Hemorrhagic vasculitis is a disease from a group of primary systemic vasculitides, affecting for the most part young people, manifested by arthralgia, a common microthrombosis, palpable purpura, a violation of the functions of the gastrointestinal tract.
Characterized by a progressive course with frequent relapses and in some cases development of chronic renal failure.
Pathology has been studied for a long time, since the beginning of the twentieth century, its in-depth study has been conducted, but many statements remain controversial until now, data on the etiology, pathogenesis and treatment of the disease are contradictory. Similarly, there is no generally accepted classification, so the topic is quite difficult to cover.
Causes of hemorrhagic vasculitis
The trigger mechanism of hemorrhagic vasculitis is acute and chronic infection. Its pathomorphosis is also influenced by drug therapy, exposure to physical and chemical agents, and adverse environmental conditions. In addition, to provoke the development of the disease can:
Most of this autoimmune disease affects children aged 4 to 12 years. It is they who often manifest the first symptoms of hemorrhagic vasculitis.
The pathological process begins when the body develops immune complexes, resulting in increased permeability of small blood vessels, and plasma with red blood cells partially falls into nearby tissues.
For hemorrhagic vasculitis, or purple Shenlaine - Genoch, as it is also called, benign course is typical with full recovery within 2-3 weeks. There are several forms of the disease, so the clinic manifests a complex of symptoms.
Forms of hemorrhagic purpura:
1) Simple or dermal. Diagnosed most often and refers to the main criteria of the disease. It shows a characteristic rash that slightly rises above the surface of the skin. Individual elements of the dumping can merge with each other and form solid areas. At first, these are reddish spots, but they quickly take the form of hemorrhages that do not disappear when pressed. The rash is localized mainly in the distal parts of the extremities( feet) with subsequent spread to the thighs and buttocks, often symmetrically concentrating in the region of the joints. It may also appear on the abdomen, back and upper limbs, but not often. In single severe cases, necrosis areas appear, covered with crusts, leaving behind scars in case of infection. But this is quite a rare phenomenon, therefore, after about two weeks, the hemorrhages disappear, gradually turning pale and changing its color. In case of frequent relapses, skin pigmentation may persist.
2) Articular form. Basically, it takes place in adults with involvement of large joints of the lower extremities in the process, less often - ulnar and wrist bands. The skin above these sites is hyperemic, edematous, covered with a rash. Fever in the inflamed joints can be combined with painful sensations in the muscles. General intoxication of the body is also characteristic. Symptoms take place within a week without a trace.
3) Abdominal form. It is expressed by strong spastic pains in the abdominal cavity, caused by hemorrhages in the intestinal wall, nausea, fever, vomiting in combination with hemorrhagic skin rashes. Often accompanied by a moderate gastrointestinal hemorrhage, blood in the stool. In cases of heavy bleeding, development of collapse and posthemorrhagic anemia is not excluded. Perhaps the development of peritonitis, necrosis and intestinal perforation, which requires prompt intervention.
4) Renal form. Clinical signs are very diverse. In some cases, the appearance of acute or chronic glomerulonephritis is common on the background of skin rashes. Do not exclude the development of nephrotic syndrome with an increase in body temperature. If there is no treatment for this form of vasculitis, renal failure may develop. But in many cases, the outlook is favorable.
5) Mixed form. This includes cases in which a disorder of the nervous system is diagnosed with the development of encephalopathy, lungs, genital organs( swelling of the scrotum in boys).
6) Lightning-fast form. It differs by symmetrical massive hemorrhages, merging with cyanotic discharge on hands, face, feet, buttocks, tissue necrosis. The course is extremely difficult, accompanied by a fever and can end with a lethal end due to the development of gangrene, coma or shock.
Treatment of hemorrhagic vasculitis
Treatment measures begin with the elimination of the effect of the factor that caused the disease, if possible. The therapeutic tactics of treating hemorrhagic vasculitis include:
the appointment of direct anticoagulants( heparin);
use of antibiotics( for the treatment of chronic infection);
vitamins;
glucocorticosteroids;
immunosuppressive agents( rarely);
cytostatics( rarely, in especially severe cases);
preparations of systemic enzyme therapy;
plasmapheresis.
Severe and often recurrent forms of hemorrhagic vasculitis are treated with stem cells, among which properties have the ability to strengthen immunity, replace damaged cells and lay new vessels instead of damaged ones. This therapeutic approach is very promising, it is often called the method of the future.
In addition to drug treatment of hemorrhagic vasculitis, diet and bed rest are also shown during the acute period of the disease. According to the indications, climatotherapy is recommended. After the treatment is mandatory there is a statement for dispensary registration and follow-up for two years.
To diagnose hemorrhagic vasculitis, special criteria have been developed, each of which has a clear definition. Their four and the presence of two or more criteria in the patient makes it possible to make a diagnosis.
Signs include palpable purpura, abdominal pain, age under 20 years, and the presence of granulocytes in biopsy. It is possible to suspect the disease with an increase in the density of lgA in blood serum and with an increase in the titer of ACL-O.Other diagnostic methods include:
general blood test, urine;
endoscopy studies;
kidney ultrasound;
Eq.
Prevention of hemorrhagic vasculitis
After hospital treatment for the prevention of recurrence it is necessary:
adhere to a hypoallergenic diet that excludes the use of chocolate, eggs, citrus fruits, nuts;
to refuse from vaccinations and samples with bacterial antigens;
to exclude physiotherapy;
use antibacterial drugs only in exceptional cases;
timely treat infectious diseases;
is regularly screened for intestinal worm infestations;
avoid supercooling and overheating;
visit a doctor regularly;
nullify the physical and mental overload;
carry out constant monitoring of urinalysis;
avoid contact with chemicals.
Recovery occurs in approximately 60% of patients, that is, the outcome of the disease can be called favorable, but provided timely treatment. In recent years there has been a more severe course of hemorrhagic vasculitis and an increase in the number of patients, due to urbanization, its domestic and professional shortcomings.
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