Hemorrhagic vasculitis is a pathology that is a type of immune vasculitis. This disease affects small blood vessels. It is characterized by an increase in the formation of immune complexes, an increase in permeability and a decrease in the elasticity of arterial and capillary walls.
The disease often occurs 10-20 days after suffering acute respiratory infections, tonsillitis, influenza or scarlet fever, which is why children are mainly at risk of this disease. Especially susceptible to pathology are children aged 3 to 7 years. By the way, boys get sick 2-3 times more often than girls. In the international classification of diseases of the 10th revision (ICD 10), hemorrhagic vasculitis was labeled D69.0, D89.1 - "Systemic hemorrhagic vascular lesions."
Causes of the disease
Why is there an ailment that is also called Shenlein-Genoch disease and capillarotoxicosis? The mechanism of development of vasculitis is quite simple: an aseptic inflammatory process occurs in the capillaries caused by the damaging effect of the immune complexes of antibodies. Shenlein-Genoch disease is manifested by hemorrhages, a malfunction in the processes of intravascular coagulation and a violation of blood circulation in small arteries.
The causes of hemorrhagic vasculitis in patients of any age are conditionally divided into several categories:
- complications after an illness (respiratory viral infection, chickenpox, herpes, scarlet fever) caused by pathogenic microflora and parasites;
- allergic reactions;
- hypothermia;
- vaccine intolerance;
- hereditary predisposition.
The main factor in the occurrence of hemorrhagic vasculitis is the formation of immune complexes consisting of antibodies. These formations are deposited on the inner surface of arterial walls, which cause microscopic damage to blood vessels and, as a result, provide conditions for the development of post-traumatic aseptic inflammation. Accordingly, with inflammation of the vascular tissue, its elasticity decreases markedly, as a result of which the walls become permeable, gaps appear. This state of arteries and capillaries leads to fibrin deposits and the formation of blood clots. The above explains the appearance of symptoms of vasculitis - bruising provoked by microtrombosis of blood vessels.
Varieties
Today, doctors know several forms of hemorrhagic vasculitis. Depending on the clinical picture, the ailment happens:
- skin;
- articular;
- abdominal, that is, developing in the abdominal cavity;
- renal;
- combined.
The disease can occur simultaneously in several forms. Among the most common combinations, cutaneous-articular vasculitis is most common. Specialists also call this form simple. Depending on the severity of the pathology, she is assigned the degree of small, medium and high activity. Another typology of hemorrhagic vasculitis is built according to the nature of the course of the disease. So, capillarotoxicosis can be:
- lightning fast (develop literally in a matter of days);
- acute (the duration of the disease usually does not exceed 30-40 days);
- protracted (leaking over 2 months);
- recurrent (symptoms of the disease are repeated several times over 3-4 years);
- chronic (the clinical picture remains unchanged for 1.5-2 years, remissions alternate with frequent or rare exacerbations).
The main signs of pathology
Speaking about the clinical manifestations of the described vascular pathology, it is worth noting that the same symptoms of hemorrhagic vasculitis are observed in adults and children. At the same time, the features of the course of the disease may vary depending on age. Mostly the disease is manifested by spotted rashes on the epidermis. They are called skin hemorrhagic rash.
In the articular form of vasculitis, patients complain of pain in the ankle joints. This symptom occurs in seven out of ten cases. With the abdominal form of hemorrhagic disease, its inevitable manifestation is a dull, aching pain in the abdomen.
In complicated hemorrhagic vasculitis, the vessels of internal organs, including the kidneys, lungs, and brain, are often affected in adults. The most favorable prognosis is given to patients in whom the disease proceeds only with superficial manifestations in the form of a hemorrhagic rash.
Stains on the skin with vasculitis
As already noted, with any form of this disease, bruising is observed on the skin. The defeat of the epidermis is the main manifestation of hemorrhagic vasculitis. It occurs in absolutely all patients, regardless of the manifestation of additional symptoms. Skin rashes can occur both at the very beginning, in the first stages of the disease, and after some time, as the pathology progresses. The most characteristic is the appearance of purpura - small-spotted hemorrhages, palpable palpation. Such a rash is symmetrical, consists of small (1 to 3 mm) nodules. First, rashes affect the feet and lower legs, but later spread throughout the limb. In addition to purpura, other elements (vesicles, redness with peeling, and in severe cases even necrotic areas of the upper layer of the epidermis) may appear.
Joint pain
Seventy percent of case histories show the severity of vasculitis symptoms such as joint pain. This symptom often appears along with rashes in the first weeks of the disease. Changes in the articular surfaces and internal departments may be accompanied by minor and short-term painful sensations, but a wider lesion is not excluded, in which not only large (ankle and knee) joints are affected, but also joints of the toes. They swell, a short-term change in their shape is also possible. However, with a normal course and timely therapy, the disease does not cause severe deformation of the articular surfaces, and on average discomfort does not bother for longer than 5 days.
Gastrointestinal tract
The severity of the course is characterized by hemorrhagic vasculitis in adults, which is accompanied by damage to the vessels of the gastrointestinal tract. Often the appearance of abdominal pain is ahead of the hemorrhagic rash. Unpleasant sensations occur when small capillaries rupture on the walls of the intestine, and therefore are acute cramping. Patients, describing the discomfort, note that it mainly occurs in the umbilical, right iliac and hypochondrium.
The pain syndrome with hemorrhagic vasculitis resembles the clinical picture caused by appendicitis, ulcer perforation or intestinal obstruction. The pain can last for 3-10 days. Often the disease occurs with nausea and vomiting, in which mucus and bloody impurities are present. By the way, blood in the feces with an abdominal form of vasculitis is a very common symptom. In severe cases, intestinal bleeding develops, resulting in a sharp drop in blood pressure and collapse.
How does the disease occur at different ages
Differences in the course of the disease in children and adults are in its development. In adults, the clinical picture is blurry. For this reason, at the initial stages, the diagnosis of hemorrhagic vasculitis is difficult, because the symptoms are not fully manifested. The patient's general well-being practically does not suffer.
In children, the pathology of small vessels has an acute onset. Vasculitis in children under 6 years of age develops rapidly, accompanied by fever, chills and weakness. Moreover, in half the clinical cases in patients of any age, an abdominal syndrome is observed, which is accompanied by dyspeptic disorders (nausea, vomiting, loose stools with streaks of blood). If the kidneys are involved in the pathological process, the presence of protein is detected in the urine tests of patients, hematuria is possible. With delayed treatment, kidney damage leads to the development of chronic diffuse glomerulonephritis with the subsequent formation of chronic renal failure.
Disease complications
To delay with the treatment of hemorrhagic vasculitis is not worth it. The likely complications of this disease include the development of:
- intestinal obstruction;
- pancreatitis
- perforation of ulcers of the stomach and intestines;
- peritonitis;
- moderate anemia;
- DIC syndrome with thrombocytopenia;
- thrombosis;
- subtotal or total heart attacks;
- neuritis;
- cerebral disorders.
Similar consequences of hemorrhagic vasculitis are possible only if you ignore the symptoms of the disease and delayed seeking medical help. That is why it is important to start therapy as soon as possible. After all, it is not a secret for anyone that it is much easier to overcome any disease in its initial stages. It is impossible to self-medicate and resort to uncontrolled taking of medicines or traditional medicine. Medications should be prescribed only by a qualified specialist after a comprehensive examination and diagnosis.
Diagnostic procedures
The treatment of hemorrhagic vasculitis is carried out by a rheumatologist. With a complicated course of the disease and damage to the internal organs, it will also require consultation of narrow-profile specialists - a dermatologist, gastroenterologist, neurologist, nephrologist, cardiologist and pulmonologist.
Suspecting hemorrhagic vasculitis in the patient, the doctor should conduct an examination, collect an anamnesis, prescribe laboratory and instrumental studies. Expert opinions will help to get an objective picture of the disease and begin appropriate therapy.
The main diagnostic methods for hemorrhagic vasculitis today are:
- coagulogram;
- analysis for the presence of immunoglobulins of classes A and G (the first indicator for vasculitis is increased, and the second is reduced), cryoglobulins and other antibodies;
- a study to detect circulating immune complexes;
- blood chemistry;
- general (detailed) blood test (leukocyte counts are taken as the basis).
After making an accurate diagnosis, they proceed to the construction of a treatment regimen.
Treatment features
Hemorrhagic vasculitis, proceeding in a mild skin-articular form, involves outpatient drug therapy with strict adherence to bed rest and diet, which completely eliminates the consumption of animal protein and obligate allergens. In the acute period, capillarotoxicosis requires hospitalization and constant medical supervision, and with the involvement of internal organs in the pathological process, the use of potent drugs prescribed in certain dosages. The group of drugs that are used to treat the disease in question (hemorrhagic vasculitis) includes the following medications:
- Disaggregants (Curantil, Trental). They are administered intravenously by drip. Additionally, for subcutaneous or parenteral injections, “Heparin” is prescribed, which is gradually canceled with a lower dosage.
- Fibrinolysis activators (drugs containing nicotinic acid).
- Steroids (plasmapheresis is possible).
- Cytostatics ("Azathioprine" or "Cyclophosphamide").
The duration of treatment of this disease in children and adults largely depends on its severity and clinical form. As a rule, for the implementation of full-fledged therapy, it takes 2-3 months with a mild course, up to six months with moderate and about 12 months with severe, accompanied by relapse and nephritis.
According to the ICD-10 encodings, hemorrhagic vasculitis is in the same group as allergic and rheumatic purpura. The treatment of these diseases is also complicated by the lack of effective drugs that directly suppress the pathological process, regardless of its location.
Nutrition for vasculitis
To prevent additional sensitization of the skin, diet is of utmost importance. With hemorrhagic vasculitis, extractive substances and all products that are difficult to tolerate by the body will have to be completely excluded from the diet. Potential allergens (chocolate, coffee, honey, nuts, citruses, strawberries, eggs, canned food) are also prohibited.
With abdominal and renal syndromes, the attending physician selects the diet taking into account individual clinical recommendations. With hemorrhagic vasculitis complicated by nephritis, it is recommended to follow diet No. 7 (its peculiarity is the inadmissibility of salt and animal products).
What are the chances of recovery
It should be noted right away that with hemorrhagic vasculitis, the prognosis is very favorable. A fatal outcome in patients with such a diagnosis can occur in the acute phase of the disease due to complications of the digestive system (internal bleeding, intussusception, intestinal infarction). In isolated cases, renal failure or serious damage to the central nervous system leads to death.