Vasculitis disease causes and treatment. Hemorrhagic vasculitis photo symptoms treatment. Causes of allergic vasculitis

All existing types of vasculitis (angiitis) are divided into two main classes: primary and secondary. The first class includes diseases in which inflammation of large and small blood vessels occurs without visible prerequisites, that is, the person did not have serious pathologies, led a normal lifestyle, did not take any specific medications, etc. It has not yet been possible to reliably determine the cause of primary vasculitis. Basically, assumptions are based on the genetic predisposition of the organism.

The second class includes pathologies that are a consequence of any previous causes or conditions. For example, an illness can manifest itself during or after an infectious disease, poisoning with biological poisons, overheating or hypothermia, mechanical or thermal damage to the skin, surgery, exposure to chemicals on the body, including serums for vaccination and various medications.

However, the classification of vasculitis is not limited only to the causes of development. There are quite a few subclasses of this disease, differing in location, specific symptoms and type of affected vessels:

• Arteritis - a disease that affects the walls of large arteries
• Arteriolitis - the walls of small arteries (arterioles) become inflamed
• Phlebitis - the membrane of veins and venules is destroyed
• Capillaritis - pathology extends to the capillaries
• Granulomatous eosinophilic vasculitis - vessels of different sizes are affected

Depending on the location, types of vasculitis are divided into:

• Segmental

In the segmental form of the disease, inflammatory processes are localized in one organ or in a specific part of the vascular system. Systemic vasculitis is classified as a group of diseases that simultaneously affect several parts of the body. In this case, the walls of the vessels are destroyed, as a result of which the blood supply decreases, ischemia develops, the structure of organs changes, and their functions are disrupted.

Depending on the classification of vasculitis, the method of treatment is selected. There are no identical therapeutic techniques for each individual case. That is why you cannot independently “classify” and treat vasculitis at home, and at the first suspicion of the disease you must immediately consult a doctor.

Essential cryoglobulinemic vasculitis

ECV or essential cryoglobulinemic vasculitis is recognized as the most mysterious and dangerous form of inflammation of small-caliber vessels (arterioles, capillaries, venules) located in the skin, liver or renal glomeruli. Only early diagnosis of the disease and adequate treatment measures provide a favorable prognosis for the patient’s life.

The etiology of cryoglobulinemic vasculitis has not been fully studied, and the pathogenesis is characterized by the formation of cryoglobulins IgM, IgA or IgC in the bloodstream, which during the immune response are converted into insoluble deposits on the walls of blood vessels.

Experts note that special protein compounds are present in the blood of many people, but cryoglobulinemic vasculitis does not occur in everyone. And in most cases of the disease, the hepatitis C virus acts as an antigen and stimulates the development of an immune response against one’s own tissues.

Symptoms of urticarial vasculitis

Inflammation of the walls of small-caliber vessels located in the epidermis is characterized as urticarial vasculitis. It is of an allergic etiology. Among the provocative main sources of pathology the following are noted:

• Acute and chronic infectious diseases;
• Food allergies;
• Hypersensitivity of the body to chemicals;
• Reaction to taking medications.

If we consider the signs of the disease, urticarial vasculitis (photo) is manifested by rashes on the skin in the form of raised blisters, which, after maturation, burst and hemorrhages form in their place. A constantly recurring rash creates a false clinical picture of urticaria. In addition to external manifestations, the symptoms of urticarial vasculitis are expressed in gastrointestinal dysfunction, joint swelling, in rare cases, inflammation of the mucous membrane of the eyes and damage to the central nervous system. To establish an accurate diagnosis, it is necessary to carry out differential diagnosis to exclude the possibility of a systemic disease.

The main direction of treatment of urticarial vasculitis is the relief of the inflammatory process that develops in the walls of blood vessels with the help of corticosteroid drugs.

Symptoms of cerebral vasculitis

Cerebral vasculitis is recognized as a less common form of inflammation of blood vessels, which affects the veins and arteries of the brain. The etiology of severe pathology of the central organ of the nervous system is not fully understood. The disease can develop either in isolation or against the background of infectious or allergic processes in the body.

Inflammation of blood vessels entails a narrowing of the lumen of blood tubular formations, which leads to poor nutrition of the brain and disruption of the main physiological functions of the body. Therefore, the symptoms of cerebral vasculitis are manifested by the following disorders of the patient’s condition:

• attacks of severe headache;
• disorganization of behavior and groundless psychoses;
• frequent sudden convulsions and loss of consciousness.

Vasculitis is an autoimmune disease

The concept of immune complex vasculitis is explained by the mechanism of development of this disease. Inflammation of the walls of blood vessels occurs due to an autoimmune reaction. The pathological process causes the destruction of cells of vascular structures and, as a consequence, the lack of normal blood supply to human organs, which leads to their functional failure.

In reality, autoimmune vasculitis has no actual known causative agents, but in most cases the patient is diagnosed with a hypersensitivity reaction. Under the influence of the allergen, the destruction of the walls of blood vessels begins, after which the connective tissue structures succumb to auto-aggression from the immune system. Inflammation of the walls of blood vessels occurs in people prone to food allergies or hypersensitivity to medications, as well as due to pathological disorders in the functioning of the immune system. Tissue destruction causes excess production of immunoglobulin E, which is an antibody that specifically binds to antigens.

Damage to small vessels of the subcutaneous tissue or toxic vasculitis develops due to prolonged intoxication of the body. Structural disorders of blood vessels are caused by individual hypersensitivity of the body to a certain class of chemicals or drugs that accumulate in the skin. Often this is a chronic form of the disease that does not require special therapy. It is enough just to exclude the etiological toxic-allergic factor from the patient’s daily life.

Experts associate the onset of the pathology, referred to as leukocytoclastic vasculitis, with hypothermia, acute respiratory viral infections, food or drug allergies. This chronic disease with periodic relapses is caused by inflammation and subsequent damage to the walls of blood vessels located in the dermis. Since this form is distinguished by a variety of morphological lesions on the skin (blisters, hemorrhages, pustules, erosions, ulcers and plaques), experts divide it into subtypes based on symptoms and localization of elements. But, in fact, they are all allergic vascular diseases of the skin.

Microbid Miescher-Storck or leukoclastic vasculitis turned out to be the most common type of skin vascular lesion. Multiple rashes in the form of spots with hemorrhages are mainly localized on the arms and legs, less often they spread over the body and affect the mucous membranes. In order to prevent frequent relapses of the disease, it is necessary to promptly diagnose and treat chronic focal infections.

Ulcerative-necrotizing vasculitis

In medical practice, necrotizing vasculitis is recognized as the most dangerous and severe type of inflammation of the walls of blood vessels located in the epidermis. The pathological state of the immune system causes necrosis of small areas of the skin due to poor blood supply.

The inflammatory process in the endothelium of the capillaries causes thrombosis of arterioles, which in turn is fraught with a decrease in the number of platelets in the blood and increased fragility of blood vessels. In the area of ​​the affected areas of blood tubular formations on the skin, foci of necrosis appear - black crusts. The precursors to scabs are hemorrhages or blisters, which progress to the stage of ulcers. Usually, ulcerative necrotic vasculitis develops acutely, with the appearance of multiple pathological elements on the lower extremities.

A complex diagnosis still has a favorable prognosis, provided there is no secondary infection at the sites of the ulcers. To avoid infection, the patient is advised to take antimicrobial drugs. The autoimmune factors that provoke it can also affect the vessels of internal organs, in which case the disease can be fatal. Only comprehensive Doppler diagnostics will help determine the location of the affected blood vessels, and it must be carried out immediately.

Nodular vasculitis

The main symptoms characterizing nodular vasculitis are pea-sized brown-bluish papules that rise above the skin, resembling nodules. After a short time, the tissues at the site of the rash die and ulcers form, which subsequently form hypertrophic scars. The nodules are localized symmetrically on the lower and upper extremities, usually in the area of ​​the extensor surfaces and around large joints. The course of the disease is protracted, with an acute onset and frequent remissions. The main symptoms are a sharp deterioration in the general condition of the body - chills, increased body temperature, joint pain.

It affects the blood tubular formations of the subcutaneous tissue. A local decrease in blood supply caused by the disease leads to the necrosis of areas of adipose tissue. Experts are confident that insoluble immune complexes with the presence of bacterial antigens play a key role in the development of pathology. Signs of the disease are symmetrical subcutaneous papules in the lower leg area and the development of ulcerated lesions in their place.

Independent inflammation of the walls of blood tubular formations is classified as primary vasculitis. Rheumatologists are mainly involved in the study and treatment of the disease. Although several specialized specialists take part in the diagnostic activities - dermatologists, cardiologists, neuropathologists and others.

Currently, the following subtypes of pathology are distinguished:

• Takayasu syndrome;
• temporal giant cell arteritis;
• polyarteritis nodosa;
• Churg-Strauss syndrome;
• Kawasaki syndrome;
• hemorrhagic vasculitis or Henoch-Schönlein disease;
• Wegener's granulomatosis;
• microscopic polyarteritis;
• cryoglobulinemic vasculitis.

A distinctive feature of these forms of inflammation is the caliber of blood vessels and the type of lesion. Due to this, pathogenesis has a variety of clinical signs and a variety of morphological changes in tissues and organs.

Wegener's vasculitis

A severe disease - granulomatosis or Wegener's vasculitis - affects the walls of small and medium-sized vessels located in the respiratory organs or kidneys. Inflammatory changes occur under the influence of antibody proteins, which, when reacting with neutrophilic leukocytes, contribute to their release of active chemical substances. Special substances form granulomas of different sizes and cause damage to the endothelium of the vascular wall. In the first stages of pathology development, Wegener's granulomatosis affects the larynx and nasal sinuses, then descends to the lungs, and the final stage of pathogenesis is kidney damage.

Hemorrhagic allergic purpura or Henoch-Schönlein vasculitis affects small-caliber blood vessels of internal organs and skin. Joints also suffer greatly from inflammation. At the moment, it is not possible to fully determine the exact cause of the development of the disease. Some experts associate the etiology of Henoch-Schönlein syndrome with chronic foci of infection, others are inclined to the genetic predisposition of the body to autoimmune pathology. The epidemiology of the disease is associated with childhood; it is most often diagnosed in preschool and school children. A feature of the symptoms are rashes that resemble dense small foci of hemorrhages under the skin, and they are localized symmetrically on the legs. May spread to the buttocks and abdomen. After the rash fades, pigment spots remain on the skin. The nature of the course of the disease can be acute, protracted and chronic with frequent relapses.

The disease, classified as Schamberg's vasculitis, has similar external signs that characterize Henoch-Schönlein hemorrhagic syndrome, namely rashes in the form of pinpoint hemorrhages. The pathology of the blood vessels of the dermis and subcutaneous tissue is of an autoimmune nature. Since patients do not experience discomfort and have no pain, most often they turn to the doctor when the pathology has reached the chronic stage. The skin process at that time is accompanied by the formation of pigment compactions. A rare chronic disease does not pose a threat to the patient’s life, but requires timely treatment, since the inflammatory process can spread to the blood vessels of internal organs.

Eosinophilic syndrome or Churg-Strauss vasculitis is a rare subtype of small-caliber vascular disease with granulomatous inflammation of the airways. In the early stages, it is almost impossible to make a diagnosis, so scientists are still intensively studying the disease. The most common criteria for diagnosing pathology are the presence of symptoms of bronchial asthma in the patient and high levels of eosinophils in the blood.

Often, Churg-Strauss vasculitis also affects the gastrointestinal tract. The syndrome is characterized by epigastric pain, diarrhea, and signs of pancreatitis. These symptoms indicate inflammation of the intestinal vessels. On the skin of patients, polymorphic rashes are observed. The prognosis of the disease will be favorable subject to adequate anti-inflammatory therapeutic actions.

Systemic ANCA vasculitis is a group of diseases characterized by the presence of antineutrophil cytoplasmic antibodies in the bloodstream. These include:

• microscopic polyangiitis;
• Churg-Strauss disease;
• Wegener's granulomatosis.

It is very difficult to diagnose these types of vascular inflammation at the beginning of pathogenesis. By affecting small-caliber vessels, associated vasculitis causes tissue necrosis, which is especially dangerous in the case of inflammation of the vascular glomeruli of the kidneys. The basis of therapy in such cases is anti-cytokine drugs, which do not affect the general functions of the immune system, but act directly on the cause of the acute autoimmune response. Rapid malignant progression of the disease without appropriate early treatment leads to death.

A disease that affects vessels of different sizes - infectious vasculitis, develops against the background of a chronic infection in the body, provoked by bacteria or viruses. The immune response to an antigen is expressed in the appearance of specific granuloma antibodies in the bloodstream. Due to their relatively large size and mass, they settle on the walls of blood vessels, causing an inflammatory process with subsequent destruction of the epithelium. Granulomas pose a particular danger to the capillaries of the nephron (renal corpuscles), since the patient gradually develops renal failure. To prevent the occurrence of pathology, it is necessary to treat focal infections in a timely manner.

Pulseless disease or Takayasu's vasculitis affects large-caliber vessels, mainly the inflammatory process affects the aorta, coronary arteries of the heart and blood branches of the pulmonary trunk.

The pathological process directly affects the elasticity of the vascular walls, leads to compaction of the inner lining of the blood tubular formations, which entails a narrowing of the lumen of the arteries. Atrophy of the muscle layer of the vessel occurs, its walls significantly weaken and expand. In principle, the disease is a fairly rare type of vascular inflammation, so its etiology is not fully understood.

The concept of granulomatous vasculitis unites a group of pathologies of small blood vessels with the formation of granulomas on epithelial tissue. The disease often affects the arterioles of the glomeruli of the kidneys, which causes a complication to develop - bilateral damage to the organ.

Antineutrophil cytoplasmic antibodies play a special role in the pathogenesis of the disease, but experts cannot yet understand the reason for their appearance. A group of antibodies provokes the release of various enzymes from cells, which can have a destructive effect on the vascular walls.

Allergic superficial vasculitis is characterized by a syndrome of individual hemorrhagic-pigmented skin rashes resulting from inflammation of the capillaries and precapillary arterioles. Pathologies in this group include:

• eczematoid purpura;
• Majocchi's disease;
• Gougerot-Blum syndrome;
• itchy purpura;
• angiodermatitis purpurosa pigmentosa and others.

Clinical types of inflammation of small vessels can be simultaneously diagnosed in one patient and most often are only stages of a pathological process that provokes fragility of capillaries and arterioles. The characteristic purple rash primarily occurs on the lower extremities and spreads throughout the body as the disease progresses.

In medical practice, secondary vasculitis is classified as a complication of focal infections or skin diseases. In some uncommon cases, inflammation of the walls of blood vessels develops against the background of a malignant tumor of internal organs. Therefore, in order to prevent pathology, doctors strongly recommend that at the first signs of exacerbation of bacterial or viral infections, promptly begin treatment in order to stop the inflammatory process in the body, without waiting for an immune response. It is noteworthy that even after chemotherapy for malignant cells, background symptoms disappear without specific therapy.

Systemic eosinophilic vasculitis is an autoimmune reaction of the body that provokes inflammation of the walls of arterioles and venules and their increased fragility. With this pathology, the perivascular space becomes saturated with eosinophils (a special type of leukocyte) and granulomas are formed, which settle on the walls of small vessels and affect epithelial tissue. This is preceded by allergic reactions, bacterial and viral infections. The pathology is quite rare, but with an acute course and pronounced intoxication of the body.

A peculiar reaction - Livedo's vasculitis is characterized by a bluish marble pattern on the skin, this occurs due to disturbances in the movement of blood in the vessels of the dermis. The pathology is classified into idiopathic and symptomatic forms. In the first case, marble mesh occurs regardless of vascular lesions, and the symptomatic type of the disease is caused by inflammation and fragility of the vascular wall. The etiology of Livedo's angiitis is not fully understood.

From this article you will learn: characteristics of vasculitis, what kind of disease it is, and how to treat it. Types of pathology, treatment methods.

Article publication date: 04/30/2017

Article updated date: 05/29/2019

Vasculitis - what is it? This is a group of diseases accompanied by inflammation and subsequent necrosis (death) of the vascular wall. This group of pathologies leads to a significant deterioration in blood circulation in the tissues surrounding the vessel. Different forms of these ailments have both characteristic and general symptoms (fever, weight loss, rash that does not disappear with pressure, joint pain). If left untreated, the primary lesion may spread and cause damage to other tissues or organs. Subsequently, the disease can lead to disability and even death.

So far, vasculitis remains insufficiently studied, and experts have not come to a consensus on the causes and mechanisms of inflammation, classification and treatment tactics. Now this disease is classified as a systemic connective tissue disease, and is treated by rheumatologists. If necessary, infectious disease specialists and dermatologists may be involved in therapy.

According to statistics, vasculitis affects both men and women equally often, and they are most often detected in children and the elderly. Every year the number of such rheumatologist patients increases, and experts believe that this increase in incidence is associated with the uncontrolled use of immune stimulants and environmental deterioration.

Types of vasculitis

A disease such as vasculitis is classified according to various parameters.

Based on the underlying cause, the following two forms of the disease are distinguished:

1. Primary - damage to the vascular wall is caused by unknown reasons, presumably it is provoked by disruptions in the immune system.
2. Secondary - damage to the vascular wall is a reaction to infections (tuberculosis, hepatitis, syphilis), systemic lesions (collagenosis, systemic lupus erythematosus, reactive arthritis), sarcoidosis, cancerous tumors, helminthic infestations or chemicals.

According to the severity of the course, vasculitis can be:

• mild degree - manifests itself only as a mild rash and does not cause a deterioration in the general condition;
• moderate degree - the patient has a profuse rash, joint pain, blood in the urine, and the general condition is moderate (loss of appetite, weakness);
• severe - the patient has a profuse rash, significant disorders in organs and joints, pulmonary and intestinal bleeding appears, kidney failure develops, and the general condition is severe.

Depending on the type of inflamed vessels, the following forms of vasculitis are distinguished:

• capillaritis – the capillary walls are inflamed;
• arteriolitis – the walls of the arterioles are inflamed;
• arteritis – arterial walls are inflamed;
• phlebitis – the venous walls are inflamed.

Depending on the location of the affected vessels, the following forms and types of vasculitis are distinguished:

General symptoms

The symptoms of vasculitis are extremely varied, and the most characteristic sign of these ailments is a rash on the skin. The severity of the signs of the disease and the general condition of the patient largely depend on the form and type of vasculitis. In severe cases and if left untreated, the disease can cause disability or death.

Rash

Signs of skin rashes due to vasculitis can be varied, but a number of them make it possible to distinguish this disease from others:

• the appearance of a rash is often associated with a past infection;
• the rash appears against the background of allergies, systemic, autoimmune or rheumatic diseases;
• the rash is located relatively symmetrically;
• the first elements of the rash appear on the legs (usually in the shins);
• elements of the rash are prone to hemorrhage, edema and necrosis;
• the rash is often represented by different elements that change their color, size and shape over time.

Patients with vasculitis may experience the following types of rashes:

1. Stains. They are red or pink rash elements that do not rise above the level of the skin. Caused by a rush of blood in response to inflammation.
2. Hemorrhagic purpura. Elements of rashes occur with significant damage to the vessel wall and hemorrhage. The rash may look like macules or telangiectasias that are purplish in color. The dimensions of elements with uneven contours can reach 3–10 mm. After some time, the rashes turn blue and then acquire a yellowish tint. After pressing, the rash does not disappear.
3. Hives. This rash is caused by an allergic reaction and is accompanied by burning, tingling and itching. It appears as pink or red bubbles with irregular contours.
4. Subcutaneous nodes. Such rash elements are caused by uncontrolled growth of connective tissue and epidermis. They appear as raised semicircular or flat nodules. Their size can vary from a few millimeters to 1–2 cm. They are painful, and over time, an area of ​​necrosis may appear in their center due to a significant circulatory disorder. Subsequently, the skin in this place of the nodule turns black and begins to be rejected.
5. Bubbles. Violations of the permeability of vascular walls in a certain area of ​​the bloodstream lead to the release of the liquid part of the blood under the skin. In such places bubbles larger than 5 mm appear. They are filled with transparent contents that may contain blood.
6. Ulcers and erosions. Subcutaneous nodes formed during vasculitis disintegrate over time, and in their place erosions (superficial damage) or deeper skin defects - ulcers - appear. When infected, they can fester.

Intoxication

Vasculitis is accompanied by circulatory disorders, leading to the formation of toxins, poisoning of the body and changes in metabolism. These processes cause the patient to experience the following symptoms:

• decreased exercise tolerance and weakness;
• loss of appetite and weight loss (up to 0.3–1 kg every month);
• frequent drowsiness;
• headaches (their intensity depends on the severity of the disease);
• temperature rise to 37.5–40 degrees (depending on the severity of the disease).

Nervous system lesions

Exposure to toxins and damage to nervous tissue caused by circulatory disorders and hemorrhages during vasculitis provoke the appearance of the following symptoms:

• sudden changes in psycho-emotional state;
• seizures;
• muscle weakness, incomplete paralysis of arms and legs,
• changes in sensitivity (usually of the “socks” and “gloves” type – that is, in these areas of the body);
• hemorrhages in the brain leading to the development.

Visual impairment

Insufficient blood supply and nutrition to the organs of vision observed with vasculitis can cause one- or two-sided damage to them:

• constantly progressive deterioration of vision (up to complete blindness);
• swelling and redness of the eye;
• feeling of difficulty moving the eyeball;
• protrusion of the eye.

Respiratory damage

The presence of an inflammatory reaction and impaired permeability of vascular walls during vasculitis leads to swelling and inflammation of different parts of the respiratory system. As a result, the following respiratory diseases may develop:

1. Prolonged runny nose.
2. Prolonged sinusitis and sinusitis.
3. Destruction of the bone walls of the maxillary sinus or nasal septum.
4. Prolonged bronchitis with an asthmatic component.
5. Bronchial asthma.
6. Pleurisy.
7. Pneumonia.
8. Bronchiectasis.

When the walls of blood vessels rupture, the patient develops bronchopulmonary bleeding of varying intensity.

Kidney damage

Many types of vasculitis lead to impaired nutrition and kidney function. Initially, the patient shows signs of decreased function, manifested by the following symptoms:

• lower back pain;
• fever;
• swelling;
• decreased volume of urine excreted;
• protein and blood in the urine.

Subsequently, the disturbance of blood circulation and nutrition in the renal tissues becomes more pronounced, and the tissues of these organs are affected more massively. Due to such changes, the patient develops acute and then chronic renal failure.

Joint damage

Vasculitis is accompanied by damage to the joint capsule, which is caused by the penetration of fluid into it, the development of inflammation and swelling. The knee joints are often the first to suffer, and the patient develops the following symptoms:

• redness, swelling and rash in the knee area;
• intense pain leading to difficulty in motor function.

After a few days, the inflammatory process spreads to neighboring joints and the pain in the knees becomes weaker. Typically, such joint damage resolves itself and does not lead to irreversible consequences.

Lesions of the digestive organs

Damage to the walls of the vessels of the mesentery and intestines causes circulatory problems and leads to hemorrhages. As a result, an inflammatory reaction develops and the patient with vasculitis develops the following symptoms:

• paroxysmal and intense abdominal pain, intensifying half an hour after eating;
• nausea and vomiting;
• frequent, watery stools (sometimes with blood inclusions).

Massive vascular damage can cause atrophy, gradual destruction and rupture of the intestinal wall. With such large-scale lesions, the patient develops peritonitis.

Treatment

Treatment of vasculitis should be comprehensive, and its tactics depend on the severity and type of disease. It can be performed on an outpatient basis or in a rheumatology department.

Indications for hospitalization of a patient with vasculitis are as follows:

1. Moderate or severe form.
2. Debut or exacerbation of the disease.
3. Pregnancy period.
4. Childhood.
5. Hemorrhagic vasculitis.

During the acute phase of the rash, patients are advised to remain in bed to help the rash disappear and stabilize blood circulation. A week after the appearance of the last elements of the rash, the regimen is gradually expanded.

Drug therapy

The choice of certain medications for the treatment of vasculitis can only be made by a doctor who takes into account the patient’s examination data, the severity and type of the disease. For a mild form, medications are prescribed for 2–3 months, for a moderate form - for about 6 months, and for a severe form - up to a year. If vasculitis is recurrent, then the patient is given therapy in courses of 4–6 months.

The following drugs can be used to treat vasculitis:

• non-steroidal anti-inflammatory drugs (Ortofen, Piroxicam, etc.) - eliminate inflammatory reactions, joint pain, swelling and rash;
• antiplatelet agents (Aspirin, Curantil, etc.) – thin the blood and prevent the formation of blood clots;
• anticoagulants (Heparin, etc.) – slow down blood clotting and prevent thrombus formation;
• enterosorbents (Nutriklinz, Thioverol, etc.) – bind toxins and bioactive substances formed during illness in the intestinal lumen;
• glucocorticosteroids (Prednisolone, etc.) – have an anti-inflammatory effect in severe vasculitis, suppress the production of antibodies;
• cytostatics (Azathioprine, Cyclophosphamide, etc.) - prescribed when glucocorticosteroids are ineffective and rapid progression of vasculitis, suppress the production of antibodies;
• antihistamines (Suprastin, Tavegil, etc.) - are prescribed only to children in the initial stages of vasculitis in the presence of drug or food allergies.

Non-drug therapy

In case of vasculitis, in order to cleanse the blood of substances that cause and aggravate the disease, the attending physician may recommend that the patient undergo various methods of gravitational blood surgery:

1. Immunosorption - venous blood is purified by passing through a device with an immunosorbent.
2. Hemosorption - blood is passed through a device with a sorbent that cleans it of antibodies, immune complexes and antigens.
3. Plasmapheresis - blood passes through a special device that purifies or replaces its plasma.

Hypoallergenic diet and nutrition

Vasculitis often predisposes to an allergic reaction, and to prevent such a complication, patients are advised to exclude the following foods from their diet:

• eggs;
• chocolate;
• red fruits and berries (especially wild strawberries);
• citrus;
• honey, royal jelly, pollen;
• products with flavor enhancers, stabilizers, dyes and non-natural flavors (sausages, flavored cheeses, pates, crackers, chips, etc.);
• mushrooms;
• pastries made from butter dough;
• canned food;
• coffee and strong tea;
• spicy, salty and fried foods;
• alcohol;
• individually intolerable products.

Herbal medicine, non-traditional and folk methods

Self-medication of vasculitis is unacceptable, since taking some medicinal herbs can provoke allergies and aggravate the course of the disease. In the absence of contraindications, a rheumatologist may recommend the following herbal remedies, non-traditional and folk methods:

• liquorice root;
• herbal mixtures based on calendula, horsetail, poplar buds, string, elderberry flowers, peppermint, yarrow, etc.;
• hirudotherapy (medicinal leeches).

Forecasts

The prognosis of this disease depends on its type and form, the age of the patient, the presence of complications and the timeliness of starting therapy. The five-year survival rate of patients with severe vasculitis, treatment of which was started immediately and with the use of glucocorticosteroids and cytostatics, is 90%. Without timely treatment, this figure is only 5% and leads to disability and death.

The following clinical cases can worsen the prognosis for vasculitis:

1. Kidney damage.
2. Damage to the central nervous system.
3. Damage to the coronary vessels and aorta.
4. Damage to the digestive organs.
5. Development of vasculitis after 50 years.

These facts mean that people with such diseases need constant medical supervision and strict adherence to all doctor’s recommendations. This attitude towards their health will help them avoid the development of serious complications.

Systemic vasculitis is a large group of diseases characterized by inflammation followed by destruction of the walls of small, medium and large vessels. As a result, ischemia of tissues and organs develops. Systemic vasculitis is characterized by a relapsing course, but it can also often worsen. Symptoms of illnesses largely depend on the location of the blood vessel, its caliber, as well as on the activity of the inflammatory process.

Clinicians refer to systemic vasculitis as:

• Kawasaki disease;
• Henoch-Schönlein syndrome. It is also known under another name - hemorrhagic vasculitis (one of the most common forms of pathology);
• Churg-Strauss syndrome;
• nonspecific aortoarteritis. In medicine, this disease is also known as Takayasu syndrome;
• cryoglobulinemic vasculitis;
• microscopic polyangiitis;
• Wegener's granulomatosis.

All of the above systemic vasculitis differ from each other in the localization of the inflammatory process and in the severity of its course. It is worth taking into account that some forms affect exclusively the skin and bring only mild discomfort to the patient, but others are extremely difficult and can even lead to death if they are not diagnosed in time and correct treatment is not carried out.

This group of ailments can be aggravated by damage to the visual apparatus, skin, heart, lungs, kidneys, joints, etc. An accurate diagnosis can only be made after several laboratory tests, visceral angiography and based on the results of a biopsy.

Systemic vasculitis is quite rare, but in recent years there has been a tendency to increase the prevalence of this pathology. Men from the middle age group are most often affected. But also some forms of the disease primarily affect women (Horton's disease, Takayasu's arteritis). Sometimes systemic vasculitis may occur in children.

Causes

The reasons for the progression of this group of diseases have not yet been fully studied by medicine. But there are already several theories directly related to the occurrence of systemic vasculitis. Clinicians are more inclined to believe that illnesses develop as a result of previously suffered severe viral diseases. As a result of this, the immune system begins to react abnormally to the infectious agent, which gives impetus to the progression of systemic vasculitis. In medicine, it is not uncommon for a person to develop vasculitis after suffering from viral hepatitis.

The second, most reliable theory, is based on the autoimmune nature of vasculitis. In this case, clinicians suggest that the development of the disease is facilitated by the fact that the immune system begins to recognize the cells that make up the blood vessels as foreign and begins to destroy them.

There is also an assumption that systemic vasculitis can be transmitted at the genetic level. It is believed that unfavorable environmental factors, as well as hereditary predisposition, greatly increase a person’s chances of developing vasculitis.

Classification

Classification by nature of occurrence:

• primary vasculitis;
• secondary vasculitis.

Primary vasculitis progress independently, without underlying ailments. They are characterized by an inflammatory process that affects the vascular walls. The development of this type of disease is associated with impaired immune reactivity. The inflammatory process penetrates all layers of the vascular wall. As a result, vessel occlusion and microcirculatory disorders develop, which, in turn, lead to ischemia. In the most severe clinical situations, tissue necrosis or even tissue necrosis may develop.

Secondary vasculitis is a disease that progresses against the background of existing pathology in the human body. Clinicians consider it as a complication or as a local optional manifestation.

The modern classification of vasculitis provides for their distribution into groups depending on the caliber of the affected vessels:

• damage to large vessels;
• damage to medium-sized vessels;
• damage to small vessels.

This classification is relevant and is used in medical institutions for accurate diagnosis and diagnosis.

Symptoms

Symptoms directly depend on the type of vessels that are affected. If this disease progresses, the patient experiences loss of appetite, fever, general weakness, weight loss, muscle and joint pain.

Also, the main symptoms can be supplemented with signs that are characteristic of various types of vasculitis.

• Takayasu arteritis. This type of vasculitis primarily affects women. “Attacks” the large arteries of the body. Main symptoms: headaches, feeling of cold or numbness in the lower extremities, complete absence of pulse in them or low pulse pressure, visual impairment, increased blood pressure;
• Behçet's disease. It primarily affects young men of working age. Main symptoms: the formation of ulcers, localized mainly on the genitals and in the mouth, damage to the skin, inflammation of the organs of vision;
• Buerger's disease. This disease is also known as thromboangiitis obliterans. It is characterized by the formation of small blood clots in the vessels of the extremities. Most often, such vasculitis occurs in representatives of the stronger sex. Symptoms: severe pain in the limbs, formation of ulcers on the fingers;
• giant cell arteritis. This type primarily affects people over 50 years of age. The pathological process begins in inflammation of the arteries of the head. As a result, the patient experiences severe headaches, pain in the cervical muscle structures, numbness of the tongue and pain when chewing food. Gradually, the symptoms intensify and the vessels of the visual apparatus are affected;
• Wegener's granulomatosis. With this type, the vessels of the nose, as well as the paranasal sinuses, kidneys and lungs are affected. At the early stage of development of the pathology, the symptoms are less pronounced, but as it progresses, difficult nasal breathing and nosebleeds appear;
• microscopic polyangiitis. This pathology affects small-caliber vessels in the skin, kidneys and lungs, and nerve endings. The patient gradually begins to lose weight, ulcers form on the skin, and fever develops. When the kidney vessels are damaged, inflammation of the glomeruli (glomeruli) is observed, which leads to a decrease in their function;
• cryoglobulinemic vasculitis. Its characteristic symptom is the appearance of purpura on the lower extremities. The person's joints also become inflamed. As the pathology progresses, they experience a feeling of numbness. This feature is due to the fact that vasculitis has already damaged the nerve endings;
• Henoch-Schönlein purpura. The pathology mainly affects young children, but it can also occur at any age. With this purpura, the vessels of the kidneys, skin, joints and intestines are affected. As a result, severe abdominal pain appears, purpura on the extremities, and urine mixed with blood may also be released;
• Churg-Strauss syndrome. This vasculitis exclusively affects the blood vessels of the lungs;
• polyarteritis nodosa. The disease can affect blood vessels located anywhere in the human body. The symptoms are quite extensive. Purpura, ulceration, deterioration of kidney function, and severe abdominal pain are noted.

Diagnostics

Diagnostics includes a complete examination of the patient by a neurologist, rheumatologist, nephrologist, as well as other specialized specialists. At the appointment, specialists clarify the history of life and the disease itself, and also conduct an examination. Diagnosis of systemic vasculitis includes laboratory and instrumental techniques.

Laboratory diagnostic methods:

• , including and ;
• blood test for CRP and RF.

Instrumental diagnostic methods:

• X-ray;
• echocardiography;
• angiographic examination;
• biopsy of affected tissue.

Treatment

Treatment of vasculitis is carried out only after a thorough diagnosis and clarification of what type of disease has affected the person. It largely depends on which system or organ in the body was affected, as well as on the stage of development of the pathology. process.

In the case of the progression of allergic vasculitis, in which only the skin is affected, in some situations doctors even do without the use of synthetic drugs. It is worth paying attention to the fact that the treatment is aimed at completely eliminating the inflammatory process that has affected the vascular wall, restoring the normal functioning of organs and systems, and also avoiding the development of possible severe complications.

In almost all cases, patients are prescribed hormonal therapy with glucocorticoids. This treatment tactic allows you to quite effectively and quickly eliminate inflammation in the blood vessels and reduce the abnormal activity of the immune system. GMPs are also used as an additional means. They are also necessary to eliminate inflammation and reduce pain. For hemorrhagic vasculitis, the course of treatment must include antimicrobial drugs. In order to improve blood circulation and prevent the formation of blood clots in the bloodstream, medications that thin the blood are prescribed.

If conservative treatment with hormonal drugs does not bring improvement, then chemotherapy with cytostatics is indicated.

In severe cases, blood purification is required during treatment. Then doctors resort to hemosorption and plasmapheresis.

Prevention

In order not to treat vasculitis, you should take measures to prevent this disease:

• complete rest;
• healthy sleep;
• hardening of the body;
• timely treatment of infectious diseases;
• proper nutrition.

It is extremely important to avoid stress on the immune system, which can lead to a malfunction of the entire immune system and can cause the progression of vasculitis.

As a result of inflammation of the walls of dermal vessels and their saturation with immune cells, skin vasculitis is formed. Their clinical manifestations are associated with damage to small and/or medium-sized cutaneous vessels. In half of the cases, the causes of the disease remain unknown; treatment depends on the severity of the inflammatory process.

Characteristics of the disease

Vasculitis is an inflammation of the blood vessels, which causes various symptoms of damage to the skin, and in some cases, internal organs. The walls of the arteries are saturated with immune cells - neutrophilic leukocytes, and undergo necrosis (death). At the same time, the permeability of the vascular wall increases, and hemorrhages occur around it.

The pathological process is often associated with the deposition on the inner surface of the arteries of circulating immune complexes, consisting of foreign substances that have entered the body (antigens) and protective antibodies. Such immune complexes, settling on the endothelium of the vessel, lead to its damage and inflammation. This development mechanism characterizes the most common form of pathology – allergic cutaneous vasculitis.

The disease can have varying degrees of severity - from minor damage to skin vessels to involvement of arteries in all internal organs with disruption of their function. Therefore, the assessment of systemic manifestations of the disease is of primary importance in treating patients.

Causes and types of pathology

Classification schemes are varied. They are based on various criteria, including the size of the affected vessels, microscopic appearance, external manifestations of the disease and its causes.

The following main types of cutaneous vasculitis are distinguished:

Polyarteritis nodosa

This is an inflammatory damage to medium and small arteries, accompanied by their necrosis, without damage to the kidneys, involvement of arterioles, small venous vessels, capillaries and the formation of glomerulonephritis.

Wegener's granulomatosis

Inflammatory process of the respiratory system with the formation of specific inflammatory nodules - granulomas, and vasculitis with necrosis of the vascular wall, affecting capillaries, small and medium-sized arteries and veins; Damage to the glomeruli of the kidneys often develops - nephritis.

Churg-Strauss syndrome

An inflammatory process with a predominance of an allergic component, affecting the respiratory system, accompanied by damage to small and medium-sized arteries, often associated with bronchial asthma.

Microscopic polyangiitis

Inflammation of a necrotizing nature with a slight immune-dependent component, often damaging capillaries, less often larger vessels; With this disease, necrotizing glomerulonephritis and lung damage are very often observed.

Henoch-Schönlein purpura

Pathology with deposition of immune complexes containing IgA class antibodies. The disease affects small vessels, including skin, kidney, intestinal, and causes pain or inflammation in the joints (cutaneous-articular vasculitis).

The process affects small vessels and is associated with the formation of special proteins in the blood - cryoglobulins; in this pathological process, the skin and kidney tissue are affected.

Cutaneous leukocytoclastic vasculitis (angiitis)

Limited inflammation of only the dermal vessels, without involvement of the kidneys and other organs.

Depending on the suspected cause of the disease, primary and secondary skin vasculitis are distinguished.

Since the etiological factors are varied, in clinical practice the most important classification is based on the size of the affected vessels. Signs of skin pathology appear when the smallest capillaries and medium-sized vessels are involved. Therefore, scientists divide all forms of vasculitis according to this criterion:

• predominant damage to capillary and smallest vessels: cutaneous leukoclastic, urticarial vasculitis and Henoch-Schönlein purpura;
• involvement of medium-sized arteries: polyarteritis nodosa;
• damage to both small and larger vessels: cryoglobulinemic variant, lesions in connective tissue diseases.

Manifestations of cutaneous vasculitis

External manifestations

The symptoms of cutaneous vasculitis are mainly determined by the diameter of the vessels involved in the process. When small vessels are involved, purpura occurs on the surface of the skin. Less commonly, a papular rash, urticaria, blisters, pinpoint petechiae, and erythema are formed.

With inflammation of medium-sized vessels, the following skin signs are observed:

• livedo reticularis;
• ulcerative-necrotic form of pathology;
• subcutaneous nodes;
• necrosis of the nail phalanges of the fingers.

Vasculitis with predominant damage to small vessels

Cutaneous leukocytoclastic angiitis

This is a diagnosis made by excluding other causes of pathology, accompanied by inflammation exclusively of the skin vessels. The onset of the disease is often associated with an acute infectious disease or the use of a new drug for the patient.

A typical sign is a limited lesion that goes away on its own after a few weeks or months. 10% develop a chronic or relapsing form of the disease. The damage looks like purpura, papules, vesicles, urticaria, and is located on areas of the skin exposed to friction.

Allergic (urticarial) vasculitis

This form occurs in 10% of patients with chronic. Differences between pathology and urticaria:

• the lesion persists for more than a day;
• in the clinic, it is not itching that predominates, but burning of the skin;
• the presence of purpura and darkening (hyperpigmentation) of the skin at the site of the lesion.

Most cases of the urticarial variant occur from an unknown cause, but others are caused by Sjögren's syndrome, lupus, serum sickness, hepatitis C, or malignancy. Also important is the long-term effect of harmful physical factors - solar radiation or cold air.

The urticarial variant is divided into 2 forms: with normal and low complement content. Complement is a system of serum proteins involved in immune responses. The low complement form is rare. It is accompanied by the development of arthritis, gastrointestinal tract, and obstructive pulmonary disease.

Urticarial form of angiitis

Henoch-Schönlein purpura

The disease (its synonym is hemorrhagic vasculitis) most often develops in childhood, often in boys 4-8 years old. It is accompanied by the appearance of purpura, noticeable to the touch, on the legs and buttocks, simultaneously with arthritis (the skin-articular form of hemorrhagic vasculitis), nephritis, and paroxysmal pain in the abdominal cavity. The disease often occurs acutely after nasopharyngeal infections. Histologically, immune complexes containing IgA are found in the tissues in and around the smallest vessels.

Henoch-Schönlein purpura

In most patients, the disease progresses favorably, but in 20% of patients kidney damage develops (cutaneous-visceral form of hemorrhagic vasculitis); this often happens when the pathology occurs in adulthood.

Damage to blood vessels and kidneys in hemorrhagic vasculitis

Vasculitis with predominant damage to medium vessels

This type includes polyarteritis nodosa. It has classic and skin varieties. The classic variant is necrotizing pathology of medium-sized arteries, not accompanied by glomerulonephritis. The disease is accompanied by muscle and joint pain, affects the skin, peripheral nerves, digestive organs, causes orchitis and congestive heart failure. The kidneys are also affected, but due to damage to medium-sized arteries, renal hypertension and renal failure occur, but not glomerulonephritis.

Skin lesions:

• purpura;
• livedo;
• ulcerative defects;
• subcutaneous nodules;
• in rare cases, gangrene of the skin on the fingertips.

In 5-7% of cases, polyarteritis nodosa is associated with viral hepatitis B.

The cutaneous form of polyarteritis nodosa occurs in 10% of cases of this disease, and is characterized only by damage to the outer covering of the body. This is the most common form of the disease in children, and is often accompanied by fever, muscle and joint pain. 20% of patients develop mononeuritis of the lower extremities.

Skin lesions are represented by painful nodules; livedo reticularis and gangrene of the nail phalanges are less common. This form of pathology is often associated with staphylococcal infection, HIV, parvovirus, hepatitis B. The disease can spontaneously stop or develop into a chronic recurrent form.

Vasculitis with damage to small and medium vessels of the skin

Cryoglobulinemic vasculitis

Cryoglobulins are proteins that precipitate when exposed to cold. They are divided into 3 subspecies. Type 1 consists of IgM antibodies, they cause blockage of blood vessels and are accompanied by blue discoloration of the extremities or Raynaud's phenomenon. Types 2 and 3 consist of antibodies directed against IgG. Cryoglobulinemic vasculitis develops in approximately 15% of patients with the presence of these proteins in the blood. This is believed to be due to their precipitation and activation of their destruction by the complement system.

Up to 75% of cases are associated with viral hepatitis C. Less commonly, autoimmune and lymphoproliferative processes are the cause.

Skin manifestations - purpura, less commonly Raynaud's phenomenon, bruising, skin nodules. Systemic signs are arthralgia, nephritis and peripheral neuropathy. It is accompanied by sensory disturbances and pain in the limbs.

Cryoglobulinemic vasculitis

Vasculitis in connective tissue diseases

Pathology can occur in patients with various autoimmune diseases, including rheumatoid arthritis, systemic lupus erythematosus, and Sjögren's syndrome. The rheumatoid variant develops in 5-15% of patients with rheumatoid arthritis, usually at the final stage of the disease and with a high content (titer) of rheumatoid factor.

Skin and peripheral nerves are affected. Well-defined purpura occurs, as well as infarctions and necrosis of soft tissue at the fingertips.

Vascular damage in lupus can affect any organ and usually occurs when the disease worsens. The disease is accompanied by hemorrhages, livedo reticularis, necrosis of the skin and superficial ulcers on it.

Pathology with Sjögren's syndrome is observed in 9-32% of patients and affects the skin and central nervous system. Severe systemic lesions correlate with the presence of cryoglobulinemia in such patients.

ANCA-associated vasculitis

Antineutrophil cytoplasmic antibodies (ANCA) are directed against one's own immune cells and are detected in many diseases. They are often associated with three cutaneous forms:

• Wegener's granulomatosis;
• Churg-Strauss syndrome;
• microscopic polyangiitis.

Detection of ANCA helps diagnose these diseases. These antibodies are involved in the development of the disease and are important in predicting relapse of the pathology. ANCA-associated conditions can affect any organ system and all have a chronic course with frequent relapses.

Ulcerative-necrotizing angiitis

Diagnostics

Recognition of the disease occurs in several stages

Confirmation of skin vasculitis

If the skin is involved in the pathological process, it is necessary to perform a skin biopsy in the affected areas that arose 12-24 hours before the study. This helps to detect neutrophilic infiltration of blood vessels and make a diagnosis.

If medium-sized arteries are suspected, a deeper (wedge) skin biopsy may be needed. Samples from nodules can be taken and usually provide more information than samples from the edges of a skin ulcer or livedo reticularis.

Diagnosis of systemic diseases

After confirming the diagnosis of skin inflammation, the doctor must determine its severity and damage to other organs. External examination reveals signs of damage to internal organs, for example:

• nasal congestion;
• hemoptysis;
• dyspnea;
• blood in the urine;
• abdominal pain;
• impaired sensitivity of the limbs;
• fever;
• weight loss;
• increased blood pressure.

If involvement of internal organs is suspected or if symptoms of the pathology persist for more than 6 weeks, additional studies are prescribed:

• detailed blood test;
• biochemical analysis with determination of urea levels, residual nitrogen, creatinine, liver tests;
• diagnosis of HIV infection, hepatitis B and C;
• determination of the level of complement, rheumatoid factor, antinuclear antibodies;
• electrophoresis of serum and urine proteins.

In case of severe damage to the lungs or kidneys, the following studies are prescribed:

• antineutrophil cytoplasmic antibodies (ANCA);
• X-ray of the lungs and paranasal sinuses.

If polyarteritis nodosa is suspected, angiography is performed to evaluate microaneurysms of the vessels of the internal organs.

With an in-depth study, other causes of pathology may become apparent, such as the effect of drugs, infectious agents, or the presence of a malignant tumor. A lung or kidney biopsy is often necessary to confirm the diagnosis.

This term combines a number of diseases accompanied by inflammation of the vascular walls. Such pathologies are characterized by a narrowing of blood vessels, which causes a deterioration in nutrition and oxygen supply to tissues. This is dangerous by disrupting the functioning of individual organs, up to their complete failure, which leads to disability and even death. Vasculitis is still not fully understood, so doctors do not identify the exact causes of development and methods of treating the disease. The treatment of such pathologies is carried out by rheumatologists, and sometimes by infectious disease specialists and dermatologists.

What is vasculitis

A group of autoimmune processes in which inflammation of the arterial or venous walls of human blood vessels occurs is vasculitis. As the disease progresses, it disrupts the blood flow to some organs, which is fraught with the development of complications. Taking into account the cause of development, vasculitis is divided into the following types:

1. Primary. Their etiology has not yet been clarified, but autoimmune disorders are considered the triggering factor.
2. Secondary. They develop against the background of other diseases - acute or chronic.

These factors, and especially a combination of several at once, can change the antigenic structure of the own walls of blood vessels. As a result, the immune system begins to perceive them as foreign. For this reason, the production of antibodies begins, damaging the vascular walls. This phenomenon triggers inflammatory and degenerative processes in target tissues. Secondary vasculitis is not only immunological disorders. Possible causes of vascular inflammation include the following:

• injuries of different localization and genesis;
• skin burns, including sunburns;
• overheating or hypothermia of the body;
• the body's individual reaction to vaccines or medications;
• contact with biological poisons or chemicals;
• hereditary predisposition;
• previous viral hepatitis;
• long-term fungal diseases, including candidiasis.

Signs

Vasculitis has many varieties, so the clinical picture and severity of symptoms of a particular form of the disease may differ. A typical symptom is hemorrhages in a small area with primary localization on the skin of different parts of the body. As the disease progresses, they appear in muscle tissue, areas of nerve endings and joint cavities. Doctors also identify several other common signs that indicate inflammation of the vascular walls:

• decreased visual acuity;
• bloody stools;
• stomach ache;
• joint pain, arthritis;
• rash;
• fever, headache;
• weight loss;
• sleep disorders;
• prolonged bronchitis, eosinophilic pneumonia, bronchial asthma;
• headache;
• pleurisy;
• neuropsychiatric disorders;
• seizures;
• changes in psycho-emotional state;
• pale skin;
• periodic loss of consciousness;
• nausea, vomiting;
• swelling in the temporal region.

Classification of vasculitis

In medicine, there are several classifications of this disease. One of the criteria for determining its types is the caliber of the vessels. Taking this factor into account, vasculitis disease is divided into the following forms:

1. Capillaritis. It involves damage to small vessels (capillaries). This may cause: Dego's disease, urticarial vasculitis (urticaria), Schamberg's purpura pigmentosa, Wegener's granulomatosis.
2. Arteriolitis. It is an inflammation of medium-sized vessels (arteries and arterioles). It manifests itself as lepromatous arteritis, polyarteritis nodosa, Kawasaki disease, familial Mediterranean fever, and striatal vasculopathy.
3. Arteritis. This is a lesion of large vessels (artery walls). These include giant cell arteritis, Takayasu's disease, Kogan's syndrome, sarcoidosis, and tropical aortitis.
4. Phlebitis. With this form of vasculitis, inflammation of the vein walls occurs.
5. Vasculopathy. With this disease, there are no clear signs of inflammatory cell infiltration of the vascular wall.

Due to its development, the disease is divided into two forms: primary (formed as an independent pathology) and secondary (formed against the background of other diseases). Depending on the degree of vascular damage, vasculitis occurs:

• mild degree - with a mild rash, burning and itching;
• moderate - with pronounced spots ranging in size from a few millimeters to 1-2 cm, weakness, loss of appetite, joint pain;
• severe - with numerous rashes, intestinal and pulmonary bleeding, significant changes in joints and internal organs.

Neurovasculitis

Neurovasculitis may develop under the influence of cold or nervous conditions. This disease is a reversible inflammation of the vessels of the extremities, associated with a disorder of reflex neurogenic reactions to irritation. The reason is the regular effect of temperatures in the range from -2 to +12 degrees against the background of high humidity or single frostbite.

Neurovasculitis occurs in adults over 25 years of age who work in damp, cold environments. Depending on the stage of the disease, a person experiences the following symptoms:

1. First. Accompanied by chilliness of the feet, pallor and cyanosis, but without swelling of the extremities.
2. Second. At the end of the working day, swelling appears, cyanosis and hyperesthesia of the skin, and pain in the feet when compressed are noted.
3. Third. Swelling at this stage does not go away, the pain becomes burning, ulcers develop, and ascending thrombophlebitis.

Rheumovasculitis

This is one of the types of rheumatoid arthritis. The mechanism of development of rheumovasculitis is associated with immune processes that take part in the development of arthritis. In almost all patients, such vascular inflammation is accompanied by general inflammatory manifestations: fever, severe weight loss. Clinical manifestations of rheumatoid arthritis are included in the group of extra-articular symptoms of rheumatoid arthritis. These include the following signs:

• peripheral gangrene;
• scleritis;
• mononeuritis;
• pericarditis;
• impaired lung function;
• damage to the skin of the periungual bed.

Perivasculitis

All vasculitis is classified into types depending on the location of inflammation. If the inner vascular layer is affected, then it is endovasculitis, if the middle one is mesovasculitis. When the tissue adjacent to a blood vessel becomes inflamed, the disease is called perivasculitis. With it, the arterial wall is completely destroyed. As a result, it ruptures, and inflammation of the outer layer of blood vessels and connective tissue begins.

As the disease progresses, it can lead to gangrene or necrosis. A more common type of perivasculitis is polyarteritis nodosa. It affects the following small and medium vessels:

• brain;
• renal;
• hepatic;
• coronoids;
• mesenteric.

Autoimmune vasculitis

This type of vasculitis has many different causes. One of the provoking factors is genetic predisposition. Vessels of certain sizes are affected by different types of autoimmune vasculitis:

• large – giant cell and Takayasu arteritis;
• medium - nodular and microscopic polyarteritis, Kawasaki disease, Wegener's disease, Behçet's disease.

The clinical picture is determined by the type of autoimmune vasculitis. Primary signs manifest themselves in skin damage: its sensitivity increases or decreases. Against this background, the following are observed: incomplete paralysis of the arms and legs, insomnia, chronic fatigue syndrome, fever, poor appetite. The disease has an undulating course, i.e. periods of remission are replaced by exacerbations. The latter occur mainly in the cold season. Depending on the type of illness, a person may experience the following symptoms of vasculitis:

1. Hematological. Causes chest pain, cough with copious sputum, shortness of breath, weakness, and kidney failure.
2. Rheumatoid. Accompanied by pain in the limbs, fainting, and mental disorders. May lead to stroke.
3. Takayasu arteritis. It is observed mainly in women 15-20 years old. Symptoms of the disease: elevated body temperature, headache, dizziness, weight loss. After 5-10 years, the pathology can lead to a heart attack.
4. Wegener's disease. It is indicated by cough, shortness of breath, prolonged rhinitis, sinusitis or sinusitis, runny nose and mucus mixed with blood from the nose, deformation of the saddle and nasal septum, impaired renal function, and protrusion of the eyeballs. Half of the patients develop conjunctivitis or optic nerve ischemia.
5. Behçet's disease. Accompanied by stomatitis, ulcers and erosions on the genitals, and inflammation of the eyes.

Hemorrhagic vasculitis

This type of vascular inflammation occurs more often in children than in adults. Boys aged 4-12 years are especially prone to developing this disease. The pathology is an aseptic (non-infectious) inflammation of the capillaries caused by the damaging effects of immune complexes. The main signs of the disease are hemorrhages (bleeding), disorders of blood circulation in small vessels and a violation of its intravascular coagulation. Depending on the clinical course, the pathology is:

• renal;
• abdominal (from the stomach);
• cutaneous;
• articular;
• combined.

The disease often develops after influenza, acute tonsillitis or scarlet fever. Causes may include drug allergies, hypothermia, and genetic predisposition. Distinctive signs of hemorrhagic vasculitis are:

• a purple rash on your legs, knees, hands, or stomach;
• pain in the ankle joints, leading to difficulty in motor function;
• stomach ache;
• kidney damage.

Allergic

The main cause of this type of vascular inflammation is an allergy to various external or internal factors: regular use of medications (sulfonamides, barbiturates, analgesics), chemical industry products, infections. Due to the reaction of the immune system to them, allergic vasculitis develops. It often occurs without involving internal organs in the pathological process. Depending on the caliber of the affected vessels, allergic vasculitis is divided into the following types:

1. Surface. It affects small venules and arteries of the skin, capillaries. Inflammation is manifested by hemosiderosis, Ruter's allergic arteriolitis, and nodular necrotizing vasculitis.
2. Deep. The pathological process involves arteries and veins of medium and large caliber, located in the subcutaneous fatty tissue and at its border with the dermis. The pathology manifests itself as acute and chronic erythema nodosum.

Necrotizing

This type of disease is rare, especially in children. The pathology is accompanied by extensive skin necrosis, fever, chills, weight loss and fatigue. Sometimes there are enlarged cervical lymph nodes, bilateral purulent conjunctivitis, swelling of the hands and feet. Necrotizing vasculitis develops more often as a complication of other forms of this disease. The following signs are observed on the skin:

• small papules on the skin - each bubble with transparent contents;
• redness of the skin;
• blueness of fingers or toes;
• non-healing wounds and ulcers;
• joint pain;
• numbness, tingling in the limbs;
• speech disorders;
• blood in urine or stool.

Diagnostics

The first step in diagnosing the disease is a careful examination of the patient by a doctor. The difficulty is that not all patients immediately turn to a rheumatologist, and the disease sometimes goes into remission, which creates the illusion of recovery. If vasculitis is suspected, the doctor prescribes a number of laboratory, instrumental and morphological studies:

1. Serological. The blood is examined for antineutrophil cytoplasmic antibodies, which helps to identify microscopic polyangiitis, Wegener's granulomatosis, and Churg-Strauss syndrome. Additionally, rheumatoid factor is examined to exclude rheumatic disease and cryoglobulins to differentiate vascular inflammation from Goodpasture's syndrome.
2. Visceral angiography. Before the procedure, a contrast agent is injected intravenously to study the blood flow through the vessels. The study itself is carried out using X-rays.
3. Doppler ultrasound. This procedure evaluates the intensity of blood flow in the vessels, which makes it possible to judge its violations.
4. Computed and magnetic resonance imaging. Help visualize changes in the structure of internal organs.
5. Detailed blood test. Inflammation is indicated by an increase in erythrocyte sedimentation rate and an increase in the number of leukocytes.
6. Analysis of urine. The pathology is confirmed by an excess of the normal amount of C-reactive protein in urine and the presence of blood elements.
7. Aortography. This is an x-ray examination of the aorta, based on obtaining an image after filling it with a contrast agent.

Treatment of vasculitis

The goal of treating the disease is to reduce the aggression of a person’s own immune system. This is necessary to achieve remission and prevent subsequent relapses. Additionally, measures are taken to prevent irreversible damage to tissues and organs. In general, the treatment regimen includes the following activities:

1. Taking medications. The patient is prescribed medications that reduce antibody synthesis and tissue sensitivity. Abnormal activity of the immune system is suppressed using glucocorticoids. If they do not have an effect, then chemotherapy with cytostatics is used. When using them, the prognosis is favorable: 90% of patients live after treatment with these drugs for more than 5 years. If the disease is bacterial in nature, the patient is prescribed antibiotics. For mild cases, non-steroidal anti-inflammatory drugs are indicated.
2. Carrying out extracorporeal hemocorrection. This includes blood purification techniques, such as hemosorption, plasmapheresis, and immunosorption.
3. Diet food. The diet is selected taking into account the reasons that led to vascular inflammation. Additionally, the possibility of developing allergies is excluded. The patient must follow a diet during an exacerbation and for some time after it.

During the acute stage, the patient is prescribed bed rest. This helps the skin rash disappear and blood circulation stabilize. A week after the first rash appears, a gradual expansion of the regimen begins. Treatment can be carried out at home or in a hospital. The main indications for hospitalization are moderate and severe forms of the disease. Inpatient therapy is also necessary in the following cases:

• hemorrhagic form of the disease;
• development of vascular inflammation during pregnancy;
• exacerbation of the disease or its first appearance;
• childhood.

Drug therapy

Certain medications for vasculitis are prescribed only by a doctor, taking into account the severity of the disease and the patient’s examination data. In case of a recurrent form of the disease, drugs must be taken in courses of 4-6 months . For mild cases, treatment lasts 8-12 weeks, for moderate cases – about a year.. Depending on the form of the pathology, the doctor may prescribe the following groups of drugs:

1. Nonsteroidal anti-inflammatory drugs: Ortofen, Piroxicam. Relieves joint pain, reduces swelling and the severity of skin rashes.
2. Antiplatelet agents: Aspirin, Curantil. They thin the blood by inhibiting platelet adhesion processes, which helps prevent the formation of blood clots.
3. Glucocorticosteroids: Prednisolone. It is the first-line drug of choice and has an immunosuppressive effect. Additionally, Prednisolone has a pronounced anti-inflammatory effect, so it is always prescribed for severe cases of the disease.
4. Anticoagulants: Heparin. By slowing down blood clotting, they eliminate the risk of developing blood clots.
5. Cytostatics: Azathioprine. Prescribed when corticosteroids are ineffective, there are contraindications to their use, or the rapid progression of the pathology. The function of Azathioprine is to suppress cellular immune responses.
6. Enterosorbents: Nutriclins, Thioverol. They bind and remove toxins formed during illness from the body.
7. Antihistamines: Suprastin, Tavegil. Their use is rational only at the initial stage of the disease in children if they have food or drug allergies.

Gravity blood surgery

This method of treatment includes techniques for purifying the blood of substances that cause the disease or aggravate its course. Among these procedures are the following:

1. Immunosorption. It involves passing venous blood through a device filled with immunosorbent. This is a special drug that binds antibodies and immune complexes that damage blood vessels.
2. Hemosorption. The blood is passed through a device with a sorbent, which also cleans it of antibodies, immune complexes and antibodies. This helps eliminate toxins, stimulate blood circulation and improve tissue nutrition.
3. Plasmapheresis. Blood from a peripheral vein is passed through a centrifuge, where the liquid is separated into red blood cells and plasma. The blood cells are then returned back into the bloodstream with donor plasma or plasma replacement solutions. This is how antibodies, antigens and immune complexes are removed from the blood.

Diet food

The diet for vasculitis should be hypoallergenic. This is necessary to exclude factors that provoke vascular inflammation. The patient should give up fried foods and switch to stewed foods. It is recommended to include fresh fruits and vegetables, dairy products, dried fruits and cereals in the menu. The following foods should be removed from your diet:

• citrus fruits – tangerines, oranges, lemons;
• red apples, wild strawberries, strawberries;
• baked goods;
• eggs;
• salty dishes;
• alcohol;
• strong tea;
• coffee, chocolate;
• chips, crackers, flavor enhancers;
• honey, pollen;
• mushrooms;
• industrial canned food.

Prevention of vasculitis

Since the primary form of the disease does not have a clearly defined cause, its prevention is difficult. In this case, it is only rational to strengthen the immune system by taking immunomodulator drugs. Additionally, it is necessary to harden the body with cold douches, swimming, and winter swimming. Proper nutrition combined with regular physical activity also helps strengthen the immune system.

Prevention of the secondary form of the disease has more principles. It is important to eliminate factors from your life that cause vasculitis. With this in mind, you need to adhere to the following recommendations:

• eliminate long-term stress;
• carry out sanitation of chronic foci of infection;
• Healthy food;
• maintain a sleep and rest schedule;
• Avoid prolonged exposure to allergens and environmental factors associated with occupational hazards on the body.

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