporeal detoxification - removal of abnormal blood cells and immune complexes from the body through very sensitive filtration.
- Pulse therapy is the use of impressive doses of cytostatics or hormones, which allows you to quickly get rid of the main symptoms of the disease. Naturally, this course takes a short time.
- Nonsteroidal anti-inflammatory drugs.
- Treatment of diseases that provokes lupus - nephritis, arthritis, etc.
It is very important to monitor the condition of the kidneys, since it is lupus nephritis that is the most common cause of death of patients with systemic lupus erythematosus.