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 one's own immune system perceives the cells of the human body as foreign. As a result, she unfolds an immune response against them and provokes organ damage. Since the elements of the immune system are present in any part of the body, then almost all organs can be affected in case of lupus. According to statistics, in about 90% of cases the disease develops in the female. In the vast majority of cases, the first symptoms of the disease manifest themselves at the age of 15 to 25 years.
Many people are interested in the origin of the name of this disease, and what does the wolves have to do with it? History goes back to the Middle Ages, when in Europe it was still possible to come across a wild wolf and seriously suffer from such a meeting. This was especially true for coachmen, who often had to go on duty at any time of the day and in any weather. When attacking them, the wolf strove 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 the lupus "butterfly" - a lesion of the skin in the area of the cheekbones and nose.
In addition to systemic lupus erythematosus, two other processes are distinguished: discoid and drug lupus erythematosus. It is not necessary to identify these concepts, because pathologies differ from each other in both clinical manifestations and the likelihood of developing 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 they can not make a decisive step forward.
- Based on the data obtained during the collection of anamnesis, the doctors managed to establish that people who spend a lot of time in the cold or fever get sick more often than others. 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, the cause of the disease is not. This is just a substratum, which is subsequently layered by other factors. Nevertheless, it is proved that the relatives of people who have encountered this problem, the risk of developing the disease is an order of magnitude higher.
- Some authors claim that lupus is a kind of systemic response of the body's immune system to frequent irritations. Irritants are microorganisms, parasites of the gastrointestinal tract, fungal infections, viruses. If human immunity is weakened and constantly exposed to negative effects, then the probability of developing a perverse reaction to its cells and tissues increases.
- The impact of some aggressive chemical compounds (also almost always the patient is confronted with them in their workplace).
Some factors can cause a worsening of a person's condition with systemic lupus erythematosus or provoke another her aggravation:
- Smoking - very badly affects the condition of the vessels of the microcirculatory bed, which already suffer from SLE.
- Reception of a woman with drugs that contain sex hormones in large doses.
- The drug can not only cause the development of drug lupus, but also cause exacerbation of SLE (remember, these are different diseases).
Pathogenesis of the disease
Many patients can not understand doctors who tell them that their own immune system suddenly began to attack its master. After all, from the school desk even people far from medicine know that immunity is the protector of the body.
Initially, in the human body, the mechanism that regulates the function fails. As already mentioned above, the causes and mechanism of this particular link are not established, and doctors still need only ascertain this fact. As a result, some fractions of lymphocytes (this 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 different tissues, mainly the walls of small vessels. Already at the attachment site, protein molecules provoke the release of enzymes from the cells, which are normally contained in special microscopic capsules. Aggressive enzymes manifest themselves and damage the normal tissue of the body, causing a variety of symptoms with which the patient seeks help from a doctor.
Symptoms of systemic lupus erythematosus
As you can see from the previous section, the provokers of the disease through the body are carried with the blood flow. This suggests that you can expect to defeat almost any organ.
At first, people do not even suspect that they developed systemic lupus because it manifests itself as follows:
- An increase in body temperature that a person can not associate with any causes;
- Pain in the muscles and fatigue during work;
- Headache and general weakness.
It is clear that such manifestations are typical for almost any disease and that individually they do not represent anything of value to doctors. However, the disease has many more symptoms, which for convenience are divided into groups depending on which organ or organ system is affected.
Manifestations from the skin
- The classic symptom of the disease is a characteristic "lupus butterfly". It is a reddening of the skin and the appearance on it of a rash in the area of cheeks, nose, nose. Despite the fact that the skin is affected in 65% of patients with SLE, this all known symptom is observed only in 30-50% of patients. Often, women claim that this redness in them is associated with excitement or overheating in the sun.
- Rashes can also be found on the trunk and arms.
- A few times a person observes hair loss and nesting alopecia.
- The appearance of sores in the mouth, vagina, nose.
- On the legs and hands in the most severe cases, the skin suffers so much that trophic ulcers appear.
- Derived 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
With lupus, connective tissue suffers most of the others, which is relatively large in the area of the joints.
- Most patients with SLE show pain in the joints. In this case, the small joints on the hands and wrists are more often and more severely affected.
- The inflammation of symmetrical joints - polyarthritis - develops less often.
- In contrast to rheumatoid arthritis, which is very similar to joint damage in systemic lupus erythematosus, there is no destruction of bone tissue.
- Every fifth patient with joint damage develops his deformity. The bad thing is that this change in shape is permanent and can be eliminated only through surgical intervention.
- In men, very often the disease is manifested by inflammation of the sacroiliac joint. In this case, pain appears in the region of the sacrum and coccyx (slightly above the buttocks). Symptom can bother a man constantly, and can manifest after physical exertion in the form of discomfort and feelings of heaviness.
Manifestations of SLE from the hematopoietic system
- One of the most specific and characteristic manifestations of lupus is the appearance in the blood of LE cells (they are sometimes called lupus). These are leukocytes, within which laboratory assistants at high magnification detect the nuclei of other blood cells. Such a phenomenon is the result of false recognition of one's own cells as dangerous and alien. Leukocytes receive a signal to destroy them, destroy them and absorb them.
- At half of patients at clinical research of a blood anemia, a thrombocytopenia, a leukopenia are found out. Not always this is a consequence of the progression of the disease - often this effect is noted as a result of drug therapy of the disease.
Manifestations of the heart and large blood vessels
- At some part of patients at inspection doctors detect pericarditis, endocarditis and myocarditis.
- During the examination, doctors do not detect an infectious agent that could trigger the development of an inflammatory heart disease.
- With the progression of the disease, in most cases the 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 thickening of the glomerulus membrane occurs, fibrin is deposited, hyaline thrombi are formed. In the absence of adequate treatment, the patient can develop a persistent decline in kidney function. The probability of kidney damage is higher for acute and subacute disease. In the case of chronic SLE flow, lupus nephritis develops much less frequently.
- Hematuria or proteinuria, which is not accompanied by pain and does not bother a person. Often, this is the only manifestation of lupus from the urinary system. Since currently diagnosing SLE is timely and effective treatment begins, acute renal failure develops only in 5% of cases.
Neurological and mental abnormalities
Progression of the disease can provoke such formidable abnormalities on the part of the central nervous system, such as encephalopathy, convulsions , impaired sensibility, cerebrovascular disease. The bad news is that all the 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 a person has 4 or more criteria, regardless of how long they appeared, a diagnosis is made - systemic lupus erythematosus. These criteria are as follows:
- "Lupus butterfly" is a rash on the cheekbones.
- Discoid rashes.
- Increased sensitivity of skin to exposure to sunlight - photosensitivity. A person may have a rash or a mild redness.
- Sores on the epithelium of the mouth.
- Inflammatory lesion of two or more peripheral joints, without involvement of bone tissue in the process.
- Inflammation of serous membranes - pleurisy or pericarditis.
- Excretion in urine per day more than 0.5 g protein or cylindruria.
- Convulsions and psychosis, other neurological disorders.
- Hemolytic anemia, a decrease in blood levels of leukocytes and platelets.
- Detection of antibodies to native DNA and other immunological abnormalities.
- Increase the titer ANF.
Treatment of systemic lupus erythematosus
The patient should initially be adjusted to the fact that the disease can not be cured in a few days or simply to perform an operation. So far, the diagnosis is exhibited for life, but you can not despair either. Correctly selected treatment allows you to avoid exacerbation and lead a fairly full life.
- Preparations with glucocorticoids - it is their patients that are taken regularly throughout their life. Initially, the dose is set relatively large - in order to eliminate the exacerbation and all manifestations. After the doctor follows the patient's condition and slowly reduces the amount of medication taken. The minimal dose significantly reduces the likelihood of the patient developing side effects from treatment with hormones, which are many.
- Cytostatic preparations.
- TNF-α inhibitors are a group of drugs that block the action of pro-inflammatory enzymes in the body and can remove symptoms of the disease.
- Extracorporeal detoxification - the removal of pathological blood cells and immune complexes from the body by very sensitive filtration.
- Pulse-therapy - 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.
- Non-steroidal anti-inflammatory drugs.
- Treatment of diseases, which provokes lupus ñ nephritis, arthritis, etc.
It is very important to monitor the state of the kidneys, since it is lupus nephritis which is the most common cause of death of patients with systemic lupus erythematosus.