Hemorrhagic vasculitis (Shenleyna-Genokh's Disease)

Description:

Gemorragicheesky vasculitis (synonyms: Shyonleyn's disease — Genokh, a rheumatic purpura, an allergic purpura) — the most widespread disease from group of system vasculites.

Symptoms of the Hemorrhagic vasculitis (Shenleyna-Genokh's Diseases):

The symptom complex of a hemorrhagic vasculitis consists of characteristic skin damages, a joint syndrome, abdominal syndrome, damage of kidneys. Skin manifestations meet most often and are characterized by emergence on extremities, buttocks, is more rare on a trunk of polymorphic symmetric papular and hemorrhagic, is more rare than the urtikarny, bubble and erythematic rashes rising over the surface of skin and not disappearing when pressing. The explicit inflammatory component is characteristic of hemorrhagic elements and it is long the pigmentation remaining after regression of skin manifestations. In hard cases the course of a disease is complicated by development of the central necroses and formation of ulcers.

The joint syndrome arises after skin rashes and remains within several days in the form of the flying large joint pain recuring at a new wave of rashes.

The abdominal syndrome in some cases advances emergence skin hemorrhages and dominates in a clinical picture at 30% of patients. The main symptom - a constant or a colicy pain in a stomach, sometimes big intensity, connected with hemorrhage in a wall of a gut and a mesentery. Hemorrhage in a wall of a gut can lead to its hemorrhagic treatment and be followed by a hematemesis, a melena, fresh blood in Calais. The period of pain alternates with bezbolevy intervals that helps to differentiate acute surgical pathology and abdominal manifestations of a hemorrhagic vasculitis. The abdominal syndrome is short and regresses in most cases in 2-3 days.

The renal syndrome is found in 25-30% of patients and proceeds as an acute or chronic glomerulonephritis with a micro and gross hematuria, a proteinuria, a cylindruria. A fate of patients the nephrotic syndrome develops. From all manifestations of a hemorrhagic vasculitis damage of kidneys remains most long, at a part of patients leading to uraemia.

Reasons of the Hemorrhagic vasculitis (Shenleyna-Genokh's Diseases):

  The aseptic inflammation of walls of microvessels, the multiple microthrombogenesis striking vessels of skin and internals is the cornerstone of a disease. The main reason causing this disease is circulation in blood of cell-bound immune complexes and the activated components of system of a complement. In a healthy organism cell-bound immune complexes eliminirutsya from an organism by special cells. Excessive accumulation of the circulating cell-bound immune complexes in the conditions of dominance of antigens or at insufficient antibody formation leads to adjournment them on an endothelium of a microcirculator bed with secondary activation of proteins of system of a complement on a classical way and secondary change of a vascular wall.

Treatment of the Hemorrhagic vasculitis (Shenleyna-Genokh's Diseases):

Planned therapy. Patients with Shenleyna-Genokh's disease need hospitalization and a bed rest. It is necessary to exclude chocolate, a citrus, berries and juice from a diet. Purpose of antihistaminic drugs is inefficient. In hard cases Prednisolonum in a dose to 60 mg a day in the form of courses for 3-5 days with obligatory background administration of the heparin preventing hypercoagulation and development of the IDCS is applied. The dose of heparin is selected individually, according to koagulogramma indicators. Criterion of efficiency of a geparinoterapiya is lengthening of AChTV twice in comparison with control.
At the expressed joint syndrome use of Voltarenum or indometacin in sredneterapevtichesky doses is effective.
Against the background of a heavy infection the hemorrhagic syndrome is connected with development of the IDCS. In this case transfusion of freshly frozen donor plasma is effective.
The most effective method of treatment of a teleangiectasia is the cryolysis or local laser therapy.
At detection of cryoglobulin the course of a medical plasma exchange with substitution by albumine and saline solutions is necessary.
Prevention of a recurrence. In prevention of a hemorrhagic vasculitis an important role is played by the prevention of aggravations of focal infection, refusal of prescription of antibiotics and other medicines without the bases, sufficient on that. Inoculations and tests with bacterial antigens are contraindicated to patients (in t. h tuberkulinovy). At patients with preferential damage of kidneys constant control of analyses of urine is necessary.