Vasculitis - symptoms, causes, types and treatment of vasculitis

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.

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.

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

Vasculitis - what is this disease?

Vasculitis (lat. Vasculum) is the collective name for a group of vascular diseases characterized by inflammation and destruction of the walls of blood vessels - arteries, capillaries, veins and others.

Synonyms for vasculitis are angiitis, arteritis.

By the nature of the pathology, vasculitis resembles atherosclerosis - it is based on thickening of the vessel wall, which reduces the lumen of the bloodstream, disrupts blood circulation, as well as the normal blood supply to one or another part of the body or organ.

Blood, in addition to delivering nutrients to all organs, also delivers oxygen to them. Naturally, due to circulatory disorders, “starving” organs malfunction, and if there is a complete interruption in the supply of blood to them, they begin to die.

The causes of vasculitis are still (as of 2021) not fully understood. There are only assumptions, for example - a combination of genetic features (predisposition), infection (staphylococci, hepatitis viruses) and unfavorable environmental factors.

The classification of vasculitis includes a large number of types and forms, however, depending on the cause, they are divided into primary (an independent disease) and secondary (appears against the background of other diseases). According to localization, there are vasculitis on the skin, in which other organs are not damaged, and internal, the consequences of which can be not only serious cardiovascular diseases, but also death.

By form, the most popular are urticarial, allergic, skin, systemic and hemorrhagic vasculitis.

Vasculitis - ICD

ICD-10: I77.6, I80, L95, M30-M31; ICD-9: 446, 447.6.

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.

Vasculitis in children

Photos of hemorrhagic vasculitis in children

Children suffer from this disease quite rarely. But all types of vasculitis have characteristic features of their course in childhood.

Hemorrhagic vasculitis in children can occur against the background of infectious diseases - influenza , ARVI, scarlet fever , chickenpox. Other provoking factors may also influence this – hypothermia, injury, allergies, etc.

The disease leads to the fact that babies develop a lot of red hemorrhages on the mucous membranes in the mouth and lips. These hemorrhages rise slightly above the mucosa. Sometimes they bleed. A papular-hemorrhagic rash appears on the body. It forms on the limbs, legs, torso, and buttocks.

Other symptoms also appear, in particular articular syndrome. Joint pain appears simultaneously with skin manifestations or a little later. The pain disappears after a few days, but when a new rash appears, it reappears. Children also often experience abdominal syndrome , the main manifestation of which is severe abdominal pain. This can complicate the diagnosis, since the pain is similar to the symptoms of appendicitis , intestinal obstruction, etc.

Lung damage in the hemorrhagic form of the disease appears less frequently in children, but in this case there is a risk of rapid pulmonary hemorrhage. With various forms of the disease, children may experience blood in their urine.

Treatment of vasculitis must be carried out in a hospital setting. As a rule, heparin , vascular drugs, sorbents, and antiplatelet agents are prescribed. In some cases, treatment with prednisolone . When the disease progresses rapidly, plasmapheresis .

During the treatment process, adherence to a strict diet is very important. If the disease has subsided, the child is under medical supervision for 5 years. After all, there is a danger of relapse.

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.

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.

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.

Types of vasculitis

Vasculitis is classified according to the 2012 Chapel Hill Consensus Conference (CHCC) as follows:

By formation:

Primary - the development of the disease is caused by inflammation of the walls of the blood vessels themselves;

Secondary - the development of the disease is due to the reaction of blood vessels against the background of other diseases. Secondary can be:

• Vasculitis associated with hepatitis B virus (HBV);
• Cryoglobulinemic vasculitis associated with hepatitis C virus (HCV);
• Vasculitis associated with syphilis;
• ANCA vasculitis (ANCA) associated with medications;
• Drug-associated immune complex vasculitis;
• Vasculitis associated with cancer (syn. “paraneoplastic vasculitis”)
• Other vasculitis.

By localization:

Vasculitis of large blood vessels:

— Giant cell arteritis (GCA, Horton's disease, temporal arteritis, senile arteritis) is an autoimmune disease characterized by granulomatous inflammation of the main branches of the aorta, most often the branches of the carotid and temporal arteries. In many cases, it is combined with polymyalgia rheumatica, pain and some stiffness in the joints of the pelvic girdle and shoulders, as well as an increase in ESR. The cause is considered to be human infection with hepatitis, herpes, influenza and other viruses. It occurs mainly in people over 50 years of age.

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— Takayasu arteritis (nonspecific aortoarteritis) is an autoimmune disease in which a productive inflammatory process develops in the walls of the aorta and its branches, leading them to obliteration. As the disease progresses, pathological processes such as the formation of fibrous granulomas, destruction of elastic fibers, necrotization of smooth muscle cells of the blood vessel wall are observed, after which, after some time, thickening of the intima and medial tunic of the vessel is possible. Sometimes there may be no pulse in the hands, which is why the disease has another name - “pulseless disease.” According to statistics, Takayasu arteritis most often develops in women, in an approximate proportion of men 8 to 1, and patients are young people, from 15 to 30 years old.

Vasculitis of medium-sized vessels:

— Periarteritis nodosa (polyarteritis nodosa, periarteritis nodosa) is an inflammatory disease of the arterial wall of small and medium-sized blood vessels, leading to the development of aneurysms, thrombosis, and heart attacks. At the same time, there is no kidney damage (glomerulonephritis). The main causes are considered to be intolerance to certain medications, as well as persistence of the hepatitis B virus (HBV).

— Kawasaki disease is an acute and febrile disease characterized by inflammatory damage to the walls of small, medium and large-diameter veins and arteries, which is often combined with mucocutaneous lymphatic syndrome. Occurs mainly in children.

Vasculitis of small vessels:

— ANCA-associated vasculitis (AAV):

• Microscopic polyangiitis (MPA) is a not fully understood disease that is associated with the production of antibodies to the cytoplasm of neutrophils, which causes an inflammatory process to develop simultaneously in several organs (most often the lungs and kidneys become victims), and granulomas do not form. Doctors note the following features of the clinical course of GPA: the development of severe pulmonary-renal syndrome (about 50%), damage to the kidneys (about 90%), lungs (from 30 to 70%), skin (about 70%), and visual organs (about 30%). ), peripheral nervous system (about 30%), gastrointestinal tract (about 10%).
• Granulomatosis with polyangiitis (GPA, Wegener's granulomatosis) is a severe and rapidly progressive autoimmune disease characterized by granulomatous inflammation of the walls of small and medium-sized blood vessels (capillaries, venules, arterioles and arteries) involving the eyes, upper respiratory tract, and lungs in the pathological process , kidneys and other organs. If not adequately treated, it can lead to death within 1 year. Doctors note the following features of the clinical course of GPA: damage to the upper respiratory tract (90% or more), kidneys (about 80%), lungs (from 50 to 70%), visual organs (about 50%), skin (from 25 to 35% ), peripheral nervous system (20 to 30%), heart (20% or less), gastrointestinal tract (about 5%).
• Eosinophilic granulomatosis with polyangiitis (EGPA, Churg-Strauss syndrome) is an autoimmune disease caused by an excess of eosinophils in the blood and outside the bloodstream, characterized by granulomatous inflammation of the walls of small and medium-sized vessels involving the upper respiratory tract, lungs, kidneys and other organs in the pathological process. Often accompanied by bronchial asthma, runny nose and other sinusitis, elevated body temperature, shortness of breath, eosinophilia.

— Immune complex vasculitis of small vessels:

• Immunoglobulin-A associated vasculitis (hemorrhagic vasculitis, Henoch-Schönlein purpura, Henoch-Schönlein disease, allergic purpura);
• cryoglobulinemic vasculitis - characterized by damage to the walls of small vessels, mainly the kidneys and skin, the main cause of which is an excessive amount of cryoglobulins in the blood serum, due to which they first settle on the walls of the vessels and then modify them.
• Hypocomplementary urticarial vasculitis (anti-C1q vasculitis);
• Anti-GBM disease.

Vasculitis that can affect blood vessels of varying sizes:

• Behçet's disease is characterized by an inflammatory process in the arteries and veins of small and medium caliber, accompanied by frequent relapses of ulcerative formations on the mucous membranes of the mouth, eyes, skin, genitals, as well as damage to the lungs, kidneys, stomach, brain and other organs.
• Cogan syndrome.

Systemic vasculitis:

• Hemorrhagic vasculitis (Henoch-Schönlein purpura) is characterized by aseptic inflammation of the walls of small vessels (arterioles, venules and capillaries), multiple microthrombosis, developing mainly in the vessels of the skin, kidneys, intestines and other organs. Often accompanied by arthralgia and arthritis. The main cause of hemorrhagic vasculitis is the excessive accumulation of circulating immune complexes in the bloodstream, in which antigens predominate, due to which they settle on the inner surface of the blood wall (endothelium). After re-activation of proteins, the vascular wall changes;
• Lupus vasculitis;
• Behçet's disease;
• Rheumatoid vasculitis;
• Vasculitis in sarcoidosis;
• Takayasu arteritis;
• Other vasculitis.

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Vasculitis of individual organs:

• Cutaneous arteritis;
• Cutaneous leukocytclastic angiitis - characterized by an isolated inflammatory process of blood vessels in the skin, without concomitant glomerulonephritis or systemic vasculitis;
• Primary angiitis of the central nervous system;
• Isolated aortritis;
• Other vasculitis.

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.

Chapter 25. Systemic vasculitis

Systemic vasculitis

Systemic vasculitis (SV) is a heterogeneous group of diseases, the main morphological feature of which is inflammation of the vascular wall, and the range of their clinical manifestations depends on the type, size, location of the affected vessels and the severity of accompanying inflammatory changes. Systemic vasculitis is a relatively rare human pathology. There are no epidemiological studies on the incidence of juvenile forms of SV. In the scientific and scientific-practical literature, SV is considered in the group of rheumatic diseases. The working classifications of systemic vasculitis proposed by experts are based on morphological characteristics: the caliber of affected vessels, the necrotizing or granulomatous nature of inflammation, the presence of giant multinucleated cells in granulomas. In ICD-10, systemic vasculitis was included in the heading XII “Systemic lesions of connective tissue” (M30-M36) with subsections “Polyarteritis nodosa and related conditions” (MZO) and “Other necrotizing vasculopathies” (M31).

Classification of systemic vasculitis ICD-10

• MZO Polyarteritis nodosa and related conditions.

M30.0 Polyarteritis nodosa.

M30.1 Polyarteritis with pulmonary damage (Churgia-Strauss), allergic and granulomatous angiitis.

M30.2 Juvenile polyarteritis.

MZO.Z Mucocutaneous lymphonodular syndrome (Kawasaki).

M30.8 Other conditions associated with polyarteritis nodosa.

• M31 Other necrotizing vasculopathies.

M31.0 Hypersensitivity angiitis, Goodpasture's syndrome.

M31.1 Thrombotic microangiopathy, thrombotic and thrombocytopenic purpura.

M31.2 Fatal median granuloma.

M31.3 Wegener's ceanoulomatosis, necrotizing respiratory granulomatosis.

M31.4 Aortic arch syndrome (Takayasu).

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.

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.

Treatment of vasculitis with folk remedies

Important! Before using folk remedies against vasculitis, be sure to consult your doctor!

Collection 1. Mix 4 tbsp. spoons of carefully crushed elderberry flowers, Japanese sophora fruits, stinging nettle leaves, yarrow grass and knotweed. 1 tbsp. Pour a glass of boiling water over a spoonful of the mixture, leave the product for an hour, and strain. The infusion should be taken during the day, 2-3 times.

Collection 2. Mix 3 tbsp. spoons of calendula flowers, elderberry flowers, mint leaves, yarrow herb, horsetail, string and poplar buds. 1 tbsp. Pour a glass of boiling water over a spoonful of the mixture, cover the container and let the product brew for 1 hour, strain. The infusion should be taken 100 ml during the day, every 3 hours.

Bergenia thickleaf. Used to cleanse the blood. To prepare the product you need 2 tbsp. Place spoons of dry bergenia leaves into a thermos and pour a glass of boiling water. The product needs to brew overnight, then filter it, add 1 tbsp. a spoonful of honey and drink it in the morning, on an empty stomach.

Bitter herbs. They are used to cleanse the intestines, which is mandatory in the treatment of vasculitis. To prepare the product you need 2 tbsp. Place spoons of one of the bitter herbs (wormwood, tansy, elecampane, immortelle) into a thermos and pour 1 liter of boiling water, leave for 2 hours, strain. You need to drink the infusion when cooled, diluting it with warm boiled water in a ratio of 1 to 1 (half a glass), 2 times a day, 30 minutes before meals.

Green tea. Green tea strengthens the walls of blood vessels, reduces their permeability, lowers the level of “bad” cholesterol in the blood, and increases the tone of the heart muscle. For a therapeutic effect, you need to drink a glass of strong green tea 3 times a day. The greatest effectiveness is achieved when taken simultaneously with other folk remedies.

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).

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.

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.