Hemorrhagic vasculitis (Allergic purpura, Henoch-Schönlein disease, Capillary toxicosis)

A common disease of immune origin, manifested as inflammation of the walls of the capillaries of the skin and internal organs, is hemorrhagic vasculitis. This is an aseptic pathology, which is characterized by the active formation of immune complexes and damage to the wall of microvessels with an increase in their permeability. The disease is systemic in nature and affects people at any age, but the largest number of patients are among children and adolescents aged 4 to 12 years. Boys get sick 2-2.5 times more often than girls.

General information

Hemorrhagic vasculitis in adults and children (synonyms - Henoch-Schönlein disease, allergic purpura, capillary toxicosis, Henoch hemorrhagic purpura) refers to systemic diseases affecting predominantly the microvasculature of the skin, gastrointestinal tract, joints, and kidneys.
The most affected part of the vascular bed includes small-caliber vessels - post-capillary venules, capillaries and arterioles with the deposition of immune complexes. The ICD-10 code for hemorrhagic vasculitis is D69.0. In 2012, the nomenclature of vasculitis was revised and Henoch-Schönlein purpura received the name IgA vasculitis, that is, vasculitis with deposition of IgA-dominant immune complexes in the vessel wall, affecting small vessels. The disease occurs in all age groups in adults, but the peak incidence occurs in children (3-8 and 7-11 years), averaging 13-18 cases/100 thousand population. Children under 3 years of age rarely get sick, which is obviously due to low immunological reactivity and the sensitization of their body has not yet taken place. The higher incidence of school-age children is due to an increase in the level of sensitization in this age period and the intensity of allergic reactions. As you get older, the incidence decreases, and after 60 years it is extremely rare. The male gender predominates in the morbidity structure (2:1). In the winter and spring periods, the incidence is higher, which is explained by a decrease in the body's reactivity during this period, an increased incidence of ARVI and intensive contacts in children's organized groups.

The specificity of the disease is the variety of clinical manifestations (skin rashes, abdominal pain, articular syndrome, kidney damage, etc.), which often at the onset of the disease leads to a visit to doctors of various specializations (local pediatrician, dermatologist, nephrologist, surgeon, neurologist), untimely diagnosis and delay of adequate treatment, thereby contributing to the development of complications and worsening prognosis.

About the development of the disease

Hemorrhagic vasculitis or Schenelein-Henoch disease is a disease in which autoimmune damage to small arteries occurs - they become fragile, their permeability increases, which causes impaired microcirculation, microthrombosis, multiple small hemorrhages (hemorrhages) as in the skin (hemorrhagic rashes on the skin and mucous membranes) , and in internal organs (gastrointestinal tract, kidneys, joints).

Most often the disease develops in childhood (6-7 years), but can also affect adults. Boys get sick 1.5 times more often than girls.

Pathogenesis

The mechanism of development of hemorrhagic vasculitis (HV) is based on a generalized immunocomplex necrotizing lesion of the blood vessels of the microvasculature of the skin and internal organs with the formation/deposition of granular IgA deposits (antigen-antibody complexes) in the vascular wall and activation of the complement system. As a consequence, a protein membrane attack complex is formed, which underlies the osmotic lysis of endothelial cells.

Also, when the complement system is activated, chemotactic factors are actively released. affecting polymorphonuclear leukocytes, which in turn secrete lysosomal enzymes that aggravate damage to the vascular wall. As a result of damage to the structure of the vascular endothelium, collagen fibers are exposed, which promotes the adhesion of platelets to the surface of the endothelium and the initiation of the blood clotting mechanism. Subsequently, fibrin deposits form in the vessels, blood rheology worsens, aggregation of erythrocytes and platelets , and intravascular disseminated blood coagulation develops.

Against the background of increased vascular permeability and the development of thrombosis with subsequent depletion of the anticoagulant (antithrombin-III) link and thrombocytopenia of consumption, ruptures of blood vessels of the microvasculature of the skin and internal organs occur, which lead to the development of clinical symptoms of hemorrhagic syndrome. The pathogenesis of hemorrhagic vasculitis is schematically presented in the figure below.

Pathogenesis of hemorrhagic vasculitis

Mechanism of vascular damage

The main factor of damage in hemorrhagic vasculitis is special complexes formed from immune cells. They are called anaphylotoxins for their destructive properties. Under normal conditions they disintegrate. In a sick person, complexes accumulate and become antigens.

Deposition on the inner wall of blood vessels with a change in permeability causes blood circulation problems:

• platelets are activated;
• the plasma content of specific antithrombin decreases;
• the process of fibrinolysis is blocked.

As a result, a mass of microthrombi is formed in small vessels against the background of allergic inflammation and reduced blood clotting.

Classification

There is no single generally accepted classification of HS. The most commonly used clinical classification of the disease is based on one or another clinical syndrome. Accordingly, cutaneous, articular, renal, abdominal and mixed forms are distinguished.

According to severity they are distinguished:

• mild: few rashes; general condition is satisfactory; arthralgia ;
• moderate: profuse rashes, general condition of moderate severity, arthritis , arthralgia , microhematuria , periodic abdominal pain, slight proteinuria ;
• severe: profuse confluent rashes, severe general condition, angioedema , gross hematuria , persistent abdominal pain, nephrotic syndrome , gastrointestinal bleeding , acute renal failure .

According to the nature of the course: acute form (1-2 months), protracted (up to 6 months), chronic with frequent relapses.

Clinical classification

Clinicians distinguish the following forms of the disease:

• simple or cutaneous - the whole process is limited to skin manifestations;
• skin-articular - in addition to the characteristic skin rashes, there are signs of inflammation of the joints;
• skin-abdominal - against the background of skin changes, symptoms of dysfunction of the stomach and intestines appear;
• renal - rare, accompanied by damage to the renal vessels with impaired filtration and urine excretion;
• mixed (combined) - there are changing symptoms of different forms.

Based on the type of disease, the following are distinguished:

• fulminant form - characterized by a rapid course, lasts several days, occurs in children under 5 years of age;
• acute - the disease has a sudden onset and lasts up to 30 days;
• subacute - lasts up to three months;
• protracted - clinical manifestations last for six months;
• recurrent - characterized by the repetition of symptoms at least four times in the next few years;
• chronic - the clinical picture of the disease lasts several years with periodic exacerbations.

Causes of hemorrhagic vasculitis

The causes of hemorrhagic vasculitis in adults have not yet been fully elucidated, however, in most cases, hemorrhagic vasculitis is of an infectious-allergic nature. Among the leading factors contributing to the development of the disease are:

• Infectious factor. Statistics show that in 60-80% of cases, hepatitis B is preceded by an upper respiratory tract infection. At the same time, the range of infectious agents is quite wide: Yersinia , streptococci , mycoplasmas , legionella , hepatitis viruses, respiratory syncytial virus , adenoviruses , Epstein-Barr , cytomegalovirus and others.
• The presence of foci of chronic infection ( tonsillitis , caries , adenoiditis , sinusitis , etc.).
• Taking medications (sulfonamide drugs, penicillin , ampicillin , erythromycin , antiarrhythmic and other drugs).
• Ingestion of potentially allergenic foods (chocolate, eggs, citrus fruits, dairy products, fish, strawberries, wild strawberries, etc.).
• Vaccination/serum administration.
• Insect bites.
• Excessive insolation/hypothermia.

There is also evidence of a genetic predisposition to hepatitis B, which is caused by deficiency of complement C7, as well as the presence of antigens A1, A2, A10, C3HLA Bw35, B8. Thus, in conditions of sensitization of the body, any etiologically significant factor listed above can be decisive in the development of hemorrhagic vasculitis. However, in a significant number of cases, the causes of capillary toxicosis cannot be determined.

How to prevent exacerbations?

After discharge from the hospital, the patient should not forget about his illness at home. Of course, by that time he will already know everything about the nature of the disease, prevention of exacerbations, behavior in everyday life, nutrition and daily routine. Having immersed himself in a homely atmosphere, the patient will not take any medications without a doctor’s prescription, will not attack foods that can provoke a relapse (allergens), but will eat well and will take quiet, easy walks in the fresh air every evening.

In addition, the following are contraindicated for a person:

• Excessive anxiety, mental stress;
• Vaccinations (possible only for health reasons);
• Hard physical labor (children are exempt from physical education lessons);
• Hypothermia;
• Administration of immunoglobulins (antistaphylococcal, antitetanus, etc.).

Due to the fact that hemorrhagic vasculitis is predominantly a childhood disease, special recommendations are provided for children (or parents?):

• Every six months the child visits the attending physician (in the absence of exacerbations);
• Clinical observation for at least 5 years if the kidneys remain healthy, but if they are damaged, control may be lifelong;
• Children are completely exempt from physical education for a year, then move to the preparatory group;
• A mandatory visit to the dentist and otolaryngologist is required 3 times a year;
• Regular urine tests (general and Nechiporenko) and fecal tests for helminths;
• Vaccinations are excluded for 2 years, and after this time, routine vaccination is carried out, but with the permission of the attending physician and under “antihistamine cover”;
• Compliance with an anti-allergenic diet – 2 years;

Advice to parents or other relatives about treatment at home, relapse prevention, nutrition and behavior at school and at home.

Symptoms of hemorrhagic vasculitis

Clinical symptoms of Henoch-Schönlein disease are represented by four typical clinical syndromes: skin, articular, abdominal and renal. The number of organ manifestations of the disease most often varies from 1 to 2 of all classical clinical syndromes, which can develop in various combinations and in any sequence throughout the entire period of the disease. In rare cases, damage to other organs may occur: lungs, central nervous system, heart.

The onset of breastfeeding is often preceded by a prodromal period lasting from 4 to 12 days. In most cases, within 1-4 weeks the patient suffers from a disease, more often a sore throat , acute respiratory viral infection, exacerbation of a chronic disease, or has a history of allergic exposure (taking medications, vaccinations, exacerbation of an allergic disease). Clinical symptoms of this period are not specific and often manifest as general malaise, fever, headache, and loss of appetite.

The onset of hepatitis can develop gradually, when the first symptoms of purpura appear gradually against the background of the patient’s well-being and complete health and the general condition of the patients does not significantly affect. This type of onset of the disease is typical for isolated skin lesions. However, in some cases the disease begins acutely.

Hemorrhagic purpura can begin with any syndrome, but more often the onset of the disease is manifested by cutaneous hemorrhagic syndrome, which is gradually joined by lesions of other systems and organs.

Skin syndrome (form). Skin lesions are observed in all patients with hepatitis B and are a mandatory and most important diagnostic criterion. Typical localization of skin rashes: lower extremities - mainly legs and feet. Less commonly, the rash spreads to the thighs, torso, buttocks, upper limbs, and extremely rarely to the face. Hemorrhagic rash in most cases is represented by purpura , petechiae or a polymorphic rash , less often - urticarial, erythematous-macular elements or a bullous-necrotic form. The photo below of hemorrhagic vasculitis in adults shows various forms of skin syndrome.

Forms of skin syndrome of hepatitis B: a) hemorrhagic, b) urticarial; c) papulo-ulcerative; d) necrotic-ulcerative; e) polymorphic

Cutaneous hemorrhagic syndrome has a number of specific features. The rash is symmetrical, has a small-spotted/petechial character, while the size of the rash elements is 2-5 mm, protrudes above the surface of the skin, does not disappear with pressure, is prone to recurrence and fusion, has a pronounced variegation of rash elements due to the proximity of newly formed ones to old ones, which are at various stages of reverse development, are localized predominantly on the extensor surfaces of the limbs around the joints. Quite often, the onset of the disease is accompanied by various types of allergic rash - urticaria (allergic vasculitis).

In the early period of hepatitis B, the elements of the rash have a reddish color, but in the process of evolution they quickly acquire a characteristic bluish-purple color, then turn pale and within 3-5 days, as they develop back, they acquire a yellowish-brown tint. In severe cases, with high activity of the pathological process, some of the skin elements undergo necrosis, which is caused by microthrombosis and tissue ischemia. The skin syndrome of hepatitis B is characterized by a wave-like course, most often 2-5 episodes are observed. At the same time, newly appearing rashes can be caused by errors in diet, taking medications, or violation of bed rest. In some cases, the rash is accompanied by itching, and in 30-35% of patients hemosiderosis (residual long-term pigmentation) is observed, accompanied by peeling.

A common form of manifestation of skin syndrome can be angioedemalocalized on the hands, feet and face. In boys, swelling of the scrotum occurs. The tissues in the affected areas are bluish in color and pasty.

Articular form (syndrome). Involvement of joints in the pathological process is second in frequency of occurrence after skin syndrome. Quite often it occurs together with skin syndrome or appears several hours/days after it (cutaneous-articular form). Great difficulties in diagnosing hepatitis B are presented by cases in which the articular syndrome appears primarily, proceeds according to the type of migratory polyarthralgia/arthritis and precedes skin manifestations. Its development is based on impaired vascular permeability, which contributes to the development of swelling of the skin/subcutaneous fat in the area of ​​medium/large joints, mainly the wrist and ankle; hemorrhages are less common. Small joints of the hand and foot are practically not involved in the pathological process.

Clinical changes in the joints are manifested by local hyperemia, increased volume, pain and tenderness on palpation, limitation of movements, and increased local temperature. Less commonly, painful contractures develop. Clinical symptoms develop against the background of an increase in body temperature to febrile levels and persist for 2-5 days, after which they disappear without a trace, leaving no deformities. Often articular syndrome occurs against the background of angioedema .

Abdominal syndrome . It occurs in 50-60% of patients and in a third of them it is preceded by skin syndrome, which significantly complicates diagnosis and is a common reason for surgical intervention. The main clinical sign is severe, sudden, cramping pain in the abdomen, without pronounced localization. The nature of the pain is similar to intestinal colic, more often in the navel, less often in the epigastric/right iliac region, often imitating the characteristic picture of a stomach ulcer , appendicitis , pancreatitis and even acute intestinal obstruction . The pain is often extremely intense, causing patients to take a forced position in bed, rushing about and screaming. The pain syndrome is caused by numerous hemorrhages in the intestinal wall, hemorrhages in the mesentery, hemorrhagic impregnation of the mucous membrane and intestinal wall, which can lead to the formation of areas of necrosis and bleeding. At the peak of pain, false urges with frequent bowel movements, vomiting mixed with blood, and the appearance of fresh blood in the stool are possible.

An objective examination reveals pain on palpation of the abdomen, bloating, however, signs of peritoneal irritation are usually absent. The duration of abdominal syndrome varies from several attacks over 2-3 days to 8-10 “waves” over several months. The syndrome is not constant, and the symptoms are unstable. Often, a relapse of abdominal pain is combined with another wave of skin manifestations.

Against the background of abdominal pain, patients may experience pale skin, a sunken face, a dry tongue, sunken eyes, and fever. With heavy bleeding, there is a high risk of developing acute posthemorrhagic anemia and collapse . Serious complications may include intestinal obstruction caused by the closure of its lumen with a hematoma, intestinal perforation , and peritonitis . The development of abdominal syndrome significantly aggravates the process and aggravates its course, requiring intensive therapy.

Kidney syndrome . Develops in approximately 30-50% of patients. This hepatitis syndrome always develops only after the appearance of a hemorrhagic rash, but it can join the symptom complex at different time periods of the disease. Most often, renal syndrome develops 1-2 months into the disease. There are 2 clinical variants of kidney damage:

• Transient urinary syndrome in the form of micro/macrohematuria or hematuria with moderate proteinuria in combination with other manifestations of the disease and with an undulating course.
• Schonlein-Henoch nephritis: occurs more often with a hematuric form, less often with a form of progressive glomerulonephritis and transition in 25-50% of cases to chronic glomerulonephritis, which sharply worsens the prognosis due to the presence of arterial hypertension/nephrotic syndrome and increases the risk of developing chronic renal failure. Therefore, careful monitoring of urine composition and kidney function throughout the disease is necessary.

The severity of clinical manifestations largely depends on the activity of the pathological process, according to which several degrees of severity of the course of hepatitis B are distinguished:

• Mild degree: skin rashes are not abundant, general condition is satisfactory, body temperature is subfebrile/normal, there are no signs of damage to organs/systems, ESR is up to 20 mm/hour.
• Moderate severity: the skin syndrome is clearly defined, the general condition is moderate, intoxication (weakness, headache, myalgia) and articular syndromes are pronounced, fever (hyperthermia over 38°C), abdominal/renal syndromes are moderate. In the blood - an increase in ESR to 40 mm/hour, an increase in eosinophils, leukocytes, neutrophils, dysproteinemia , hypoalbuminemia .
• Severe degree: the general condition is severe, the symptoms of intoxication are very pronounced (weakness, high fever, myalgia, headache). Almost all the main syndromes are expressed - skin, articular, renal, abdominal (paroxysmal abdominal pain, vomiting mixed with blood); there may be damage to the peripheral nervous system and central nervous system. In the blood there is anemia, severe leukocytosis, neutrophilia, ESR more than 40 mm/hour.

Henoch-Schönlein purpura can also occur with the involvement of other systems (cardiovascular, gastrointestinal tract, central nervous system) and organs (liver, lungs) in the pathological process, however, such variants of the disease are much less common.

General and skin symptoms

Symptoms of hemorrhagic vasculitis usually appear suddenly without a prodromal stage. The clinic grows over several weeks. In most cases (especially in adults), a high temperature rises or a low-grade fever is maintained. Patients feel unwell, weak, and have a headache. These are the initial signs of intoxication. The diagnosis is still unclear, since many diseases occur this way.

A more typical sign is a variety of rashes on the body. It can appear on the back of the feet, buttocks, and legs. Less often on the stomach, arms, back. Usually symmetrical in nature.

For hemorrhagic vasculitis, skin manifestations on the face, neck, palms and soles of the feet are uncharacteristic. This sign is taken into account in differential diagnosis.

According to the description, the elements of the rash are different:

• bright red small blisters;
• papules that turn pale when pressed;
• possible location of red papules on the mucous membrane of the mouth and pharynx;
• purple nodules;
• minor bruises.

The diameter of small elements of the rash reaches 1 cm. They last up to four days, then turn pale and disappear. The next wave of skin manifestations should be expected after 7–10 days.

Initial manifestations most often appear as a small petechial rash, localized on the legs and tops of the feet.

Gradually the rash reaches the thighs and buttocks

Much less often the arms, back and stomach are involved in the process. At the same time, patients feel severe itching in the area of ​​the rash, on the surface of the head covered with hair, around the eyes.

On examination, swelling of the lips, eyelids, and perineum is noted. The rash area is painful when touched. After the spots disappear, pigment spots remain, and during relapse they begin to peel.

Tests and diagnostics

The diagnosis of hepatitis B is made by identifying specific clinical syndromes, first of all, the presence at the time of examination/history of skin hemorrhagic bilateral rashes and establishing a connection between the onset of breastfeeding and infectious diseases, an allergic history, and changes in diet. There are no specific laboratory tests. Laboratory research:

• OAC - thrombocytosis and nonspecific changes common to any inflammatory process, accelerated ESR, leukocytosis.
• OAM—hematuria/proteinuria.
• Biochemical blood test - blood for CRP, liver tests, creatinine, urea.
• Coagulogram - hypercoagulation is noted, accompanied by a decrease in the activity of antithrombin III and plasmin, and a decrease in chlorides.
• Immunogram - an increase in the concentration of IgA in the blood serum.
• Fecal occult blood test (positive for gastrointestinal bleeding).

In confirming the clinical diagnosis, a leading role is played by a skin biopsy and an immunohistochemical study to reveal the fixation of IgA-containing immune complexes in the vascular wall. If necessary, an ultrasound of the kidneys and abdominal organs and an ECG are prescribed.

Differential diagnosis is necessary with vasculitis in autoimmune diseases ( rheumatoid arthritis , Crohn's disease , systemic lupus erythematosus , ulcerative colitis ), vasculitis of infectious etiology, infections (hepatitis B and C, infectious subacute endocarditis , tuberculosis ), drug allergies and malignant neoplasms.

How does the disease progress depending on the degree of activity?

To choose the correct therapy for hemorrhagic vasculitis, the doctor needs to know the degree of activity of inflammatory phenomena in the blood vessels. According to the clinic and the simplest laboratory tests, it is customary to distinguish 3 degrees:

• Grade I - the patient’s condition is satisfactory, the temperature is not elevated or slightly above 37, there are moderate non-widespread skin rashes, the ESR in the blood is not higher than 20 mm/hour, there are no other manifestations.
• Stage II - the patient’s condition is moderate, skin manifestations are intense, body temperature is over 38 degrees, there are signs of intoxication (muscle and headaches, weakness), there are clear signs of articular syndrome, abdominal and kidney symptoms are moderate. In the blood test: leukocytosis with eosinophilia and neutrophilia, ESR increases to 40 mm/hour, the ratio of protein fractions is disturbed towards a decrease in albumin.
• Degree III - the patient’s condition is objectively assessed as severe, there is a constant high temperature, intense symptoms of intoxication. The patient suffers from the simultaneous manifestation of skin, articular, renal and abdominal syndromes. Possible brain damage. Blood test: high leukocytosis due to neutrophils, ESR exceeds 40 mm/hour, possible decrease in red blood cells, hemoglobin and platelets (anemic syndrome).

The ankle and knee joints are most often affected, rarely the elbows and wrists.

Diet

Hypoallergenic diet

• Efficacy: therapeutic effect after 21-40 days
• Timing: constantly
• Cost of products: 1300-1400 rubles. in Week

Diet Table No. 1

• Efficacy: therapeutic effect after 3 weeks
• Terms: 2 months or more
• Cost of products: 1500 - 1600 rubles. in Week

Diet 7 table

• Efficacy: therapeutic effect after a week
• Terms: 1 month or more
• Cost of products: 1200-1300 rubles per week

A hypoallergenic diet is prescribed, which involves excluding obligate allergens from the diet: milk, fish, citrus fruits, chocolate, cocoa, orange and red vegetables/fruits, as well as extractive substances. For abdominal syndrome, a modified Table No. 1 according to Pevzner is indicated until it is completely relieved. For nephritis - dietary Table No. 7 with a gradual transition to a hypochloride diet.

How is the treatment carried out?

Treatment of hemorrhagic vasculitis begins with the doctor’s recommendations on the regimen and diet.

In case of exacerbation, strict bed rest is necessary. It is advisable to hospitalize the patient in a hospital, since correction and selection of medications are necessary. They are often administered by drip and intramuscular injection.

Outside of exacerbations, patients should not be in the sun and physical activity should be limited.

The diet for hemorrhagic vasculitis should exclude foods that cause allergies and increase blood clotting.

Not recommended for use in food:

• coffee and cocoa;
• candies;
• citrus fruits;
• eggs;
• spicy seasonings, mayonnaise;
• red varieties of berries;
• fried and smoked meat and fish dishes.

Chocolate is considered a strong food allergen

With the development of abdominal syndrome, the patient is prescribed nutrition with intravenous administration of special solutions. Dietary dishes are prepared according to the principle of table No. 1 (exacerbation of peptic ulcer disease).

Typically, hemorrhagic vasculitis is treated and observed in a clinic by a rheumatologist. Medicines of different groups are used.

To prevent hypercoagulability, the following is prescribed:

• disaggregants (Curantil, Trental);
• Heparin (dose is calculated based on the patient’s weight);
• as an activator of the fibrinolysis process - nicotinic acid in injections.

To relieve inflammation, corticosteroids are used, possibly a combination of tablets and intramuscular injections.

To limit the autoimmune process, cytostatics (Azathioprine, Cyclophosphamide) are rarely used. They are prescribed only in severe cases, for example, with autoimmune glomerulonephritis.

The dosage is selected individually in each case. You cannot change or cancel it yourself.

According to indications, plasmapheresis and transfusion of blood components are performed.

Prevention

The main measures to prevent hepatitis B in its relapses include:

• Adequate treatment of acute infectious diseases of the upper respiratory tract and timely sanitation of infectious chronic foci of ENT organs.
• Exclusion/minimization of contacts with allergens (if there is a history of allergic reactions) and control of medications.
• Preventing hypothermia of the body.
• Strengthening the immune system (hardening, rational nutrition).
• Dispensary observation of ill persons for 2 years.

Other symptoms

Among the symptoms of hemorrhagic vasculitis, it is important to highlight damage to internal organs and joints.

Classic signs of arthritis include:

• painful movements;
• swelling;
• redness.

Articular syndrome can develop simultaneously with skin rashes or appears gradually in 70% of patients, becoming more pronounced with each exacerbation. For adult patients it is more typical; in ¼ of patients it is preceded by skin rashes, and the pain is migrating in nature.

Depending on the intensity of pain, options are possible: from moderate discomfort during movements to severe inflammation with subsequent change in shape due to severe swelling. There are no joint deformities or persistent movement disorders. The pain lasts for several hours.

From the digestive organs the following are observed:

• short-term cramping abdominal pain (colic);
• nausea and vomiting;
• an admixture of blood in the stool due to bleeding of the intestinal mucosa.

In children, enlargement and tenderness of the inguinal lymph nodes are detected.

Abdominal syndrome is present in 23% of sick children. The pain is associated with swelling and rashes along the peritoneum. They usually do not cause serious bowel problems. They appear simultaneously with skin rashes or later, do not cause a significant deterioration in the patient’s condition, and are often identified during a targeted interview.

If the treatment of hemorrhagic vasculitis is delayed, then the adult patient experiences vomiting and diarrhea with blood as signs of gastric and intestinal bleeding.

Moderate, harmless bleeding occurs in half of patients. Cases of massive blood loss occur in 5%. Rarely, serious complications are possible:

• perforation of the wall with the development of peritonitis;
• intussusception.

During endoscopic examination, patients show signs of hemorrhagic or erosive gastritis, duodenitis, and less often - other localization in the intestine.

The elements are capable of merging and forming widespread areas of purple color

Anemic syndrome is caused by a decrease in the content of hemoglobin and red blood cells. It manifests itself:

• dizziness;
• weakness;
• noise in the head;
• fainting states;
• flashing “flies” before the eyes;
• tachycardia.

Kidney damage occurs in 10–60% of patients and causes:

• acute glomerulonephritis of autoimmune nature;
• there is an admixture of blood in the urine (red blood cells pass through the wall of inflamed arterioles).

Usually begins 1-3 weeks after the skin rash. The isolated form is rare.

If the respiratory tract and lobes of the lungs are affected, the patient experiences coughing and shortness of breath. The sputum may contain an admixture of blood due to increased bleeding of the capillary bed.

On the part of the cardiovascular system, pronounced tachycardia and a tendency to arrhythmias are noted.

Damage to the nervous system is considered an unfavorable consequence of the disease. They are caused by the transition of inflammation from blood vessels to the meninges. Therefore, they manifest themselves as symptoms of meningitis, encephalitis (severe headaches, loss of coordination, convulsions).

Consequences and complications

The main complications of hepatitis B include: intestinal obstruction , pancreatitis , perforation , peritonitis , posthemorrhagic anemia , thrombosis / infarction in organs, neuritis , cerebral disorders. The lethal outcome develops mainly as a result of abdominal complications (intestinal perforation, peritonitis), the development of chronic renal failure , and hemorrhages in organs (brain).

Causes

The etiology of the disease has not been fully studied; it is only recognized that the nature of hemorrhagic vasculitis is infectious-allergic. More often the disease develops when the weather is cold and damp.

The influence of respiratory infections on the development of the disease has been noted: as a rule, the first signs of vasculitis begin to make themselves felt a week to a month after suffering a severe infectious disease. The trigger can be tracheobronchitis, tonsillitis, rhinopharyngitis.

Cases have been recorded where the onset of pathology was provoked by taking antibiotics, especially the penicillin group, or antiarrhythmic drugs. A provoking factor such as preventive vaccination has also been reported if it was carried out immediately after an acute respiratory illness.

Deviations in the functioning of the immune system can also cause disease. The risk group also includes people with allergic dependence - be it a drug, food, or cold allergy.

Hemorrhagic vasculitis can be caused by insect bites, hypothermia, burns and other injuries. Pregnancy, diabetes mellitus, the presence of malignant tumors, cirrhosis of the liver, and decreased immune functions can also serve as a background for the development of the disease.

Note! Hemorrhagic vasculitis is not a common disease: on average, 14 out of one hundred thousand people get it. In children under three years of age, this pathology is very rare.

Forecast

The prognosis is generally favorable and in 75% of cases patients recover within 1-2 years after the disease. In a number of patients with a chronic relapsing course, the disease can continue indefinitely with periodic relapses of one (skin) or several syndromes. The frequency of relapses throughout the entire period can vary from one to monthly, acquiring a monosyndromic character over time: mainly in the form of cutaneous purpura or a skin-articular form, less often - chronic nephritis in the form of a hematuric form with preservation of kidney function over a long period.

The prognosis is less unfavorable with the development of nephritis , especially when the process becomes chronic with manifestations of glomerulonephritis and transition to chronic renal failure. Purpura fulminans has a poor prognosis.

Diagnostic signs

The international community of rheumatologists has established the main diagnostic signs (criteria) of Henoch-Schönlein disease, which form the basis for differential diagnosis with other vasculitis:

• purpura - rashes that rise above the surface, which can be felt on palpation, and are not associated with low platelet levels;
• patient age under 20 years;
• abdominal pain, especially after eating, signs of intestinal ischemia, bleeding;
• The result of the biopsy is granulocytes in the wall of small vessels (arterioles and venules).

It is believed that if a patient has 2 or more signs, the accuracy of the diagnosis is 87%.

List of sources

• Lyskina G. A. Systemic vasculitis. In the book: Pediatric rheumatology. Guide for doctors / Ed. A. A. Baranova, L. K. Bazhenova. M.: Medicine, 2002. pp. 221–270.
• Kulaga V.V., Romanenko I.M., Afonin S.L. Allergic diseases of the blood vessels of the skin. Lugansk: “Etalon-2”, 2006. 168 p.
• A practical guide to childhood diseases, edited by prof. Kokolina V.F. and prof. A.G. Rumyantsev, volume IV. Hematology/oncology of childhood (edited by Rumyantsev A.G. and E.V. Samochatova). Medical practice – M. Moscow 2004 Zinovieva G.A., Frolkova E.V. Hemorrhagic vasculitis. Clinic and treatment // ros. pediatrician, journal — 1998. -N12. P.24-26.
• Krivosheev O.G., Gulyaev CB, Semenovykh A.G. Modern principles of treatment of Henoch-Schönlein purpura // Doctor. 2007. No. 4. -P.54-55.

How does the lightning form proceed?

The basis of the pathology of the fulminant form is determined by a hyperergic reaction with the subsequent development of acute necrotic thrombosis and vasculitis.

The pathology most often affects children 1 to 2 years old and accompanies childhood infections (chickenpox, rubella, scarlet fever). It manifests itself as typical extensive areas of hemorrhages on the skin, necrosis, cyanotic large confluent spots on the hands, feet, buttocks, and face. Next, gangrene of the hands and feet, a state of shock, and coma develop.

You can learn more about the clinical manifestations of Henoch-Schönlein disease in children here.

What to do during the recovery period?

Treatment during the rehabilitation period is aimed at preventing relapses and transition to a chronic form. The child needs:

• comply with dietary requirements throughout the year;
• identify and treat chronic foci of infection;
• monitor for possible hypothermia;
• protect yourself from viral infections;
• Do not allow sunbathing.

Dental sanitation is required

Clinical follow-up is carried out by a rheumatologist over the next 3–5 years. During this period, vaccinations are contraindicated.

If the kidneys and intestines are damaged, special diets should be followed until complete recovery.

Drug treatment

All medications when treating a child with hemorrhagic vasculitis are used very carefully, taking into account individual sensitivity. Doses of medications are calculated based on the child’s weight individually. Not recommended:

• antibiotics,
• sulfonamides,
• calcium supplements,
• ascorbic acid.

The treatment process must resist the mechanisms of damage.

To remove toxins and waste from the intestines, medications with a sorbing effect (enterosorbents) are used:

• Thioverol,
• Polyphepan,
• Nutricleans.

They are prescribed for a period of one to three months.

Antiplatelet agents - prevent thrombosis, improve microcirculation. Are used:

• Chime,
• Aspirin,
• Ticlopedin.

The duration of the course depends on the complete relief of purpura. Reopoliglyukin, Reomacrodex - solutions for intravenous administration, improve blood viscosity.

Heparin is the main anticoagulant that suppresses blood clotting factors.

Antihistamines are aimed at suppressing food and drug allergies. Drugs of choice:

• Tavegil,
• Terfen,
• Diazolin,
• Fenkarol.

Antibacterial treatment is prescribed for:

• active infection, possibly causing vasculitis;
• in the stage of exacerbation of chronic inflammatory foci;
• obvious glomerulonephritis;
• persistent course of purpura.

In such cases, the use of macrolide antibiotics is justified:

• Sumameda.
• Klacida.

Glucocorticoids are hormonal anti-inflammatory drugs to suppress the inflammatory response. Prescribed in severe and moderate conditions.

In the case of an undulating course of hemorrhagic vasculitis, additional agents are used in treatment to stabilize the inflammation.

Nonsteroidal anti-inflammatory drugs have the ability to suppress a number of enzymes, promote the synthesis of essential prostaglandins, and increase the mobility of neutrophils. Ortofen is used and should not be combined with Aspirin. Plaquenil prevents sensitization of cell membranes.

A group of cytostatics is used if:

• there are contraindications to the prescription of hormonal therapy;
• despite treatment, nephritis progresses or recurs;
• the therapy being carried out is ineffective.

In the treatment of children, the least severe drug is used - Azathioprine.

Vitamin supplements (vitamin E, Retinol, Rutin) enhance other medications, stimulate defense, and remove toxins.

Treatment regimen and nutritional features

Treatment of a child with hemorrhagic vasculitis must begin in a hospital setting. Three weeks of bed rest followed by extension is usually suggested.

Complex therapy necessarily includes a strict diet. The following are excluded from the diet:

• coffee, cocoa, products containing them (all types of chocolate);
• fruits of citrus trees (oranges, lemons, tangerines, grapefruits);
• fresh berries;
• nuts;
• candies and sweets;
• butter dough;
• eggs;
• canned foods.

Meat and fish dishes are prepared only boiled. Spicy and salty seasonings and pickled vegetables are not allowed.

If abdominal syndrome is suspected, a gentle semi-liquid diet is prescribed, as for peptic ulcer disease (table No. 1). If kidney damage is detected, then dietary table No. 7 is used.

If bed rest is violated early, the rash may recur (orthostatic purpura)

Treatment

When the acute phase of the disease occurs, patients are prescribed bed rest, following a hypoallergenic diet and limiting the intake of liquid and salt. Avoid taking medications (including antibiotics) that increase sensitization – increased sensitivity of the body to allergens.

For any clinical manifestations of the disease, antiplatelet agents are prescribed - drugs that block the formation of blood clots in the vessels, as well as fibrinolysis activators, which dissolve existing blood clots.

Therapy for hemorrhagic vasculitis of the skin is carried out with the use of corticosteroids (drugs with anti-inflammatory effects). If the expected effect is not present, cytostatics can be used to slow down the process of cell division.

In approximately 50% of cases, doctors manage to completely cure hemorrhagic vasculitis of the skin; for the remaining patients, they manage to transfer the disease into remission, which after some time can again turn into an acute form.

Volatile pain in the joints is relieved with the help of anti-inflammatory drugs (indomethacin, ibuprofen); aminoquinoline derivatives, drugs used for rheumatic diseases and systemic connective tissue diseases, can also be prescribed.

In case of kidney damage, cytostatics and glucocorticoids are used for treatment - steroid hormones produced by the adrenal cortex. Electrophoresis with nicotinic acid and heparin may be prescribed to the kidney area. A severe stage of the disease does not exclude the use of hemodialysis or kidney transplantation.

Abdominal syndrome involves the use of glucocorticoid drugs. In case of intestinal obstruction or penetrating damage to the intestinal walls, surgical intervention is required.

In severe cases of hemorrhagic vasculitis, a treatment method such as extracorporeal hemocorrection can be used. The essence of the method is that blood taken from a patient’s vein is freed from pathological substances using special microfilters, filled with drugs designed to suppress the disease, and then the blood is returned to the body.

Classification of the disease

Vasculitis is classified based on etiology, pathogenesis, type of vessels affected and the nature of the rash. Genetic and demographic factors, clinical manifestations and predominant lesions of internal organs are also taken into account.

Based on the factors preceding the disease, primary and secondary vasculitis are distinguished. Primary - caused by inflammation of the vascular wall, secondary - in which vascular inflammation is a reaction to another disease (lupus erythematosus, rheumatism, diabetes mellitus, granulomatosis, sarcoidosis, hepatitis).

according to the diameter of the affected vessels :

• small vessels - affects capillaries and venules, arterioles and small arteries of internal organs;
• middle vessels - mainly affects the abdominal arteries and their branches. Often cause stenoses and aneurysms;
• large vessels - affect the aorta and its main branches.

In addition, there are groups of vasculitis :

• small vessels with immune complexes;
• with trigger connective tissue diseases;
• with an established etiology of a metabolic or infectious nature;
• with damage to one organ.