Systemic lupus erythematosus - results from genetic disorders caused by immune regulation, manifested an uncontrolled production of autoantibodies to the cells and their components with the development of immunocomplex inflammation in many organs and systems.
There are acute, subacute and chronic variants of the course. Syndrome is a leading joint-muscle (80-90%), skin lesions (85%), febrile reaction. Frequently affects the heart: pericarditis, endocarditis, myocarditis. Pleuropulmonary changes occur in 40-80% of children. Approximately half of patients have neurological symptoms: seizures, motor and sensory disorders, mental disorders. Typically kidney-type diffuse glomerulonephritis with nephrotic component. Joint and muscle syndrome is characterized by lesions of the joints, tendons, tendon sheaths of the muscles. Muscle pain can be very powerful.
On palpation of the muscles are painful, sometimes sealed. Arthritis occurs acutely, subacutely, often chronically. Acute rheumatic arthritis reminiscent of "volatility" of defeat, like rheumatoid subacute, with a characteristic deformation of the joints, muscle atrophy, stiffness in the mornings and control functions. Manifestations of the disease can be kept for a long time, but then undergo a complete back development.
Joint and muscle syndrome occurs in waves. His aggravation is combined with the defeat of most serous membranes and skin, the development of severe vistseritov. Unusual polymorphisms and various localization characteristic rashes. Most often they manifest erythema and edema, infiltration, hyperkeratosis with a tendency to the formation of necrosis with subsequent scarring and pigmentation. Erythematous rash often located not only on the face, but also on the exposed parts of the body, primarily in the upper third of the chest and back.
Pathognomonic of systemic lupus erythematosus "butterfly" on the face, in the bridge of the nose and cheeks occurs in about 1 \ 3 of the patients. In the acute stage of the disease in children is often observed cheilitis manifesting swelling and hyperemia of the red border of lips with the formation of crusts and etozy. Of great importance is the estimation of the degree of activity of the process.
Providing 4 degrees of activity of systemic lupus erythematosus:
Low activity (I degree) - overall condition is usually not disturbed, laboratory values changed little, the signs of internal organ involvement are defined only for complex surveys.
With moderate activity (II degree) the condition of patients is usually mild, low-grade fever. Symptoms of internal organ involvement and laboratory findings are expressed in moderation.
The high activity of (III degree) the condition of patients difficult, there is a high fever. Observed striking changes in the internal organs, skin, musculoskeletal system, marked changes in laboratory parameters.
When lupus Stroke patient's condition is very serious or critical condition. Due to the involvement in the pathological process in many organs and systems and the development of severe functional impairment krizovogo body. In recent years, the 10-year survival of patients with childhood is 85-93% and the duration of the disease is often more than 20-30 years or more.