System lupus erythematosus

Description:

System lupus erythematosus - the autoimmune general disease of connecting fabric and vessels which is characterized by formation of a set of antibodies to own cells and their components and development of an immunocomplex inflammation with damage of many bodies and systems.

Symptoms of the System lupus erythematosus:

Damage of skin and mucous covers - the most frequent syndrome of a system lupus erythematosus. Only 10-15% of patients have no skin changes. Dubois (1976) specifies that there are 28 options of skin changes at this disease. The following is the most characteristic:

    * the isolated or merging erythematic spots of various form and size, edematous, delimited from healthy skin. Are most often observed on a face, a neck, a breast, in elbow, knee, ankle joints. Diagnostically high-significant the figure of a butterfly is especially characteristic (an arrangement of erythematic spots on a nose and cheeks). Quite often erythematic spots happen extremely bright, red (as after a sunblister), edematous;
    * at a chronic current of a system lupus erythematosus the erythematic centers are characterized by infiltration, a hyperkeratosis, a peeling and a cicatricial atrophy of skin;
    * a lyupus-cheilitis - the expressed reddening of lips with grayish scales, crusts, erosion, with the subsequent development of the centers of an atrophy on a red border of lips;
    * capillarites - in the field of small pillows of fingers, on palms, soles are available red edematous spots with teleangiectasias, a skin atrophy;
    * an enantema of a mucous membrane of an oral cavity - sites of an erythema with hemorrhages and erosion;
    * violent, knotty urtikarny, hemorrhagic rashes, mesh a livedo with skin ulcerations;
    * trophic disturbances - a xeroderma, a hair loss, fragility, fragility of nails;
    * on a mucous membrane of an oral cavity and a nose - the erosive, ulcer centers, whitish plaques, erythematic spots, perforation of a partition of a nose is possible;
    * at a subacute skin lupus erythematosus there are ring-shaped rashes with teleangiectasias, a depigmentation in the center. They are located on a face, a neck, a breast, extremities. Similar changes of skin are often observed at patients with HLA DR3 and B8

Defeat of bone and joint system is shown by the following signs:

    * one or several joint pains intensive and long;
    * symmetric polyarthritis with involvement of proximal interphalangeal joints of brushes, a spot - phalanx, carpal and metacarpal, knee joints;
    * the expressed morning constraint of the affected joints;
    * development of flexion contractures of fingers of hands owing to tendinites, a tendovaginitis;
    * formation of a revmatoidnopodobny brush due to changes in periartikulyarny fabrics; erosion of joint surfaces are uncharacteristic (can be only at 5% of patients);
    * development of aseptic necroses of a head of a femur, humeral and other bones is possible.
Damage of muscles is shown by mialgiya, the expressed muscular weakness, the polymiositis, similar to a dermatomyositis sometimes develops.

Damage of lungs is shown by the following signs:

    * dry or vypotny pleurisy with stethalgias and the expressed asthma at accumulation of a large amount of liquid; as a rule, bilateral pleurisy is observed;
    * the lupoid pneumonitis (a pulmonary vasculitis) is characterized by short wind, dry cough, sometimes a pneumorrhagia; at X-ray inspection of lungs discal atelectases in lower parts come to light, infiltrative shadows are sometimes visible. At the chronic course of a lyupus-pneumonitis defeat of intersticial tissue of lungs with strengthening of the pulmonary drawing is observed;
    * syndrome of pulmonary hypertensia;
    * the thromboembolism of a pulmonary artery is possible.

Defeat of cardiovascular system:

Development of a pancarditis is possible, but the pericardis, usually dry is most often observed, but the heavy exudative pericardis sometimes develops. At a high degree of activity the diffusion myocarditis which is complicated by a circulatory unefficiency is observed.

Defeat of an endocardium (Liebman-Saks's endocarditis) is observed at a pancarditis and leads to defeat of the valve device of heart. More often mitral insufficiency forms, is more rare - aortic incompetence. Heart diseases give at auscultation the corresponding noise (systolic noise in the field of a heart top at mitral insufficiency, diastolic noise over an aorta at aortic incompetence). Warty imposings on valves can be defined at an ekhokardioskopiya.

Vessels, preferential arteries of average and small caliber are surprised. There are messages on involvement in pathological process of a subclavial artery, coronary arteries that can lead to development of a myocardial infarction. Thrombophlebitises of superficial veins of a shoulder, the front surface of a thorax are rather often observed.

Gastrointestinal tract disease and liver:

Patients are disturbed by nausea, vomiting, lack of appetite. Damage of a gullet is shown by its dilatation, erosive changes of a mucous membrane. Quite often ulcerations of a mucous membrane of a stomach and a duodenum are found. Defeat of vessels of a mesentery leads to severe pains in a stomach, preferential around a navel (abdominal crisis), prelum abdominale muscle tension.

Damage of a liver is shown by clinic of lupoid hepatitis (increase in a liver, jaundice of various expressiveness, increase in content in blood of aminotransferases).

Damage of kidneys (lyupus-nephrite):

According to the WHO classification, distinguish the following morphological options of lyupus-nephrite:

I - lack of changes in a bioptata; II - mezangialny nephrite; III - a focal proliferative glomerulonephritis; IV - a diffusion proliferative glomerulonephritis; V - hymenoid glomerulonephritis; VI - a sclerosing glomerulonephritis.

Lupoid nephrite is shown by the following clinical forms (M. M. Ivanova, 1994):

    * quickly progressing lupoid nephrite (a heavy nephrotic syndrome, malignant arterial hypertension, bystry development of a renal failure);
    * a nephrotic form of a glomerulonephritis (unlike not lupoid nephrite, the proteinuria is less expressed, arterial hypertension and a hamaturia are more often observed, the hypercholesterolemia is less expressed);
    * active lupoid nephrite with the expressed uric syndrome (the proteinuria is more than 0.5 g/days, a microhematuria, a leukocyturia);
    * nephrite with the minimum uric syndrome - a proteinuria is less than 0.5 g/days, a microhematuria - single erythrocytes under review, a small leukocyturia, arterial pressure normal.

Defeat of a nervous system is observed almost at all patients and is caused by vasculites, thromboses, heart attacks and hemorrhages in various departments of a brain. In recent years the big part in defeat of a nervous system is assigned to antineyronalny antibodies which strike membranes of neurons.

Defeat of a nervous system can have the following clinical manifestations: headaches, mental disorders, a convulsive syndrome (as temporal epilepsy), dysfunction of cranial nerves, mononeuropathy, polyneuropathy, disturbance of cerebral circulation (owing to thromboses, a hemorrhage). The myelitis is seldom observed.

Reasons of the System lupus erythematosus:

The cause of illness is unknown. The etiological role of the following factors is supposed:

  1. A persistent viral infection (the RNA-containing and slow retroviruses). The proof is detection in blood of patients with a system lupus erythematosus of a set of antibodies to these viruses and detection by means of a submicroscopy of inclusions of a virus in endothelia, lymphocytes, in bioptata of kidneys and skin.
  2. Genetic factor. Increase in frequency of a system lupus erythematosus in families of patients with this disease is established. At a system lupus erythematosus more often than in population, HLA A1, B8, DR2, DR3 meet. It is supposed that the risk of a disease of a system lupus erythematosus is caused by four independent segregating genes.

Treatment of the System lupus erythematosus:

    * Glucocorticosteroids (Prednisolonum or other)
    * Cytostatic immunodepressants (Azathioprinum, Cyclophosphanum or other)
    * Blockers ФНО-α (Infliksimab, Adalimumab, Etanertsept).
    * Extracorporal detoxication (plasma exchange, hemosorption, krioplazmosorbtion)
    * Pulse therapy high doses of glucocorticosteroids and/or tsitostatik
    * Non-steroidal anti-inflammatory drugs
    * Symptomatic treatment.