Description lupus erythematosus
Lupus erythematosus is characterized by a systemic disease, which means that it relates to multiple organs (systems) in the human body compared to diseases which are linked to one functional unit – e.g. ulcers of the stomach and duodenum for the digestive tract, tonsillitis on tonsils or cystitis to the excretory tract.
Properly is therefore called systemic lupus erythematosus(erythema = erythema = rash) and most often affects the skin, joints, heart, blood vessels, kidneys, lungs and brain.
Not expressed in the symptoms of these organs, lupus is quitevariable, so that one patient dominate lung problems, has the second and the third kidney disease are treated for cardiology. For most of these patients (80-90%), however, in common that havecutaneous manifestations, and joint pain.
There is also a separate form affecting the skin only (discoid lupus erythematosus), which is due to the fact that there is a system, of course, a minor, on the other hand, in systemic lupus may go later.
Lupus occurs most frequently among women (90%) of childbearing age, it is between 20- 40 years of age. These women is not recommended pregnancy, as is the risk of heart disease causes the fetus while the mother deterioration lupus itself.
It is a chronic disease that occurs in phases of activity and remissions – transient recovery. Active stage is characterized by high fever and other symptoms of organ.
Fortunately, the incidence of this disease so common, according to epidemiological studies ill for 1-10 people sick per 100 000 population. However, this is different in different populations – the European average is 20 to 30 patients per 100,000 inhabitants, while it among the population afrokaribského origin, the average of around 206 patients in the same population.
Prevention and risk factors in lupus erythematosus
Unfortunately, we do not know the prevention of systemic lupus erytematodu. It has to do with the fact that we do not know clear cause of the disease. We only know that it is an autoimmunedisease that is characterized by excessive activation of the immune system (particularly B-lymphocytes and related antibodies) that is directed against the healthy cells of the body.
Under normal circumstances the immune system is in equilibrium and it is necessary to eliminate any infection (e.g., bacterial or viral), which enter into the body. In the case of lupus, however, the white blood cells do not eliminate virus infected cells or bacteria, but the body’s own cells.Exagerated reaction further deplete some organs accumulation of antibodies (in particular regarding the kidneys).
Why to excessive activation of the immune system occurs it is not entirely clear. Genetic studies suggest a role of heredity, since autoantibodies were detected (or antibodies against its own cells) in healthy family members of patients with lupus.
Great attention is paid to the effect of infection as the triggering factor disease, discusses the herpes viruses (cold sores), Epstein-Barr virus (mononucleosis) and others.
It demonstrates also the effect of female sex hormones, especially estrogen and prolactin as to significantly affect the quality of immune responses.
It follows that we can not influence risk factors, some are not sufficiently specific and some are utterly uncontrollable – genetic predisposition, gender and race.
Lupus erythematosus may be zapříčeněn and certain medications (used to arrhythmia – procainamide and medications for high blood pressure – hydralazine and methyldopa). After the withdrawal of these drugs wears off, however, so it really is not about lupus in the true sense of the word.
Signs and symptoms of lupus erythematosus
Systemic lupus erythematosus is a connective tissue disease that is somehow damaged autoimmune process. The binder includes a fibrous and cartilaginous tissue and because it occurs in almost all organs, is explained by the fact that it violated several organs. Additionally, the binder is contained in the arteries and perhaps every body is permeated with blood vessels.
Depending on which organs are more or less affected, the patient present the symptoms. Speeches are therefore very varied and for this reason, the disease has earned designation magna simulatrix (simulating high – imitating various other diseases).
Patients often complain of intolerance to sunlight, hair loss, cramps, painful swollen lymph nodes, fatigue, weight loss, excessive sweating and pain in joints and muscles, which are coupled withmotýlovitým reddening of the skin on the face of the first manifestation of the disease.
Conspicuous may also be fever over 38 ° with chills (although some patients may have a raised temperature), giving the impression of acute infection and sometimes leads patients to infectious department.
For mental disorders and convulsions that can mimic epilepsy, the proportion of patients receiving the psychiatry and neurology and detection of lupus and takes longer.
For cutaneous manifestations may not be the only pea redness in the face, but other rashes, darkening or scarring and ulcers on the skin of the head, neck, chest, arms, upper torso and limbs, and the mucous membranes may indicate lupus.
The rash is usually to places not covered by clothing and exposed to the sun. Another skin symptom is the increased sensitivity of the fingertips rest (sometimes legs) to the cold and it shows whitening first fingers (downloading vessels), then gradually zmodráním and redness when vascular fingertips returns blood.
If you have any of the above symptoms, seek dermatologist and he certainly correctly determine whether the lupus or sends you to further examination.
Heart problems, has 30-50% of patients. It is an inflammation of the lining around the heart(pericarditis), which are probably the most common, or inflammation of the heart muscle(myocarditis), which show increased heart rate. The most severe heart disease is endocarditis.Patients with lupus also have a higher incidence of myocardial infarction.
Joint pain with systemic lupus have been known for ages. Usually relate to multiple joints, and must be distinguished from other joint diseases (e.g. from rheumatoid arthritis, which also has a systemic and chronic in nature, but as the joints concerned, patients suffering from their morning stiffness, which during the day enhances).
Pulmonary involvement is manifested gradually developing dyspnea (a man with bad breath), it can be a chronic inflammation of the lungs, less often acute. Most often, but it is a simple pleuritis,or inflammation of the membrane that surrounds the lung.
Kidneys are part lupus almost always, but only some patients (about 40%) have problems. Clinical entities, there is a wide range, depending on which part of the kidney is the most affected by immune reactions, collectively they are called glomerulonephritis.
If they are given individual speeches to the brain and can be very diverse and nonspecific. The patient may have altered behavior, impaired memory, learning and other basic functions. With more brain damage may develop dementia. At other times, the effect of brain stroke, seizures or visual disturbances.
Treatment of systemic lupus erythematosus
After the final diagnosis preceded mainly blood tests (in particular the circulating antibodies) and other auxiliary examination by the speeches in various organs, the doctor will start your treatment consisting depressed immune processes. It is the best of glucocorticoids that have besides anti-inflammatory effect at higher doses also immunosuppressive effect (thus dampening the immune system).
The disadvantage is that, in addition, have a variety of side effects.
Most used prednisone (Prednisone) in kidney, brain, lung and other symptoms, then prednisolone or methylprednisolone (Medrol). Necessary are also the long-term maintenance doses.
In patients with predominantly cutaneous form or where there is less disease activity, can be takenhydroxychloroquine (Plaquenil), which is otherwise used to treat malaria.
In the case of more serious complications are administered immunosuppressant drugs such asazathioprine (Azaprine, Imuran) or cyclophosphamide (Cyclophosphamide Orion, Endoxan) tablets, however, are sometimes necessary infusion. Even this treatment has its pitfalls, they can develop infections because of it, against which a weakened immune system can not defend, can also give formation of tumor growth and cyclophosphamide treatment could cause with bloody cystitis. As a result, today instead began using cyclosporine (Consupren, Equoral, Sandimmun Neoral), especially in renal impairment.
Besides medication, it is important to avoid moments that bring the disease into the active phase such as the sun (which appears or worsens rash), childbirth, stress, drugs or other intoxicants. When moving out therefore prefer to use creams with SPF. It is suitable Sleep and plenty of rest, at least at the time of disease activity.
Complications of lupus erythematosus
Every manifestation of the disease can result in the body for some of the complications. From the heart, it is primarily a bacterial inflammation inside the heart (ie. Bacterial endocarditis), and bleeding, inflammation of the pericardium, which fortunately is very rare. Lupus patients often have coronary heart disease (angina) and are thus at risk of myocardial infarction.
Preference is it associated with glucocorticoid therapy, which help the development of accelerated atherosclerosis. The vessels are deposited fats and thus the lumen narrows, so it can be closed vessel supplying the heart, and this is the basis of myocardial infarction.
For pulmonary complications may be an acute pneumonia with acute severe respiratory insufficiency that is very serious.
Kidney disease in lupus and in some cases lead to their failure and need dialysis. Other times may appear a dangerous infection.
Even cerebrovascular accident (stroke) is actually a complication since it can compromise the patient in a similar manner as myocardial infarction (obstruction of an artery).
Other complications arising from treatment with corticosteroids and other drugs that suppress the immune system. Patients have few red blood cells, platelets and white blood cells that are important in the defense against inflammations and although they have these patients relatively higher defense responses (over activity of the immune system) and are rather poorly directed (against cells own body), and treatment with immunosuppressants is suppresses just against common infections.
Other names: systemic lupus erythematosus, SLE, lupus, systemic rheumatic disease, connective tissue disease, magna simulatrix
