System vasculitis

Description:

System vasculites - group of diseases which cornerstone generalized defeat of vessels with an inflammation and a necrosis of a vascular wall is that leads to ischemic changes of bodies and fabrics.
Mean generalized defeat of vessels of immune genesis by primary vasculitis as an independent disease.
Secondary vasculites develop as reaction to an infection, an invasion helminths, at influence of chemical factors, radiation, at tumors. Also the vasculites arising at other general diseases belong to secondary.
The vasculites connected with cell-bound immune complexes:

    * Hemorrhagic vasculitis.
    * Vasculites at a system lupus erythematosus and a pseudorheumatism.
    * Bekhchet's disease.
    * Cryoglobulinemic vaskul

The vasculites connected with organospetsifichesky antibodies:

    * A disease of Kawasaki (with products of antibodies to an endothelium).

The vasculites connected with anti-neurophilic cytoplasmatic antibodies:

    * Wegener's granulomatosis.
    * Microscopic polyarteritis.
    * Allergic (eosinophilic) granulematozny angiitis (Charga-Stross's syndrome).
    * Classical nodular polyarteritis.

Symptoms of the System vasculitis:

Clinically vasculites are characterized by systemacity, defeat of several or many bodies, fabrics and vascular pools. The clinical picture depends first of all on a nosological form and caliber of the struck vessels, however a number of symptoms свойствен to the majority of system vasculites: fever; skin manifestations in the form of the marble drawing, cyanosis of extremities, rashes of various character (papular, knotty, hemorrhagic), trophic disturbances; muscular pains, spasms  at rest; polyarthralgias, mono - and polyarthritis; polyneuritis signs. Consecutive damage of various internals is characteristic. Involvement in process of coronal arteries of heart is shown by pains like stenocardia, developing in some cases of a myocardial infarction, damage of a muscle of heart with development of myocarditis and the subsequent myocardiosclerosis. Changes of vessels of lungs lead to development of pneumonia, a bronkhospastichesky syndrome, a heart attack of a lung, a pneumosclerosis, formation of pulmonary hypertensia and a pulmonary heart. Digestive tract cankers, heart attacks of an intestinal wall, bleeding, increase in a liver and spleen, the phenomenon of acute and chronic pancreatitis, a mesenteric thrombosis meet the subsequent gangrene of a wall of a gut. Damage of kidneys is characterized by development of nephrite with changes in urine in the form of a proteinuria, a hamaturia, increase in the ABP, a nephrotic syndrome. Involvement in pathological process of a nervous system leads to development of a polyneuritis, disturbances of cerebral circulation. At defeat of vessels of extremities the alternating lameness, Reynaud's syndrome and the migrating phlebitis are noted.
At most of patients at a blood analysis the disturbances testimonial of existence of an inflammation come to light: increase in SOE, level of fibrinogen, the maintenance of A2 globulins, S-reactive protein, but specific changes at system vasculites are absent.

Reasons of the System vasculitis:

The etiology of the majority of primary system vasculites is unknown. The role of the following etiological factors is supposed:

  1. Viral infection (viruses of hepatitis B and C, B19 parvovirus, cytomegalovirus, HIV, virus Epstein-Burra, etc.)
  2. Bacterial infection (streptococci, iyersiniya, chlamydias, salmonellas, etc. bacteria). The most frequent form of the vasculites connected with an infection is the vasculitis of small vessels of skin, and also a hemorrhagic vasculitis, a vasculitis of arteries of small and average caliber. Especially many data are available about a role of viruses of hepatitis B and C in development of a nodular periarteritis.
  3. Hypersensitivity to various medicines (to antibiotics, anti-tubercular, anti-virus, etc.). Hypersensitivity to medicines can provoke development of a necrotizing vasculitis.
  4. Hypersensitivity to components of tobacco causes development of an obliterating thromboangitis.
  5. Genetic factors play a large role in development of system vasculites. Immunogenetic markers of system vasculites are certain antigens of HLA system.

The role of genetic factors in development of giant-cell arteritis (communication with HLA DR4) is convincingly proved, to Takayasu's disease (communication with HLA Bw52, Dw12, DR2 and DQw1.

Treatment of the System vasculitis:

It is possible to allocate several main stages in treatment of system vasculites.

  1. Bystry suppression of an immune response in a disease debut - remission induction.
  2. Long not less than 0,5-2 years a maintenance therapy immunosuppressants in the doses sufficient for achievement of clinical and laboratory remission of a disease. Bystry stopping of an immune response at exacerbations of a disease.
  3. Achievement of permanent, full remission of a vasculitis, definition of a damage rate of bodies or systems of an organism for the purpose of their correction, holding rehabilitation actions.
Now glucocorticoids for treatment of vasculites are widely used. They are appointed practically with all their forms.
For treatment of system vasculites cytotoxic drugs of three main classes are used: alkylating agents (cyclophosphamide), purine analogs (Azathioprinum), antagonists of folic acid (methotrexate). The last in low doses have no explicit cytotoxic activity.
Recently apply the immunoglobulin entered intravenously to treatment of vasculites. This drug is used in clinical practice for therapy of autoimmune diseases within already more than 15 years.
It is also widely applied плазмофорез.