Nodular arteritis. Causes and risk factors. Video: periarteritis nodosa, mini-lecture

Periarteritis nodosa (polyarteritis) is a disease that manifests itself in the form of inflammation and necrotic damage to the walls of small and medium-sized blood vessels internal organs and limbs. This disease is always accompanied by arterial aneurysm and secondary pathologies internal systems and organs.

The most obvious and characteristic symptom of periarteritis is damage to the arteries of the kidneys. The circulatory system of the pulmonary circulation is not exposed to pathological effects, but sometimes damage to the bronchial artery is possible. Unlike other forms of vasculitis, this disease does not manifest itself in the form of granuloma formation, increased eosinophil levels or exacerbation of allergic reactions.

Pathogens

This disease is quite rare, and therefore doctors still do not have reliable data about the causes of its occurrence. According to statistics, every year from 0.5 to 1 case of polyarteritis is recorded for every 100 thousand people. Moreover, it is most often registered in men aged 49 years and older. Periarteritis nodosa in women occurs 2-5 times less frequently than in the stronger sex.

On this moment specialists know that the causative agent of this disease can be drug intolerance or the hepatitis B virus. About 100 types of medications have been registered that can provoke periarteritis nodosa. In medical practice, diseases of this kind are called drug-induced vasculitis. The development of this disease is explained by allergic reactions to medications.

Scientists have been studying for a long time viral origin periarteritis. In approximately 35% of patients, the presence in the blood of hepatitis B surface antigen and antibodies, immune structures that arise during the development of the virus, was detected.

Hepatitis C was detected in 3-7% of patients, but doctors cannot yet say for sure whether it is the causative agent of polyarteritis.

There is an assumption that some people have a genetic predisposition to the development of periarteritis nodosa; the symptoms of the disease are associated with certain characteristics in the DNA of patients. Although scientists have not yet been able to clearly prove this fact.

The development of this type of vasculitis occurs due to a malfunction of T-lymphocytes. Patients are found to have circulating immune complexes that include the Australian antigen. These formations can be observed mainly in blood vessels or kidney tissue.

Periarteritis nodosa is characterized by necrosis and inflammation of small and medium-sized arteries. Pathological processes, which manifest themselves in the gradual destruction of connective tissue, affect the entire vascular wall.

If the disease actively progresses, then cell infiltration begins in the affected areas, followed by inflammation. As a result, fibrosis of the vessel walls occurs, and aneurysms with a diameter of about 1 cm are formed.

Most often, periarteritis affects those places in the circulatory system where branching of blood vessels occurs. The process of damage to healthy tissue occurs segmentally, that is, between the areas of affected cells there are parts of the vascular walls that are not touched by the pathogenic process. This manifestation of the disease has a nodular structure, which is where its name comes from.

Symptoms of the disease

The clinical picture is manifested by a number of symptoms:

A prolonged fever occurs that does not subside even with treatment with a course of antibiotics.
The patient experiences a loss of strength, decreased mobility, and sudden weight loss.
The skin turns pale. Hands and feet take on a marbled appearance. Blood vessels begin to become clearly visible through the surface of the dermis, forming tree-like patterns on the body. Appears various kinds rash (erythermase, papular, hemorrhagic, urticarial). Sometimes necrotic formations are possible. In every fifth patient, when examining the skin of the legs, thighs or forearms, small painful lumps (arterial aneurysms or granulomas) are felt.
The patient begins to feel constant pain in the muscles, especially noticeable in the legs. The muscles are weak, quickly succumb to atrophy, and feel unhealthy to the touch. Joint pain appears, which can periodically migrate. Sometimes inflammation can develop in the knees, ankles, shoulders or elbows.
Cardiovascular syndrome appears. Against this background, angina pectoris and myocardial infarction occur. An attack of “silent heart attack” is possible, which has no obvious severe symptoms. In almost all cases, arterial hypertension is observed.
The vast majority of patients (75-95% of cases) have kidney damage due to vascular nephropathy. Against this background, microhematuria, cylindruria, and the rapid manifestation of renal failure. In some cases, if renal artery thrombosis occurs, a kidney infarction may occur. In this condition, the patient experiences severe pain in the lower back, his temperature rises, and there is blood in the urine. During ultrasound diagnostics, 60% of patients experience changes in the structure of renal vessels. Half of them show signs of arterial stenosis. A small percentage of patients have ruptured aneurysms, which is accompanied by the formation of perinephric hematomas.
Periarteritis nodosa also negatively affects the lungs. The patient begins to cough frequently, there is pain in the chest, and heaviness occurs when breathing. Various noises and wheezing are heard, the pulmonary pattern is distorted. In some cases, hemoptysis occurs.
The disease also affects the organs of the digestive system. IN different areas Intense pain occurs periodically in the abdomen. The anterior abdominal wall is in constant voltage. The patient experiences frequent belching, vomiting, and diarrhea with traces of blood and mucus. Pancreatic necrosis develops, intestinal ulcers break out, stomach bleeding. The liver suffers greatly, which leads to jaundice.
Affected nervous system. Possible stroke or epilepsy. The patient begins to feel burning pain in the limbs, general sensitivity at the same time it decreases.
The retina of the eye is irreversibly damaged. Aneurysms of the fundus vessels occur.
In a certain number of patients, the peripheral arteries of the arms and legs are affected. This leads to ischemia of the limbs, which can trigger the development of gangrene.
There are malfunctions endocrine system. Against the background of hepatitis B, 80% of men develop orchitis, epididymitis or similar diseases testicles. Functioning is impaired in both women and men thyroid gland and adrenal glands.

Forms of flow

There are several different forms of polyarteritis nodosa. They are distinguished depending on who, how the circulatory system is affected, as well as on the organs that suffer:

When the classic form of periarteritis nodosa develops, symptoms include fever, muscle pain, joint inflammation, skin rash, sudden weight loss. The clinical picture is represented by damage to the kidneys, heart, lungs, the development of abdominal syndrome, damage to both the peripheral and central nervous systems.
The second form is cutaneous thrombangitic, expressed by nodules and development hemorrhagic purpura. Most of the subcutaneous nodules are located along the extremities, on which necrotic and ulcerative formations constantly appear. The symptoms and treatment of this form of the disease differ significantly from other types of the disease, since cutaneous thrombangitic polyarteritis is not characterized by inflammation of the tissues of internal organs.

There is also a monoorgan type of the disease. He's characteristic pathological change blood vessels of an organ after a biopsy or removal of part of it.

Depending on the speed of the disease, doctors distinguish benign, slowly progressive, fulminant, acute and rapidly progressive polyarteritis.

Treatment options

Treatment of periarteritis nodosa usually occurs quite long time(from 2 to 3 years). Therapy is carried out in a complex form with multiple effects on all affected organs and systems. Usually, in order to effectively combat the disease, joint help from a rheumatologist, cardiologist, neurologist, pulmonologist, etc. is required. Depending on the speed and form of periarteritis nodosa, treatment is prescribed individually for each patient.

For therapy early forms For diseases without serious complications, doctors most often resort to corticosteroid treatment, repeating the course several times a year.

In addition, butadone or acetylsalicylic acid is prescribed.

Treating the chronic stage of this disease is much more difficult. To combat neuritis and muscle atrophy, massage, hydrotherapy and physical therapy are used. Extracorporeal hemocorrection reduces autoimmune manifestations and normalizes the chemical composition of the blood, which prevents the development of thrombosis.

In contact with

Another disease that is systemic in nature and has an autoimmune origin is. Provoking factors are stress, hypothermia, some viruses (hepatitis B virus is suspected).

Among adults, periarteritis nodosa is more common in men, in childhood and girls.

With this pathology, immune complexes settle on the walls of medium- and small-caliber arteries, and areas of their expansion (aneurysms) appear, followed by narrowings. Then normal blood flow slows down, shaped elements, fibrin settles and becomes fixed on the walls, blood clots form and proper blood circulation becomes impossible. Since such changes occur everywhere, the function of many organs and systems is disrupted.

The onset of the disease is usually characterized by persistent fever, resistant to conventional antipyretics. Increased weakness, sudden weight loss, and sweating. There is pain in the legs, very strong, along the vessels and nerves, in the calf muscle. , joint damage very rarely leads to any serious consequences. There is flabbiness and muscle atrophy.

Characteristic appearance skin - against a pale background, a translucent vascular pattern gives a marbled appearance; in some patients, dense, slightly painful nodules are felt along the arteries. A swelling may appear - from simple spotty, when its elements do not rise above the surface, to papular and hemorrhagic, with necrosis and hyperpigmentation.

Complications of periarteritis nodosa

Unfortunately, very often and dangerous manifestation Periarteritis nodosa is kidney damage. Protein appears and a small amount of red blood cells in urine, cylinders. Kidney failure develops rapidly, making treatment very difficult and significantly worsening the patient's condition.

The appearance of cough, shortness of breath, and hemoptysis indicates pulmonary involvement and is developing interstitial pneumonia, vasculitis. When thrombosis of a large vessel occurs pulmonary infarction. When listening to the patient, various wheezing is detected, the radiograph reveals focal, infiltrative shadows, increased pulmonary pattern, and subsequently signs of pneumosclerosis.

Damage coronary vessels heart (feeding the heart itself) leads to the development of angina pectoris, small focal infarctions. Each such zone of necrosis, then being replaced connective tissue, leads to the development of cardiosclerosis, disruption heart rate, circulatory failure. Malignant hypertension is characteristic.

Changes in the intestinal vessels are characterized by diffuse, severe pain in the abdomen, ulcerative-necrotic changes in its walls, sometimes complicated gastrointestinal bleeding and perforation of the walls with the development of peritonitis. May develop chronic pancreatitis. The liver and spleen are quite resistant to the disease and changes in them do not have a significant effect on the course of the disease.

From the nervous system, both its central part (bleeds in the brain, meningitis, encephalitis) and its peripheral part (neuritis) are affected.

The diagnosis is made by a combination of characteristic signs; the most accurate data will be provided by histological examination tissue obtained by biopsy.

The main drugs for the treatment of periarteritis nodosa are: steroid hormones(prednisolone and its analogues), in large doses, from the first days after diagnosis. In some cases, immunosuppressants (cyclophosphamide, azathioprine) are added to the hormones. After relief of the main manifestations, the dose of medication is gradually reduced, brought to the minimum possible, preventing exacerbations (maintenance). It is advisable to prescribe vitamins from time to time, especially ascorbic acid, potassium orotate. Depending on the symptoms that appear, symptomatic therapy is carried out.

Periarteritis nodosa is a disease that affects small and medium-sized vessels. IN official medicine The disease is called necrotizing vasculitis. Common names include periarteritis, Kussmaul-Mayer disease, and panarteritis. As the pathology develops, aneurysms form, repeated damage not only to tissues, but also to internal organs.

clear age restrictions Periarteritis nodosa does not, and even affects children. However, according to statistics, people 30–40 years old are most often at risk. The disease affects men much more often. It is impossible to say exactly what is the causative agent for the pathology. This can be either a virus or the body’s reaction to introduced synthetic drugs. medical supplies, serums, vaccines.

Etiology

Allergies come first:

food intolerance;
allergies to medications;
"cold" allergy.

As for medications, the most common allergens are antibiotics, drugs containing iodine, chlorpromazine, and B vitamins. This is why periarteritis nodosa can affect even children.

Also a factor that can cause the development of the disease is the hepatitis B virus. It is detected in 60% of patients with periarteritis.

In addition, the disease can develop due to:

hypothermia;
acute viral infection;
severe emotional distress or long stay in a stressful situation;
respiratory infection.

Symptoms

On initial stage The disease exhibits the following symptoms:

weakness;
sudden weight loss for no apparent reason;
headache that does not disappear even under the influence of painkillers;
fever;
in men, pain in the testicles, for no apparent reason;
abdominal pain.

As the disease progresses, and depending on the location of periarteritis, spots on the skin may appear of blue color like bruises. Typically, the disease can affect:

kidneys;
heart;
lungs;
central nervous system;
skin;
lungs;
gastrointestinal tract;
peripheral nervous system.

In other words, periarteritis nodosa can affect almost any internal organ and skin. Depending on the location of the disease, common list symptoms may be added.

Kidney damage

This subtype of pathology is observed most often. According to statistics, kidney damage is observed in 75% of patients. Diagnose at an early stage of development this pathology quite difficult because lab tests() may not show any changes. For more later stages development, changes in the functioning of the heart () and even deterioration of vision, even blindness, are possible. Without timely and adequate treatment the following complications develop:

aneurysm;
spicy;
rupture of intrarenal arteries;
kidney infarction;
acute thrombosis of renal vessels.

CNS lesions

At this localization to general list The following symptoms are added:

mental disorders;
seizures;
convulsions.

In some cases, memory problems may occur. In addition, the patient's sleep is disturbed. In severe cases without special drugs the patient cannot sleep.

Localization in the gastrointestinal tract

At the initial stage, diagnosing the disease is quite difficult, since the symptoms are very similar to food poisoning or basic indigestion. This same circumstance significantly slows down the treatment process, since the patient does not see a doctor on time. On initial stage the symptoms are the same as described above. As periarteritis develops, the following is observed:

bleeding;
almost constant abdominal pain;

The danger of this type of localization is that the pathology can develop to. The result is death. According to statistics, periarteritis nodosa of the gastrointestinal tract is observed in 50–60% of patients.

Lung damage

This subtype of pathology is observed quite rarely. Typically most common in women. The most characteristic symptoms of periarteritis nodosa are the following:

fever;
frequent cough with little sputum;
dyspnea;
unstable temperature.

In some cases, the pathology may be supplemented. IN mandatory diagnostics X-ray examination must be present.

Heart damage

Clinical periarteritis of the heart is practically not recognized. Most often, this happens through morphological studies in 70% of patients with the disease. With this pathology, minor disturbances in the heart rhythm and pain of a short, sharp nature may be observed. Without timely diagnosis and complex treatment of the disease can lead to.

Damage to the peripheral nervous system

It is this type of localization of periarteritis that is the most striking indicator of the disease at an early stage. In this case, the following occurs:

pain in the lower extremities;
difficulty moving;
amyotrophy;
decreased tendon reflexes.

The consequences of this type of pathology can be very different. Up to the loss of the ability to move independently.

Periarteritis nodosa of the skin

According to statistics, such skin damage is observed in almost half of patients. The “pattern” of blood vessels clearly appears on the skin, its color becomes purple. Most often, such signs of pathology are visible on the lower extremities. At the same time, there are no unpleasant physical sensations (itching, burning, peeling). However, the skin may become more vulnerable.

Clinical picture of the disease in children

As for children, the pathology much less often affects the central nervous system.

There is no exact cause of the disease in children. But, as medical practice shows, periarteritis nodosa can develop due to a virus and allergic reaction for scheduled injections.

The symptoms of the disease are almost the same as described above. In children early age The vessels of the small intestine are most often affected. In this case, all symptoms indicate acute. Therefore, correct treatment can begin only after a full diagnosis has been carried out.

Older children are characterized by the same localization of periarteritis nodosa as in adults - the gastrointestinal tract, nervous system, brain and kidneys.

The disease is especially dangerous for infants and young children (up to 7 years). Failure to see a doctor promptly can result in death. As a rule, the reason for this circumstance is:

heart attack;
renal failure;
hemorrhage in the brain.

Therefore, if you have the above symptoms in children, you should immediately consult a doctor.

Diagnostics

In the mandatory diagnosis of this disease, laboratory and instrumental studies. After a personal examination and identification of symptoms in the patient, he is prescribed laboratory tests - a general urine test and. In addition, the following is carried out:

biopsy muscle tissue;
(if there is a suspicion of gastrointestinal damage);
angiography of internal organs;
biopsy of the intestinal mucosa.

Only after a complete diagnosis can a course of treatment be prescribed.

Diagnosis of periarteritis on early stages very complex, since symptoms and tests may indicate another disease - kidney failure, heart attack or heart rhythm disorder, heart failure. Therefore, incorrect treatment may be prescribed initially.

Treatment

For periarteritis nodosa, the emphasis is on drug treatment. In case of significant complications, even a blood transfusion is possible. Since the disease primarily affects the kidneys, an organ transplant may be needed.

Concerning drug treatment, then the doctor prescribes the following synthetic medications:

corticosteroids;
ACE inhibitors;
antiviral drugs.

Be sure to adhere to special diet. Especially if gastrointestinal tract damage is diagnosed. Also, during the period of treatment you should completely stop drinking alcohol and smoking.

Forecast

If you do not apply for it in a timely manner medical care, do not start correct treatment, then even death is possible. The main reason for this is kidney failure, heart attack, and cerebral hemorrhage.

If left untreated, the average survival rate is 5%. Much depends on at what stage periarteritis is detected, how the disease develops and general state sick. As practice shows, a more optimistic prognosis from the course of treatment is observed with the progressive development of the disease, rather than fulminant development. In any case, the sooner the patient seeks medical help, the greater the chance of success. However, it is almost impossible to completely recover from periarteritis nodosa.

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Periarteritis nodosa: causes, symptoms/signs, diagnosis, treatment

Periarteritis nodosa (polyarteritis) - acute or chronic inflammation arterial wall, leading to the development of organ failure. This is systemic, caused by immune complex inflammation and damage to small and medium-sized arteries of the muscular-elastic type with the formation of aneurysms. The disease is manifested by fever, myalgia, arthralgia, severe intoxication and specific symptoms of damage to internal organs: kidneys, lungs, heart, skin, digestive tract.

IN inflammatory process all layers of the arterial wall are involved. Bacterial toxins, viruses and drugs are antigens that disrupt the body's immunological homeostasis. Tissue structures are damaged, including blood vessels. Angiogenic stimuli are formed, and an autoimmune reaction is formed. Cellular infiltration and fibrinous necrosis are the main pathogenetic stages of the disease, leading to thrombus formation, the formation of perivascular nodules, and infarction of internal organs.

Periarteritis nodosa has several common names - polyarteritis nodosa, disseminated angiitis, Kussmaul-Mayer disease. The disease was first identified as an independent nosological entity in the mid-19th century.

The pathology develops mainly in men aged 30-50 years, in children and the elderly.

photo: necrosis vascular wall with polyarteritis nodosa

Morphological forms of the disease:

Classic with the presence of renal-visceral or renal-polyneuritic symptoms - characterized by the rapid progression of kidney damage and the development of malignant hypertension,
Asthmatic,
Skin - has a benign course with persistent remissions and rare exacerbations,
Thrombangiitis - a slow course with symptoms of polyneuritis, dyscirculatory disorders in the extremities,
Monoorgan.

Causes

The etiology of the disease is not fully understood. There are 5 theories of the origin of the disease that are not confirmed by official medicine:

Syphilitic damage to the arteries,
Mechanical damage to blood vessels,
Severe intoxication with alcohol or other substances,
Acute bacterial infection
The phenomenon of hypersensitivity and local anaphylaxis.

Currently, the most relevant is the viral hypothesis, according to which periarteritis develops in individuals infected with viruses hepatitis B, HIV, influenza, rubella, cytomegalovirus infection, Epstein-Barr virus.

Predisposing factors are: immunization, allergies to certain medications, radiation, hypothermia, hereditary predisposition. Patients develop a delayed hypersensitivity reaction, forming antigen-antibody complexes that circulate in the blood and settle on the walls of the arteries, affecting them.

The risk group includes children with diathesis, food allergies, hypersensitivity to drugs, as well as adults with bronchial asthma, dermatitis, coronary disease heart, hypertension.

Clinic

Among common symptoms The most common and significant diseases are fever, arthralgia, myalgia, cachexia.

Persistent undulating fever does not respond to antibiotics and disappears after taking glucocorticosteroids.

Cachexia and progressive weight loss is characterized by a sharp loss of 30-40 kg in a short period of time.

Myalgia and arthralgia occur in the leg muscles and large joints and are accompanied by muscle weakness and atrophy.

In patients, the skin turns pale and acquires a marbled tint. A rash appears on the skin, and subcutaneous painful nodules form in the thighs, legs and forearms, located along large neurovascular bundles, singly or in small groups. These Clinical signs are symptoms of the classic form of pathology.

Specific symptoms are caused by damage to internal organs:

The acute course of the disease usually occurs in children and lasts about a month. and is characterized by rapid progression of the process with the development of myocardial infarction, disorders cerebral circulation or hypertensive crisis. In the subacute course, exacerbations are often replaced by periods of remission. This pathology lasts up to 6 months and occurs quite often. Chronic periarteritis nodosa progresses slowly and takes years to treat.

Periarteritis nodosa in children is characterized by progressive vascular damage and is complicated by the development of necrosis and gangrene of the extremities. The disease is most common among girls of all ages. age periods. The pathology develops acutely. In patients, body temperature rises to 39-40°C, profuse sweating, weakness, and malaise are noted. Persistent, tree-shaped bluish spots appear on the skin against a background of marbled pallor. Subcutaneous or intradermal nodules are usually the size of a bean or millet grain and are palpable along the large vessels. Painful, dense swelling located in the area large joints, later they either disappear or are replaced by foci of necrosis. Severe paroxysmal pain in the joints is accompanied by a burning or bursting sensation. Sick children sleep poorly at night and become restless and moody. In the absence of timely and adequate treatment, foci of necrosis spread to surrounding tissues.

Diagnostics

changes in renal vessels on angiography

Diagnosis of periarteritis nodosa includes collecting complaints and medical history, instrumental examination sick, laboratory research blood and urine.

Additional diagnostic methods:

Arteriography is a method of studying arteries by introducing contrast agent and conducting a series x-rays. In patients, arterial aneurysms and their occlusion are detected.
Biopsy of the calf muscles or abdominal wall allows us to identify characteristic changes vessels.
Microscopy of material taken from the patient's skin.
On plain radiograph lungs - clear deformation of the pulmonary pattern.
ECG and ultrasound of the heart show signs of cardiopathy.

Treatment

Treatment of periarteritis nodosa is complex, long-term and continuous. Rheumatologists, cardiologists and doctors of other related specialties select medications for each patient individually.

Patients with acute form pathologies recommend bed or semi-bed rest, gentle nutrition with a maximum of vitamins and microelements. After the exacerbation subsides, walking on fresh air, compliance correct mode day, psychotherapy.

Main groups of drugs:

Patients are prescribed corticosteroids in large doses. As the patients' condition improves, the dose is gradually reduced. Glucocorticosteroid hormones are most effective on early stages diseases. Long-term use Prednisolone, Dexamethasone, Triamcinolone helps get rid of arterial hypertension, retinopathy and renal failure. They have a pronounced anti-inflammatory and immunosuppressive effect.
Cytotoxic drugs help avoid the development of severe complications. Patients are prescribed Cyclophosphamide and Azathioprine.
Aminoquinoline derivatives - "Plaquenil", "Delagil" are prescribed after long-term use cytostatics.
Preparations of the pyrazolone series - Butadione, Aspirin - are taken together with Prednisolone. NSAIDs enhance the effect of glucocorticoids.
To correct hyperthrombocytosis and prevent disseminated intravascular coagulation syndrome, Trental and Curantil are prescribed. They normalize microcirculation and hyperbaric oxygenation.
If present on the skin infectious complications— purulent foci are treated with antibiotics.
Symptomatic treatment is painkillers and antihypertensive drugs. Vitamins, antihistamines, diuretics, and cardiac glycosides are administered parenterally.

After removal acute inflammation move on to physical therapy, massage, physiotherapy. IN severe cases resort to methods of extracorporeal hemocorrection - plasmapheresis, hemosorption.

Periarteritis nodosa - relatively rare systemic disease affecting small and medium-sized arteries muscular type. The disease belongs to polyetiological, but monopathogenetic pathologies.

Video: periarteritis nodosa, mini-lecture

Prevalence and causes of periarteritis nodosa. Symptoms and diagnosis of Periarteritis nodosa. Author's technologies for the treatment of periarteritis nodosa

Inclusion in the treatment of periarteritis nodosa technologies of extracorporeal hemocorrection give a chance:
V short time suppress clinical manifestations diseases

sanitize foci of chronic infections and thereby interrupt pathological stimulation immune system

increase sensitivity to traditional medications

reduce the dose of immunosuppressive drugs or completely stop these drugs

This is achieved by using:
technologies Autoplasma cryomodifications, allowing the removal of inflammatory mediators, circulating immune complexes, auto-aggressive antibodies, and coarse proteins from the body

technologies Extracorporeal Immunocorrection, capable of suppressing the activity of autoimmune processes without reducing the potential of the immunological defense of the body as a whole

technologies Extracorporeal Pharmacotherapy, making it possible to deliver drugs directly to the site of the pathological process

Periarteritis nodosa

(Polyarteritis nodosa)

Definition of Polyarteritis Nodosa

Periarteritis nodosa- This systemic necrotizing vasculitis– disease of medium and small caliber arteries without involvement of arterioles, capillaries and venules in the pathological process. Periarteritis nodosa was first described in 1866 by Kussmaul and Meyer.

The disease occurs with the formation of vascular aneurysms and secondary lesion organs and systems. Characteristic sign periarteritis nodosa– damage to the arteries of internal organs, especially the kidneys. The small circle vessels are not affected, but the bronchial arteries may be affected. Granulomas, eosinophilia and tendency to allergic diseases for the classic variant of periarteritis nodosa are uncharacteristic. Another common name for this disease is the term " polyarteritis nodosa" According to ICD-10 – M30 – Periarteritis nodosa and polyarteritis nodosa are related conditions.

Prevalence of periarteritis nodosa

Periarteritis nodosa– is not common, so its epidemiology has not been studied enough. 0.2 – 1 are registered annually new case diseases per 100 thousand population. The disease begins on average at 48 years of age. Men suffer from polyarteritis nodosa 3 to 5 times more often than women.

Causes of Periarteritis Nodosa

In development Periarteritis nodosa Two significant factors can be identified:

drug intolerance
persistence of hepatitis B virus

About 100 drugs are known that can be associated with the development of periarteritis nodosa. Drug-induced vasculitis most often develops in individuals with a history of allergic reactions.

Much attention is paid to viral infection. In 30-40% of patients with periarteritis nodosa, hepatitis B surface antigen (HBsAg), or immune complexes including HbsAg, as well as other hepatitis B antigens (HBeAg) and antibodies to the HBcAg antigen, which is formed during viral replication, are detected in the blood. The hepatitis C virus is found in 5% of patients with periarteritis nodosa, but its pathogenetic role has not yet been proven.

There are facts that indicate genetic predisposition to Periarteritis nodosa, although no connection has been established with a specific HLA antigen.

In the pathogenesis of periarteritis nodosa, the immune complex process and delayed-type hypersensitivity are important, in which lymphoid cells and macrophages play a leading role, and dysfunction of T-lymphocytes is noted. Circulating immune complexes (CIC), which include the Australian antigen, were found in patients with periarteritis nodosa. These immune complexes are found in blood vessels, kidneys and other tissues.

Pathomorphology of Periarteritis Nodosa

Periarteritis nodosa is characterized by inflammation and necrosis of small and medium-sized arteries of the muscular type, and the entire vessel wall is involved in the process; all phases of development of connective tissue damage occur in it.

At further development periarteritis nodosa, in the same areas, inflammatory cellular infiltration of all layers of the vessel wall appears, carried out by polymorphonuclear leukocytes with an admixture of eosinophils, which is replaced by infiltration of lymphocytes and plasma cells. The outcome of this process is fibrosis of the vessel wall, which leads to the formation of aneurysms with a diameter of up to 1 cm.

With periarteritis nodosa, the arteries are not affected along their entire length; branching sites are more often affected.

Since the process is segmental, there are intact sections of the vessel between the microaneurysms, which gives rise to formations such as nodules.

These changes in the vessel in periarteritis nodosa cause damage to the corresponding organ, and damage to the intima and its proliferation contribute to thrombosis. Violation of the patency of the vessel leads to infarctions in the corresponding areas, ranging from the kidneys to myocardial infarction. Throughout one vessel, all stages of the process can be encountered.

The inflammatory process with periarteritis nodosa can spread to nearby veins. The defeat of the venules is uncharacteristic and speaks in favor of micropolyangiitis or mixed vasculitis.

On acute stage periarteritis nodosa– all layers of the vascular wall and adjacent tissues are infiltrated by neutrophils, which causes proliferation of the intima.

On subacute and chronic stages of periarteritis nodosa- lymphocytes appear in the infiltrate. Fibrinoid necrosis of the vascular wall develops, the lumen of the vessel narrows, thrombosis, infarction of the tissue fed by the affected vessel, and hemorrhage are possible.

Healing– is accompanied by fibrosis and can lead to even greater narrowing of the lumen, up to occlusion.

With periarteritis nodosa, many organs may be involved in the process; the clinical and histological picture depends on the location of the affected vessels and the severity of ischemic tissue damage.

As already mentioned, with periarteritis nodosa, the pulmonary vessels are not affected, and the bronchial vessels are rarely affected, while with micropolyangiitis, capillaryitis often occurs in the lungs.

Kidney damage in periarteritis nodosa is characterized by arteritis without glomerulonephritis; micropolyangiitis, on the contrary, is characterized by glomerulonephritis. Patients with severe arterial hypertension usually have glomerulosclerosis, sometimes in combination with glomerulonephritis. Besides, in various organs discover the consequences of arterial hypertension.

Symptoms of Periarteritis Nodosa

Systematicity of the disease at periarteritis nodosa– can be traced from the very beginning of its clinical manifestation. The process begins gradually, less often acutely (after taking certain medications), with fever, myalgia, joint pain, skin rashes and losing weight. Sometimes the onset resembles polymyalgia rheumatica.

Fever- a symptom that occurs in the vast majority of patients with periarteritis nodosa. However, a prolonged isolated increase in temperature with periarteritis nodosa is rarely observed. At the onset of the disease, pronounced weight loss up to cachexia is characteristic. Significant loss body weight, as a rule, indicates high disease activity.

Arthritis, arthralgia and myalgia occur in 65–70% of patients with periarteritis nodosa. These symptoms are usually associated with inflammation of the vessels supplying blood to the striated muscles and joints. Intense pain is typical calf muscles, sometimes to the point of immobilization. Arthralgia occurs more often at the onset of periarteritis nodosa. In about a quarter of cases, there is transient, non-deforming arthritis affecting one or more joints.

Skin lesions observed in 40–45% of patients, and may be one of the first manifestations of periarteritis nodosa. Symptoms such as vascular papulopetechial purpura are typical, and less commonly, bullous and vesicular rashes. Subcutaneous nodules are rarely found.

Polyneuropathy with periarteritis nodosa – occurs in 50–60% of patients. This syndrome is one of the most common and early signs of the disease. Clinically, neuropathy manifests itself intense pain and paresthesias. Sometimes movement disorders precede sensory impairment.

Often noted headache. Described hyperkinetic syndrome, cerebral infarctions, hemorrhagic stroke, psychoses.

Kidney damage observed in 60–80% of patients with periarteritis nodosa. By modern ideas with classic periarteritis nodosa – predominates vascular type renal pathology.

Inflammatory changes usually affect interlobar arteries and rarely arterioles. It is believed that the development of glomerulonephritis is uncharacteristic of this disease and is observed mainly with microscopic angiitis.

Fast worsening renal failure usually associated with multiple renal infarctions. Most common symptoms kidney damage in classic periarteritis nodosa is moderate proteinuria (loss of protein

There is also an unrelated urinary infection leukocyturia. Arterial hypertension is registered in a third of patients with periarteritis nodosa.

Signs of defeat Of cardio-vascular system observed in 40% of patients with periarteritis nodosa. They are manifested by left ventricular hypertrophy, tachycardia, and cardiac arrhythmias. Coronaritis with periarteritis nodosa can lead to the development of angina pectoris and myocardial infarction.

Gastrointestinal lesions– a very characteristic and most severe form of organ pathology in periarteritis nodosa. Occurs in periarteritis nodosa in 44% of cases. Clinically, it most often manifests itself with symptoms such as nausea and vomiting. Abdominal pain is a symptom that is observed in about a third of patients with periarteritis nodosa; their development is usually caused by ischemia of the small intestine, less often of other parts.

Sometimes the disease manifests itself with clinical picture acute abdomen with symptoms of peritonitis, acute cholecystitis or appendicitis. Diffuse abdominal pain in combination with melena occurs with thrombosis of mesenteric vessels.

Genitals– affected by periarteritis nodosa – in 25% of cases. The lesion is manifested by pain in the scrotum, pain in the uterine appendages.

You can also point to liver, eye damage etc.

A local manifestation of periarteritis nodosa without systemic involvement is possible, although the presence of the latter is more typical.

Diagnosis of periarteritis nodosa

Laboratory changes in periarteritis nodosa are nonspecific.

Usually defined:

acceleration of ESR,
leukocytosis,
thrombocytosis,
increase in the concentration of CRP,
moderate normochromic anemia,
rarely eosinophilia, which is more characteristic of Churg-Strauss syndrome,
increased concentrations of alkaline phosphatase and liver enzymes during normal level bilirubin,
severe anemia, usually observed with uremia or bleeding,
a decrease in the C3 and C4 components of complement correlates with damage to the kidneys, skin and general disease activity,
HBsAG is detected in sera in 7-63% of patients,
A common but not pathognomonic sign of classic polyarteritis nodosa is aneurysms and stenosis of medium-sized arteries. The size of vascular aneurysms varies from 1 to 5 mm. They are predominantly localized in the arteries of the kidneys, mesentery, and liver and can disappear with effective therapy.

In patients with kidney damage during research urinary sediment take place:

moderate proteinuria,
hematuria.

Periarteritis nodosa should be excluded- in all patients with fever, weight loss and signs of multiple organ damage (vascular purpura, multiple mononeuritis, urinary syndrome).

To make a diagnosis of periarteritis nodosa, as a rule, along with clinical data, morphological confirmation is also necessary. Skin biopsy examination reveals lesions small vessels, but this sign is not specific enough and does not always correlate with systemic vascular damage.

Classification criteria for periarteritis nodosa

American College of Rheumatology:

Weight loss > 4 kg (loss of body weight since the onset of the disease by 4 kg or more, not related to dietary habits, etc.)
Livedo reticularis (patchy, reticular changes in the skin pattern on the limbs and trunk)
Testicular pain or tenderness (a feeling of pain or tenderness in the testicles not associated with infection, injury, etc.)
Myalgia, muscle weakness or soreness lower limbs(diffuse myalgia, excluding shoulder girdle or lumbar region, muscle weakness or soreness in the muscles of the lower extremities)
Mononeuritis or polyneuropathy (development of mononeuropathy, multiple mononeuropathy or polyneuropathy)
Diastolic pressure > 90 mm Hg.
Increased blood urea or creatinine (increased urea >40 mg/% or creatinine >15 mg/%, not associated with dehydration or impaired urine output)
Hepatitis B virus infection (presence of HBsAg or antibodies to hepatitis B virus in the blood serum)
Arteriographic changes (aneurysms or occlusions of visceral arteries detected by angiography, not associated with atherosclerosis, fibromascular dysplasia and other non-inflammatory diseases)
Biopsy: neutrophils in the wall of small and medium-sized arteries (histological changes indicating the presence of granulocytes or granulocytes and mononuclear cells in the arterial wall)

The presence of three or more of any criteria in a patient allows us to diagnose diagnosis of periarteritis nodosa with a sensitivity of 82.2% and specificity of 86.6%.

Course of periarteritis nodosa

The course of periarteritis nodosa is usually severe, since many vital organs are affected. important organs. The rate of development of the disease and progression of periarteritis nodosa may vary. In assessing the activity of the disease, in addition to clinical data, laboratory parameters are important, although they are nonspecific. Marked accelerated ESR, leukocytosis, eosinophilia, increase in γ-globulins, the number of CEC increases, the complement content decreases.

Prognosis for Periarteritis nodosa (polyarteritis nodosa)

Both with periarteritis nodosa and with micropolyangiitis in the absence of treatment the prognosis is extremely unfavorable. The disease progresses either lightning fast or with periodic exacerbations against the background of steady progression. Death occurs due to renal failure, gastrointestinal damage (especially intestinal infarction with perforation), cardiovascular pathology. Damage to the kidneys, heart and central nervous system is often aggravated by persistent arterial hypertension, which is also associated with late complications which are the cause of death. Without treatment, the five-year survival rate is 13%, at treatment with glucocorticoids - exceeds 40%.

Treatment of periarteritis nodosa

When treating periarteritis nodosa, a combination of GCS with cyclophosphamide or azothioprine is necessary. With an active process, cytostatics are used at a rate of 3-2 mg/kg body weight against a background of 20-30 mg of prednisolone. Having received a certain clinical effect, it is necessary to keep patients on a maintenance dose for a long time depending on the condition in the future.

In 90% of patients, long-term remission is achieved, which persists even after treatment is discontinued.

The most important thing is the correction of blood pressure by all by known means(peripheral vasodilators, β-blockers, saluretics, clonidine, etc.). Treatment of arterial hypertension in periarteritis nodosa can reduce damage to the kidneys, heart and central nervous system and the severity of the immediate and long-term complications associated with it.

For periarteritis nodosa, agents are used that improve peripheral circulation and have antiplatelet properties: chimes, trental.

Below is a generally accepted treatment regimen for patients with severe forms of systemic necrotizing vasculitis, including the use of extracorporeal hemocorrection technologies.

Application of extracorporeal hemocorrection technologies in the treatment of polyarteritis nodosa

Escalation therapy with active serious illness with an increase in creatinine > 500 mmol/l or with pulmonary hemorrhages: 7-10 plasmapheresis procedures over 14 days (removal of plasma in a volume of 60 ml/kg with its replacement with an equal volume of 4.5-5% human albumin) or pulse therapy with methylprednisolone (15 mg/kg/day) for 3 days. If the age of the patients

Induction therapy 4 – 6 months: cyclophosphamide 2 mg/kg/day for a month (maximum 150 mg/day); reduce the dose by 25 mg if the patient is > 60 years old. The leukocyte count should be > 4.0*10 9 /l. Prednisolone 1 mg/kg/day (maximum 80 mg/day); reduce every week to 10 mg/day for 6 months.

Maintenance therapy. Azathioprine 2 mg/kg/day. Prednisolone 5-10 mg/day.

When carrying HBsAg, the management of patients with periarteritis nodosa is generally no different from that of other patients. However, when markers of active replication of the hepatitis B virus are detected, the prescription of antiviral drugs (IF-α and vidarabine) in combination with medium doses of GCS and repeated plasmapheresis procedures is indicated, while the use of high doses of cytostatics is less advisable.

In addition to the above - from our point of view, it should be added that the use in the treatment of periarteritis nodosa of recently developed technologies of extracorporeal hemocorrection, which allows selective removal from the body of such pathogenicity factors as: circulating immune complexes and auto-aggressive antibodies, as well as technologies of extracorporeal immunopharmacotherapy, capable of changing the activity of the immune system systems in the required direction - makes it possible to significantly improve the results of treatment of this disease.

Besides - the use of modern technologies of extracorporeal hemocorrection, as a rule, allows for a significant reduction in course doses

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