What does vasculitis look like on the legs and face, the reasons for its appearance

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

Accurate and timely diagnosis determines 50% of the effectiveness of treatment for vasculitis of any form or type. Of no small importance is the elimination of initial organ damage and associated diseases.

When treating pathologies with severe symptoms, an integrated approach is required. Only complex therapy will quickly relieve the unpleasant symptoms of the disease, improve the patient’s condition and avoid serious complications.

Any type of treatment for vasculitis should be agreed with the attending physician and carried out only after a comprehensive diagnosis and diagnosis.

Drug treatment

Conducted for the purpose of:

• eliminating pathological reactions from the immune system that underlie the disease;
• prolongation of periods of stable remission;
• therapy for relapses of pathology;
• preventing the occurrence of secondary ailments and complications.

Treatment with medications involves prescribing and taking the following medications:

• Glucocorticoids . A special group of hormonal drugs that have a pronounced effect: antiallergic, anti-inflammatory, anti-stress, immunoregulatory and some others. Taking the drugs has high rates of therapeutic effectiveness and provides longer and more stable periods of remission. The most popular and frequently used glucocorticoids for vasculitis are Hydrocortisone and Prednisolone.
• Cytostatics . Medicines that slow down the growth and division of all cells in the body, including tumor cells. Taking medications in this series is especially effective for vasculitis accompanied by kidney damage. For systemic vasculitis, simultaneous administration of glucocorticoids and cytostatics ensures the fastest possible relief of pathological symptoms and improvement of the condition. The average course of taking cytostatics for vasculitis is from 3 to 12 months. Common cytostatics recommended for use for vasculitis are Doxorubicin, Methotrexate, Cyclophosphamide, etc.
• Monoclonal antibodies . A special type of antibody produced by the body's immune system. Drugs from this category are indicated for patients who, for one reason or another, cannot undergo cytostatic therapy. Medicines in this series have their own contraindications for use. The main one is viral hepatitis B. The most popular monoclonal antibodies among doctors to prescribe are Rituximab.

• Immunosuppressants . Drugs that are used as part of complex therapy together with glucocorticoids and provide an inhibitory effect on the immune system. Azathioprine and Leflunomide are the most popular drugs in this series.
• Human immunoglobulins . Medicines that are most effective for severe infectious lesions of the kidneys and other organs, hemorrhagic alveolitis.
• Anti-infectives . Used in case of development of vasculitis against the background of diseases and pathologies of an infectious nature of bacterial origin. Viral lesions are treated with antiviral medications - Interferon, Lamivudine. Viral hepatitis is treated with drugs based on the type of hepatitis virus.
• Anti-intoxication therapy . The main purpose of its implementation is to remove toxins from the body. For binding, popular absorbent preparations are used - Atoxil, Enterosgel.
• NSAIDs . They are used in the case of thrombophlebitis with nodular forms of vasculitis, persistent inflammatory processes, and extensive necrotic foci. Ibuprofen, Indomethacin, Acetylsalicylic acid are the most popular and frequently prescribed non-steroidal anti-inflammatory drugs with high rates of effectiveness.
• Anticoagulants . Indicated for use by patients with a tendency to thrombosis. Drugs in this category prevent the formation of blood clots in blood vessels, improve circulatory processes, normalizing blood flow in the bloodstream. Coagulants effective for vasculitis are Heparin and Warfarin.
• Antihistamines . Antiallergic drugs are prescribed when allergic reactions occur - food or drug allergies. Tavegil, Claritin, Diazolin are relatively inexpensive, but quite effective antihistamines.

For certain indications, ACE inhibitors and vasodilators can be used for vasculitis.

Quite often, for vasculitis with damage to the skin, local agents are used - creams, ointments, gels with anti-inflammatory and analgesic effects. They are applied directly to the affected areas of the skin. The main purpose of their use is to reduce the severity of the inflammatory process, as well as relieve intense pain.

In patients with areas of necrosis on the skin, as well as ulcers, regular dressings with the use of local antiseptic drugs, as well as ointments that accelerate epithelialization processes are indicated.

Non-drug therapy

It includes modern methods of extracorporeal hemocorrection - special procedures for purifying the blood of substances that cause the occurrence and development of vasculitis.

Main types of non-drug treatment:

• Hemocorrection . Provides for purification of the patient's blood using a special sorbent. Blood is drawn using a venous catheter. Afterwards, the blood is passed through a special apparatus, which introduces an absorbent component into it. The blood then returns to the bloodstream. At the same time, the sorbent does its job - cleanses the blood and its components, improves blood circulation and tissue nutrition.
• Immunosorption . A procedure during which the patient’s blood is passed through a device filled with immunosorbent. It is this substance that binds antibodies produced by the immune system, which provoke vascular damage.
• Plasmapheresis . A special method of purifying blood plasma using a special centrifuge. The procedure has a beneficial effect on the condition of blood vessels, reduces the severity of inflammation, and also prevents the risk of developing renal failure, and generally improves kidney function.

Diet

The main principle of the diet for vasculitis is to exclude from the diet foods that can cause or intensify allergies. For this purpose, the following products are excluded from the patient’s diet:

• milk and eggs;
• seafood;
• strawberries and wild strawberries;
• mango, bananas, citrus fruits;
• some vegetables - bell peppers, carrots, tomatoes;
• canned food;
• chocolate;
• baked goods;
• products to which the patient has an individual intolerance.

If vasculitis is accompanied by kidney damage, the doctor may recommend diet No. 7. For patients with severe disorders of the gastrointestinal tract, table No. 4 is recommended.

Preventive actions

They are necessary not only to prevent the occurrence of pathology, but also to speed up the recovery process, reduce the risk of complications, and also prolong periods of stable remission.

Basic preventive measures:

• minimizing stress;
• rational and proper nutrition with the exclusion of harmful foods from the diet;
• maintaining body weight within normal limits;
• refusal of any self-medication - taking medications without a doctor’s prescription;
• timely treatment of emerging diseases to avoid the transition of ailments to a chronic form;
• maintaining an active lifestyle;
• giving up bad habits - alcohol, smoking, etc.;
• eating a large amount of foods rich in vitamins, minerals, micro- and macroelements.

In children, treatment of vasculitis is carried out according to the same scheme and with the same drugs as in adults. After recovery or achieving stable remission, the child is registered with a rheumatologist and subsequently undergoes routine examinations by a specialist at least 3-6 times a year.

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

Diagnostics

Diagnosis of the disease can be difficult, especially in adults. Hemorrhagic vasculitis is often confused with other forms of vascular inflammation (see the “Disorders with similar symptoms” section of this article). Conventional laboratory tests are usually not accurate enough to diagnose the disease. The platelet count is usually normal, although the white blood cell count and sedimentation rate may be elevated.

The disorder is diagnosed by a combination of the presence of skin signs and/or joint tenderness in combination with a confirmed test for blood in the urine (urinalysis) and a skin biopsy that shows inflammation of the arterial and venous capillaries.

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.

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.

Basic principles of treatment

Treatment of vasculitis is prescribed depending on the activity of progression of the pathological process, the degree of damage to the skin, and an assessment of the systemic manifestation of the disease. If doctors have been able to establish the root cause of the pathology, therapy to eliminate the provoking factors must be prescribed.

Measures must be taken to stop inflammatory processes and speedy healing of damaged areas of the skin. In the photo you can see the different forms of manifestation of pathology. And the earlier treatment measures were taken, the greater the chance of completely clearing the skin, quickly curing inflammation, and eliminating associated symptoms. The rash, which manifests itself as hemorrhagic vasculitis, is periodically treated with Dimexide applications and lubricated with special ointments (Actovegin, Maxidex, Iruksol).

Read also: Hemorrhagic vasculitis in adults photos

To treat pathology that has spread to internal organs, drugs from the NSAID group (Diclofenac, Aspirin), anticoagulants and nicotinic acid are used. If therapy with these drugs is ineffective, doctors recommend undergoing plasmapheresis. Treatment of the disease is a comprehensive approach that eliminates the skin manifestations of the disease, cause and symptoms.

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.

Treatment of vasculitis

Treatment of hemorrhagic vasculitis usually does not require the use of any special medications, and its symptoms go away on their own after a short time. Anti-inflammatory drugs, such as ibuprofen, are often prescribed for joint pain, provided there is no inflammation in the kidneys. The results of some studies indicate the validity of the use of steroid drugs to speed up the treatment of abdominal pain in children, as well as eliminate their skin symptoms.

A number of medications, such as dapsone, can be used to treat the cutaneous form of this disease. If the kidneys or intestines are involved in the inflammatory process, treatment with steroids or other immunosuppressants may be required to reduce inflammation. In the renal form of the disease, high blood pressure sometimes develops. To eliminate hypertension, adjustment of salt intake and use of antihypertensive drugs may be required.

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.

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.

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.

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.

Signs of the disease

Let's consider the stages and first symptoms of hemorrhagic vasculitis. With the skin type, first of all, starting from the lower leg to the foot, rashes of a reddish hue appear, then they can become more active up to the gluteal region; when pressed, the spots do not disappear, unlike a common allergic reaction.

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With a mild course of the disease, the temperature does not rise, the rash covers small areas of the skin. The disease can manifest itself more aggressively, at first you will feel unwell, have a headache, have an upset stomach and the temperature will rise to 38C, and a rash will cover a large area of ​​the body.

Severe vasculitis is expressed by the symptoms described above, to which may be added a disorder of the heart muscle, kidney damage, severe pain in the joints, and even the possible appearance of vomit with blood.

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.