Periarteritis nodosa: gradual damage to all organs. Periarteritis nodosa: symptoms with photos, causes, treatment and possible consequences

Periarteritis nodosa (polyarteritis), or Kussmaul-Meier disease (from Latin Periarteriitis) is a pathological condition of the vascular walls, in which their prolonged or acute inflammation is noted, which is characterized by necrotic formations on the stacks of blood vessels, and leads to insufficiency of the organs that feed the vessel.

This disease is a systemic vasculitis in which inflammation occurs due to immune complexes.

As a result, small and medium-sized arteries are affected with a violation of the normal structure of the walls (protrusion of the walls). This condition leads to impaired blood circulation, nutrition of individual organs and tissue areas.

Nodular disease, also referred to as polyarteritis, affects all layers of the arterial vessel wall.

The accumulation of negative particles in cells, with the formation of fibrous scars, are the main clinical stages of the pathology, which lead to the formation of blood clots, nodules and the death of tissues of internal organs.

In most cases, periarteritis nodosa manifests itself in males in the age category from thirty to fifty years, in children and the elderly.

What is special about the disease?

For the first time such a disease as periarteritis nodosa appeared in the second half of the nineteenth century.

This is vasculitis (inflammatory process of blood vessels) of a special form.

Especially the fact that such a disease affects small-sized arteries in organs of different localization.

The organs most commonly affected are listed below:

• The most common sites of injury in periarteritis nodosa are the vessels that nourish the kidneys and supply blood to the heart, brain, and liver, as well as the vessels of the mesentery of the intestine. The defeat of these vessels can lead to complications incompatible with life;
• Secondarily, the arterial vessels of the skeletal muscles, adrenal glands, pancreas and stomach suffer. The defeat of the arteries of these organs is fraught with violations of the functionality of the body;
• The last to be affected are large-sized vessels - the subclavian and carotid arteries. Their defeat is extremely rare.

Read the article about the functions and location of the carotid artery -

Pathology belongs to a little common, and is registered in one case per one million of the world's population. It is characteristic that the defeat of the male occurs twice as often as the female.

The final preventive measures, classification and treatment have not been worked out, since the disease is still being carefully studied.

Periarteritis nodosa is very dangerous and develops in the shortest possible time, or proceeds in a chronic form. In the absence of effective treatment, the survival rate is thirteen out of a hundred people affected by periarteritis nodosa.

Vascular necrosis in polyarteritis nodosa

Also, almost everyone who has had the disease remains disabled. Pathology is studied by specialists, being at the border of different disciplines, as it affects a large number of organs.

A final cure for the disease is not possible, but sometimes doctors achieve a stable period of remission.

If you find the first signs of nodular periarteritis, you need to contact a dermatologist, or a doctor for infectious diseases.

Further treatment and consultations of other specialists are based on which vessels are affected and which organ they nourished.

That is why the most effective treatment requires accurate differential diagnosis and consultation of a specialized specialist.

Renal involvement with periarteritis nodosa

Classification

The classification of periarteritis nodosa occurs according to three clinical forms and five variants of development with different life expectancy.

The clinic of the disease, today, implies three forms of periarteritis nodosa, shown in the table below.

Form of the disease	Characteristic
classical development	This form is characterized by an increase in body temperature, a sharp decline in weight, pain in the muscles and joints.
	This form affects mainly the gastrointestinal tract, nervous system, heart and kidneys.
Skin form	It is characterized by fever, weight loss, a sharp feeling of weakness, pain in the muscles.
	This form is characterized by the formation of nodules under the skin, located in the limbs, where ulcers and necrotic skin changes can progress.
Diverse development options	No characteristic signs are observed.
	Diagnostic criteria are determined after a skin biopsy, or surgery, with the study of the material in a specialized laboratory. Since the disease is not fully understood, simultaneous damage to other organs cannot be ruled out.

The division into types of periarteritis nodosa occurs according to the variants of its course, which affect life expectancy and the quality of daily life.

Type	Symptoms	Lifespan
benign course	Local skin inflammation, remission lasting up to five years	Does not differ in any way from the life expectancy of healthy people
Sluggishly developing	Inflammatory process of the peripheral nervous system and failure of blood circulation in the extremities	In the absence of burdens, life expectancy is more than ten years from the moment the first signs appear
Type with relapses	Relapses occur with a decrease in the dosage of drugs, infection, hypothermia and colds	If left untreated, life expectancy is about five years in thirteen percent of patients.
		With effective treatment, the survival rate rises to forty percent
Fleeting	Kidney pathologies and pathological high blood pressure	The patient lives until the moment of rupture, or complete narrowing of the kidney artery
lightning type	Pathological high blood pressure, kidney pathology, heart failure, intestinal ulcers, thrombosis of the intestinal arteries	Five months to one year

With a benign form of periarteritis nodosa and the effectiveness of treatment, a combination with professional activity and a normal quality of life is possible.

Other types of progression of periarteritis nodosa are characterized by disorders of individual organs that require constant and effective treatment.

Even the use of treatment leads to temporary disability, and then to the definition of disability.

Causes of nodular periarteritis

The causes of periarteritis nodosa have not been finally determined.

The main versions of the origin of pathology are:

• Infectious lesions of a bacterial nature;
• Explicit damage to the toxins of alcoholic beverages, or other substances;
• Syphilis;
• Allergic reactions, or vascular hypersensitivity;
• Mechanical damage to the vascular walls.

According to modern hypotheses, the most common version of the defeat of nodular periarteritis is the presence of HIV infection, influenza, rubella, hepatitis B and other viral infections in people.

Vessel changes in periarteritis nodosa - A - normal, B - autoimmune inflammation

Also, there are some risk factors, including:

• hypothermia;
• genetic disposition;
• Irradiation of the body;
• Allergic reactions of the body to certain types of drugs;
• Immune complexes that settle on the vascular walls, provoking their damage;
• Children with allergic reactions to food, high sensitivity to drugs, coronary heart attack, high blood pressure.

Symptoms of nodular periarteritis

The progression of the disease has pronounced symptoms, which are shown in the table below.

clinical sign	Characteristic
Increase in body temperature	Temperature indicators for periarteritis nodosa have characteristic elevated measures, which do not decrease during antibiotic treatment
Skin abnormalities	Paleness of the skin, vasodilation under the skin on the lower extremities, subcutaneous nodules that hurt
Rapid weight loss	A sharp decline is characterized by losses of up to thirty kilograms per month, with general weakness and apathy for activity.
Kidney pathologies	Narrowing of the artery of the kidney, its rapid wrinkling, the formation of protein, the death of kidney tissue and its insufficiency
Lung pathologies	Pain in the chest, heavy breathing, coughing, expectoration with blood, lung tissue death
Pathology of the eye structure	Protrusions, or seals in the walls of the vessels of the eye, deformation of the retina, leading to a decline in vision
Pathologies of the gastrointestinal tract	Pain in different parts of the stomach, vomiting, diarrhea, hemorrhages from the stomach, nausea, increased tone of the muscles of the anterior wall of the abdominal region, pancreatic tissue death, intestinal ulcers capable of rupture
Pathological condition of the arterial vessels of the legs	Insufficient blood supply to the toes, which is fraught with gangrene. Protrusions of vessels can be deformed with further internal hemorrhages
Endocrine Disorders	Inflammation caused by the production of antibodies to one's own cells, localized in the male testicles, malfunction of the adrenal glands and the thyroid gland
Damage to muscles and joints	Pain, general weakness, muscle atrophy, arteritis of large joints, possibly several at once
Cardiovascular syndrome	Inflammatory process in the vessels of the heart, leading to chest pain, irregular heartbeat, death of heart muscle tissue, mitral valve insufficiency, and high blood pressure
Pathologies of the nervous system	The defeat of one or many nerve endings, which are accompanied by burning pain, a feeling of weakness in the leg, inflammation of the meninges, seizures

Diagnosis of polyarteritis

Diagnosis occurs by comparing the symptoms shown in the table below.

When the three criteria match, then periarteritis nodosa is diagnosed.

Criteria for diagnosing periarteritis nodosa - A, B - livedo reticularis; C - characteristic changes in the biopsy (a tissue sample obtained during a biopsy)

Criteria for diagnosis	Characteristic
Hepatitis B	Fixing a given diagnosis in a patient, or antibodies produced against it
Pain in testicles	That are not caused by injuries or infectious diseases
Weight loss of more than four kilograms per month	Occurring without changing the diet and diet
Increase in blood pressure
Mesh livedo	It is a pathological condition of the skin, in which a bluish tint is recorded in it, due to translucent vessels overflowing with blood
Myalgia	Feeling of weakness and pain in the muscles of the legs. Pain in the shoulders and lower back is not observed
An increase in the level of urea, or creatine, in the blood	When exceeding creatine more than 133 mmol / l, urea - more than 14.4 mmol / l. In addition, there is no dehydration, or obstruction of the urinary tract.
Biopsy examination	When tissue is taken for a skin biopsy, it is noted that it is impregnated with various forms of leukocytes, granulocytes, etc.
Abnormalities in angiography	When studying vessels with a contrast agent, protrusions or overlaps of small-sized arterial vessels leading to individual organs are recorded. Atherosclerotic deposits are not detected, as well as other defects not associated with inflammation.
Mononeuritis (polyneuropathy)	Inflammation of one or many nerve endings in the peripheral nervous system is recorded

Treatment of nodular periarteritis

The protocol for the treatment of a disease such as periarteritis nodosa is a long and continuous therapy.

The course consists of a doctor of different specializations, depending on the localization of the affected vessels.

Patients affected by periarteritis nodosa need bed rest, proper nutrition with maximum saturation with vitamins and nutrients, as well as properly selected therapy.

The most common drugs used for periarteritis nodosa are:

• Glucocorticosteroids in high dosages (Prednisolone, Dexamethasone, Triamcinolone)- The drugs have the greatest effect in the initial stages of the development of the disease. Long-term use of them lowers blood pressure and eliminates kidney failure. Medicines help relieve inflammation and suppress the immune system;
• Pyrazolone medicines (Aspirin, Butadion)- are used together with non-steroidal anti-inflammatory drugs to enhance the effect of glucocorticosteroids;
• Cytotoxic drugs (Cyclophosphamide, Azathioprine)- help to avoid the progression of serious burdens;
• When correcting hyperthrombocytosis and preventing DIC (Trental, Curantil)– restore healthy local blood circulation;
• Antibiotics- are used for infectious lesions of the skin;
• To eliminate the symptoms - painkillers and high blood pressure. Also, the use of vitamin complexes, diuretics and cardiac glycosides is used.

After the acute inflammatory process subsides, physical therapy, massage, and physiotherapy are used. In extreme cases, plasmapheresis, or hemosorption, is used.

What are the preventive measures?

• Proper nutrition. The diet should contain a large amount of vitamins and nutrients so that the walls of the vessels remain elastic;
• Compliance with the daily routine. The working day should contain a sufficient amount of rest and healthy sleep;
• Maintaining water balance. Consume at least one and a half liters of clean drinking water per day;
• More active lifestyle. It is recommended to walk for at least one hour a day, as well as to engage in active sports;
• Timely treat infectious diseases;
• Carefully study the instructions for medicinal products, in order to avoid allergic reactions;
• Get a checkup once a year.

All actions are aimed at maintaining a healthy state of blood vessels.

Video: Polyarteritis nodosa.

What is the forecast?

Prediction for nodular periarteritis is not very favorable. Serious damage to blood vessels, which leads to dysfunction and death of organ tissues, can lead to death. According to statistics, remission is achieved in half of registered patients.

The effectiveness of the treatment of periarteritis nodosa depends on the correctness of its appointment, the timeliness of diagnosis and compliance with preventive recommendations.

If you find the slightest symptoms - go to the hospital for a full examination.

Do not self-medicate and be healthy!

Systemic vasculitis refers to diseases that are based on widespread necrotic-inflammatory changes in blood vessels. Vasculitis of varying severity can occur with such diffuse connective tissue diseases as rheumatoid arthritis (RA) And systemic lupus erythematosus (SLE), largely determining their course. First of all, independent, “primary” systemic vasculitis is considered, in which inflammatory changes in the vessels (primarily -) form the basis of the clinical and anatomical manifestations of the disease at all its stages.

Causes

The causes of these diseases are largely unknown. In the mechanisms of development, serious importance is attached to immune disorders. This point of view is consistent with such facts as the possibility of developing severe necrotizing vasculitis with serum sickness and other immunocomplex diseases. In patients with certain variants of systemic vasculitis, deposits of immunoglobulins and complement are found in the vascular wall. Finally, prednisone and immunosuppressants form the basis of therapy for systemic vasculitis.

Morphologically, fibrinoid changes and necrosis of the vascular walls are noted, in combination with cellular infiltration, which also extends perivascularly. The infiltrate in the acute stages consists of neutrophils; subsequently, monocytes, lymphocytes and plasma cells appear in it, and in some variants of vasculitis, also giant cells.

Edema and proliferation of the endothelium in combination with frequently observed perivascular hemorrhages lead to narrowing of the lumen of small-caliber vessels and, thereby, to ischemia of the corresponding organs. The most frequent localization in most systemic vasculitis is the arterial system, i.e., we are talking primarily about systemic arteritis. In some forms of vasculitis, other vessels suffer to no lesser extent.

Classification

There is no single classification of vasculitis. Usually, the authors tend to group vasculitis based on the commonality of morphological changes, clinical manifestations, and individual mechanisms of development. The caliber of predominantly affected vessels is extremely important, which is usually underestimated. The prognosis and course of the disease often depend on which of the largest arteries are involved in the process among all the vessels affected in this case.

For example, with polyarteritis nodosa, a patient may be affected by arterioles, small and medium-sized arteries, but only the defeat of the latter carries a real threat of myocardial infarction. In general, with vasculitis, the following levels of predominant damage are distinguished: the smallest vessels (arterioles, capillaries, venules), small arteries (intraorganic), medium arteries (coronary, mesenteric, hepatic, renal), large arteries (vertebral. temporal, aorta).

The classification of vasculitis proposed by D. Scott (1986) is very convenient:

1. Systemic necrotizing arteritis.

A. The group of polyarteritis nodosa, which primarily includes classic polyarteritis nodosa (periarteritis, as well as arteritis of a similar type in diffuse connective tissue diseases, in particular in RA and SLE).

B. A group of granulomatous arteritis, the main representatives of which are allergic (eosinophilic) granulomatous angiitis and Wegener's granulomatosis.

2. Immune vasculitis of small vessels, which includes hemorrhagic vasculitis (Schonlein-Henoch disease), essential mixed cryoglobulinemia and vasculitis of a similar type, sometimes found in patients with systemic lupus erythematosus, rheumatoid arthritis and other systemic rheumatic diseases.

3. Arteritis of large vessels. Examples of these diseases are giant cell arteritis (Horton's disease, temporal arteritis) and Takayasu's arteritis.

There are transitional forms between classic polyarteritis nodosa and granulomatous arteritis, which justifies their inclusion in the same qualifying category of systemic necrotizing arteritis. It must be borne in mind that vasculitis can exist both as an independent nosological entity, and as an obviously secondary syndrome in other rheumatic diseases, and different types of vasculitis may occur in one patient.

This disease, called (not quite accurately) until recently, periarteritis nodosa, is actually panarteritis, since it is characterized by the involvement of all layers of the vascular wall in the process. To the greatest extent, this disease is characterized by damage to small and medium-sized arteries. Histologically, there is inflammatory cell infiltration and fibrinoid necrosis of the adventitia, media, and endothelium. In the active stage of the disease, especially in the early stages, neutrophils predominate, and the abundance of "scraps" of cell nuclei from decaying cells draws attention.

In the later stages of the disease, mononuclear cells are also visible in the infiltrates, possibly a moderate amount of eosinophils. In rare cases, single giant cells are found. Upon completion of inflammation in a particular area of ​​the vessel, the inflammatory infiltrate disappears, fibrous replacement of the affected focus develops (especially the subendothelial layer) with the destruction of the internal elastic membrane. Simultaneous presence in one patient of various stages of arterial lesion is characteristic.

The formation of large perivascular nodules (aneurysms or inflammatory infiltrates), which gave the disease its first name, is actually a rarity. Deep damage to the arterial wall leads to both vascular thrombosis and the formation of aneurysms. The result of these processes are frequent heart attacks and hemorrhages, so typical of polyarteritis nodosa.

Polyarteritis is a rather rare disease. Its frequency is estimated at approximately 1:100,000, and the development of new cases of the disease - as 2-3:1,000,000. Men get sick 3 times more often than women. All age groups can be affected, but most often the disease begins between 40 and 60 years.

Etiology and pathogenesis

Views on the pathogenesis of polyarteritis are essentially the same- most authors believe that it is based on immune mechanisms. For the first time, such a point of view arose in the 20s due to the similarity of morphological vascular changes in this disease and typical immunopathological syndromes resulting from sensitization by a foreign protein, in particular, with the Arthus phenomenon and serum sickness. Of fundamental importance were the studies of A. Rich and J. Gregory (1943), who first obtained a model of nodular periarteritis in experiments on rabbits by sensitization with horse serum and sulfadiazine.

A. Rich (1942, 1945) also showed that in some patients the disease develops according to the type of immune reaction to the administration of therapeutic sera, sulfonamides and iodine preparations. In the future, ideas about the immune pathogenesis of nodular periarteritis were further strengthened. There have been many descriptions of the development of this disease after the use of drugs that have a sensitizing effect.

These include various chemotherapeutic drugs, antibiotics, vaccines, serums, halogens, etc. The increase in cases of polyarteritis over the past decades is associated precisely with the increasing use of new pharmacological agents. In a number of clinical observations, polyarteritis developed after bacterial or viral infections, which made it possible to raise the question of the etiological role of the corresponding antigens.

Later studies have shown that type III immune tissue damage is essential in the pathogenesis of polyarteritis - immune complex deposition antigen- antibody in the arterial walls. These complexes are capable of activating complement, resulting in direct tissue damage, as well as the formation of chemotactic substances that attract neutrophils to the lesion.

The latter phagocytize delayed immune complexes, resulting in the release of lysosomal enzymes capable of destroying the main membrane and the inner elastic membrane of the vascular wall. Complement activation and neutrophil infiltration play a critical role in the development of polyarteritis. Removal of complement components (C3 to C9) or neutrophils from the body of experimental animals prevents the development of vasculitis, despite the deposition of immune complexes in the vascular wall.

Of particular importance is the interaction of immune complexes and neutrophils with endothelial cells. The latter have receptors for the Fc fragment of human IgG and for the first complement component (C1q), which facilitates binding to immune complexes. Neutrophils are able to actively “stick” to the endothelium and, in the presence of complement, be cytotoxic due to the release of activated oxygen radicals. Endothelial cells produce a number of factors involved in blood coagulation and promote thrombosis in conditions of inflammation of the vascular wall.

Among the few specific antigens, the participation of which in the pathological process in polyarteritis has been objectively proven, the hepatitis B surface antigen (HBs-Ag) attracts special attention. In 1970 D. Gocke et al. described for the first time the deposition of HBs-Ag and IgM in the arterial wall of a patient with polyarteritis. Subsequently, this fact was confirmed in relation to the affected arteries of different caliber and localization.

The combination of these results with a decrease in serum complement concentration and an increase in circulating immune complexes led to the assumption that polyarteritis may be an immunocomplex disease in which HBs-Ag can be a trigger antigen, i.e., the main etiological factor. At the same time, it should not be considered that HBs-Ag plays a specific role in the development of polyarteritis. It is much more likely that it is one of the most common antigens that cause the development of the disease, but by no means the only possible etiological factor.

This is proved by the presence of patients with polyarteritis who have immune complexes (circulating and in the walls of arteries) that do not contain HBs-Ag. In most of these cases, a specific antigen cannot be established, but in some patients it is identified. There is a message about a patient with cancer and polyarteritis, whose immune complexes included a tumor antigen. It should also be borne in mind that many people are carriers of HBs-Ag and it does not cause a pathological process in them. Known persons with polyarteritis, in which the corresponding antigen was found in the blood, but the immune complexes were not registered.

In accordance with these data, it is most likely to consider polyarteritis as a predominantly immune complex disease caused by various antigens: bacterial, viral, drug, tumor, etc. At the same time, there is no reason to believe that the formation and deposition of immune complexes are the only possible mechanism for the development of the disease. It is highly likely that different pathogenetic pathways lead to systemic arterial inflammation with a very similar or even identical clinical picture.

In any case, the absence of deposits of immune complexes in the vessels of patients with polyarteritis is not uncommon. Interestingly, the experiment was able to show the possibility of developing both immune-complex viral vasculitis (in mice infected with lymphochoriomeningitis virus) and vasculitis due to direct viral damage to the endothelium and vascular intima (in equine viral arteritis). It is believed that in humans, direct damage to small arteries with their necrosis can be caused by rubella viruses and cytomegalovirus.

In the experiment, changes in the arteries, indistinguishable from the morphological signs of polyarteritis, are caused by various non-immune effects: high arterial hypertension induced by compression of the renal arteries; the introduction of deoxycorticosterone acetate together with sodium chloride; the appointment of an extract of the anterior pituitary gland against the background of unilateral nephrectomy. Apparently, the main common factor is the effect of a sharp increase in the tone of the arteries with possible necrotic changes in their walls.

It is noteworthy that antibodies to the components of the arterial walls in patients with polyarteritis could not be detected. There are descriptions of this disease in individuals with a congenital deficiency of the second complement component or a natural inhibitor of proteolytic enzymes (ai-antitrypsin). The association with specific histocompatibility antigens is not entirely clear; there are separate supervision about a combination with HLA-DR-7.

Thus, there is reason to believe that polyarteritis is a heterogeneous disease, in the development of which various causal and pathogenetic factors may be involved, among which the immunocomplex mechanism seems to be the most frequent and significant.

The clinical picture of polyarteritis is determined mainly by the localization, prevalence and degree of vascular damage. By themselves, the symptoms of the disease are not at all characteristic, but their combinations and considerable diversity are of significant diagnostic value. The onset of the disease is often acute, or at least fairly distinct. The gradual development of the disease is less common.

Among the first signs is characterized by an increase in body temperature from periodic rises, not exceeding 38 ° C, to hectic or constant, resembling in severe cases that of sepsis, miliary tuberculosis or typhoid fever. The similarity with these diseases is sometimes exacerbated by the general condition of patients with polyarteritis (especially in its most unfavorable course: prostration, blurred consciousness, dry coated tongue, shortness of breath, oliguria).

More than half of the patients have significant and rapid weight loss. Pain syndrome of various localization is very often expressed (primarily severe and prolonged pain in the muscles and joints, less often in the abdomen, in the region of the heart, head, etc.). Fever and myalgia are the most important clinical features to differentiate polyarteritis from rheumatoid and hemorrhagic vasculitis.

Particular manifestations of polyarteritis

Skin lesions occur in approximately ¼ of patients with prilearteritis, sometimes being one of the initial symptoms of the disease. The predominance of skin changes in some cases has led some authors to isolate predominantly the "skin form" of polyarteritis. The nature of skin pathology can be very different: urticaria, erythema multiforme, maculopapular rash, livedo reticularis with a pronounced pattern of "marbling" of the skin, small hemorrhages.

Very rarely, in the subcutaneous tissue, it is possible to palpate small nodules up to 5-5 mm in size (sometimes slightly painful or itchy), which are aneurysms of small or medium-sized arteries or granulomas localized in their outer shell. Relatively rare necrotic changes in the skin due to infarcts of the skin vessels and manifested by ulceration are characteristic. Usually they are multiple and small, but in the case of blockage of larger arteries, they are extensive and combined with peripheral gangrene of the tissues of the extremities. Bubble and bullous eruptions are extremely rare.

Skin changes(primarily ulcers, nodules, livedo) with a typical histological picture of polyarteritis sometimes occur without signs of a systemic disease or are combined with moderate muscular and neurological symptoms (but related only to the limb on which these skin changes are localized). In such patients, the complement level is normal, immune disorders and HB-Ag are not detected. These forms of the disease have a chronic favorable course, their prognosis is good. There are indications of their possible association with inflammatory bowel disease.

Changes in the locomotor system are associated primarily with the involvement of the vessels of the muscles and the synovial membrane of the joints in the process. Myalgia is a very common and early complaint; occur in 65-70% of patients; they are especially characteristic in the muscles of the legs. In about half of these cases, the symptoms of muscle involvement are not limited to pain (spontaneous and during movements), but also include tenderness on palpation, atrophy not associated with neuritis, focal seals, muscle weakness, i.e., clinical signs of myositis. These data explain the difficulties that sometimes arise in the differentiation of polyarteritis and dermatomyositis.

Joint lesions are also very common and sometimes the first symptoms of the disease. Arthralgia is characteristic of most patients. True arthritis is also not uncommon, which, against the background of a general serious condition and severe muscle pain, can fall out of sight. Reversible arthritis of large joints is characteristic, which does not lead to deformities and erosive bone changes. Arthritis is more common in the early stages of the disease, tends to affect the lower extremities, and may be asymmetrical. When analyzing synovial exudate, nonspecific inflammatory changes with moderate neutrophilic leukocytosis are detected. With the help of a biopsy of the synovial membrane, it is possible to establish vascular changes typical for polyarteritis.

Kidney damage in polyarteritis is observed in 80-85% of cases. Of greatest importance are changes in the glomerular vessels, which occur clinically, as a rule, according to the type of glomerulonephritis and, with significant severity, have an unfavorable prognostic value.

In the initial stages, the main signs of kidney damage are hematuria and proteinuria, including very moderate ones. Edema is uncharacteristic. Hypertension is common, but normal blood pressure does not rule out renal pathology. As the changes in the renal glomeruli progress, the filtration capacity of the kidneys decreases, creatininemia increases, and renal failure develops relatively quickly. This explains the high mortality of patients with polyarteritis from uremia - approximately 20-25% of all cases with a fatal outcome.

In addition to the glomerular changes characteristic of polyarteritis, others are described that are much less common and usually associated with damage to larger vessels. So, arterial thrombosis can be the cause of kidney infarction with the appearance of severe pain in the lumbar region and massive hematuria. Papillary necrosis is possible. Rupture of an aneurysm of a relatively large arterial trunk sometimes causes profuse, life-threatening hematuria. In other cases, extensive hemorrhages occur in the kidney tissue and surrounding tissue with the formation of a perirenal or retroperitoneal hematoma. The latter can simulate a pararenal abscess, given the high fever inherent in polyarteritis.

nephrotic syndrome is rare and usually results from renal vein thrombosis. Of the other lesions of the urinary system, the involvement of the vessels of the bladder (clinically manifested by dysuria) and the ureters is occasionally noted. In the latter case, with the help of ureterography, it is possible to establish a spasm of the ureters with the expansion of the overlying sections. Violation of the outflow of urine due to functional narrowing of the ureters threatens the development of hydronephrosis with a very likely secondary infection.

The cardiovascular system is affected in polyarteritis, according to post-mortem studies, in approximately 70% of patients. As the main cause of death, these lesions rank second, second only to renal pathology. The high frequency of involvement in the process of the arteries of the heart naturally leads to coronary insufficiency, the clinical manifestations of which are not always distinct, and sometimes completely absent. This feature of the disease is explained by the predominant lesion of small and medium-sized arteries, which in many patients is not accompanied by typical angina pains. In polyarteritis, small, painless myocardial infarctions have been described. In such cases, an electrocardiographic study is of great help.

Most often, congestive circulatory insufficiency develops, which is difficult to treat. Various rhythm and conduction disturbances are characteristic, especially supraventricular extrasystoles and tachycardia. Such rhythm disturbances may be the result of damage to the vessels of the sinoatrial node, which is very actively vascularized.

In some patients, the cause of death is ruptured aneurysms of the coronary vessels, which are observed even in infants. Contrary to previous ideas, exudative pericarditis is common - in almost 1/3 of patients. However, the effusion is usually small and clinically manifest little. Therefore, echocardiographic examination is indicated for all patients with polyarteritis. Endocarditis (usually of the mitral valve) is uncharacteristic of polyarteritis and is usually not diagnosed during life.

In the genesis of circulatory failure, in addition to coronary arteritis, hypertension is important, which occurs in most patients due to simultaneous kidney damage. The negative effect of elevated blood pressure is exacerbated by the fact that it usually develops relatively acutely, making it difficult to implement compensatory mechanisms. Myocardial hypertrophy (if it has time to develop) or its dilatation is to a large extent associated precisely with hypertension of renal origin.

The defeat of the venous trunks, sometimes proceeding according to the type of migrating phlebitis, and Raynaud's syndrome are rare manifestations of polyarteritis.

Pulmonary lesions are not very characteristic of classical polyarteritis, but are characteristic of other vasculitis. Nevertheless, even with true polyarteritis, in rare cases, arteritis of the branches of the pulmonary artery occurs with their thrombosis, hemoptysis, and diffuse intrapulmonary hemorrhages. Digestive and abdominal organs. The defeat of the vessels of the digestive tract occurs in almost half of the patients and gives severe clinical symptoms.

Localization of damages is various; most often, changes are found in the arteries of the small intestine and mesenteric, the stomach suffers less often. Thrombosis and rupture of the affected vessels are the cause of extremely characteristic pain syndrome and bleeding (intestinal, less often gastric) for polyarteritis. The combination of these features is of particular value for diagnosis. Arterial thrombosis can lead to necrosis of the intestinal walls with their rupture and the development of peritonitis.

The earliest and most frequent signs of involvement in the process of the gastrointestinal tract are abdominal pain, which can mimic an acute abdomen. Often in such cases, surgical intervention is performed, and often only after a biopsy of the removed tissue can a correct diagnosis be made. Of great diagnostic value is angiography, which allows to detect aneurysms of the arteries of the abdominal cavity (in particular, intestinal and hepatic) in most patients.

Abdominal pain may be due to ischemia or microinfarctions of the liver, spleen, or mesentery. Damage to the liver vessels, in addition to heart attacks and necrosis, is sometimes accompanied by proliferative reactions in the interstitial tissue of the organ, which contributes to the development of hematomegaly. A relatively common cause of the latter is circulatory failure due to heart damage.

Liver function tests are often abnormal. The spleen increases in a small number of patients, and even in persons with obvious splenic arteritis, an increase in the organ is not always found. Among the rare abdominal syndromes of polyarteritis, the syndrome of "abdominal toad" and acute pancreatitis deserve mention.

Sigidin Ya.A., Guseva N.G., Ivanova M.M.

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

Periarteritis nodosa (polyarteritis) is an acute or chronic inflammation of the arterial wall, leading to the development of organ failure. This is systemic, due to 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.

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

Polyarteritis nodosa has several common names - polyarteritis nodosa, disseminated angiitis, Kussmaul-Mayer disease. For the first time, the disease was singled out as an independent nosological unit in the middle of the 19th century.

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

image: necrosis of the vascular wall in polyarteritis nodosa

Morphological forms of the disease:

• Classical 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,
• Thromboangiitis - a slow course with symptoms, polyneuritis, dyscirculatory disorders in the extremities,
• Monoorgan.

Causes

The etiology of the disease has not been fully elucidated. There are 5 theories of the origin of the disease, not confirmed by official medicine:

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

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

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

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

Clinic

Among the general symptoms of the disease, the most common and significant 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 muscles of the legs and large joints and are accompanied by muscle weakness and atrophy.

In patients, the skin turns pale and acquire a marble hue. A rash appears on the skin, subcutaneous painful nodules form in the thighs, shins and forearms, located along the large neurovascular bundles singly or in small groups. These clinical signs are symptoms of the classic form of pathology.

Specific symptoms are due to damage to internal organs:

The acute course of the disease usually occurs in children, lasts about a month and is characterized by rapid progression of the process with the development of myocardial infarction, cerebrovascular accident or hypertensive crisis. In the subacute course, exacerbations are often followed by periods of remission. This pathology lasts up to 6 months and is quite common. Chronic nodular periarteritis slowly progresses and is treated for years.

Periarteritis nodosa in children is characterized by progressive vascular lesions and is complicated by the development of necrosis and gangrene of the extremities. The disease is most common among girls of all ages. Pathology develops rapidly. In patients, the body temperature rises to 39-40 ° C, profuse sweating, weakness, and malaise are noted. Persistent bluish spots of a tree-like form appear on the skin against the background of marble pallor. Subcutaneous or intradermal nodules are usually the size of beans or millet grains and are palpated along large vessels. Painful dense swellings are located in the area of ​​large joints, in the future they either disappear or are replaced by foci of necrosis. Severe paroxysmal pain in the joints is accompanied by a burning sensation or fullness. Sick children do not sleep well at night, become restless and capricious. In the absence of timely and adequate treatment, foci of necrosis spread to the surrounding tissues.

Diagnostics

changes in the vessels of the kidneys on angiography

Diagnosis of nodular periarteritis includes the collection of complaints and anamnesis of the disease, instrumental examination of the patient, laboratory blood and urine tests.

Additional diagnostic methods:

1. Arteriography is a method of examining the arteries by injecting a contrast agent and taking a series of x-rays. In patients, aneurysms of the artery and their occlusion are detected.
2. A biopsy of the muscles of the lower leg or abdominal wall reveals characteristic vascular changes.
3. Microscopy of material taken from the patient's skin.
4. Plain radiograph of the lungs shows a clear deformity of the lung pattern.
5. On the ECG and ultrasound of the heart - signs of cardiopathy.

Treatment

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

Patients with an acute form of pathology are recommended bed or semi-bed rest, sparing nutrition with a maximum of vitamins and microelements. After the exacerbation subsides, walking in the fresh air, observing the correct daily regimen, and psychotherapy are shown.

The main groups of drugs:

• Patients are prescribed high doses of corticosteroids. As the condition of patients improves, the dose is gradually reduced. Glucocorticosteroid hormones are most effective in the early stages of the disease. Long-term use of "Prednisolone", "Dexamethasone", "Triamcinolone" helps to get rid of arterial hypertension, retinopathy and kidney failure. They have a pronounced anti-inflammatory and immunosuppressive effect.
• Cytotoxic drugs avoid the development of severe complications. Patients are prescribed "Cyclophosphamide" and "Azathioprine".
• Aminoquinoline derivatives - "Plaquenil", "Delagil" are prescribed after prolonged use of cytostatics.
• Preparations of the pyrazolone series - "Butadion", "Aspirin" are taken together with "Prednisolone". NSAIDs enhance the effect of glucocorticoids.
• To correct hyperthrombocytosis and prevent DIC, Trental and Curantil are prescribed. They normalize microcirculation and hyperbaric oxygenation.
• In the presence of infectious complications on the skin - purulent foci, antibiotic therapy is performed.
• Symptomatic treatment - painkillers and antihypertensive drugs. Vitamins, antihistamines, diuretics, cardiac glycosides are administered parenterally.

After the removal of acute inflammation, they proceed to physiotherapy exercises, massage, physiotherapy. In severe cases, they resort to methods of extracorporeal hemocorrection - plasmapheresis, hemosorption.

Periarteritis nodosa is a relatively rare systemic disease that affects small and medium-sized muscular arteries. The disease belongs to polyetiological, but monopathogenetic pathologies.

Video: periarteritis nodosa, mini-lecture

PERIARTERITIS NODULOSIS (periarteritis nodosa; Greek peri around, about + arteritis; syn.: Kussmaul-Meyer disease, nodular panarteritis) is a disease of an allergic nature from the group of systemic vasculitis with secondary angiogenic damage to various organs and systems and severe vascular complications.

For P. at. characteristic is the defeat of small and medium-sized arteries of the muscular type with the formation of vascular aneurysms (“nodules”), thanks to which the disease got its name. Due to the fact that the inflammatory process is not limited to the outer shell (adventitia) of the vessel, but captures all layers of the vascular wall, P. at. it is more correct to call it nodular panarteritis, however, in the USSR the name nodular periarteritis, proposed in 1866 by A. Kussmaul and R. Maier, is retained. In Russia, the description of the first two cases of P. at. belongs to A.P. Langovoy (1883), who worked in the clinic of prof. A. A. Ostroumova. Intravital diagnosis of P. at. for the first time in our country, it was delivered in 1926 by E. M. Tareev during a biopsy of a subcutaneous nodule.

The generally accepted classification of P. at. does not exist. In the WHO classification (1980) P. at. classified as a systemic vascular disease. In the USA classification Zik is accepted (RM Zeek, 1953) in which allocate classical P. at., allergic P. at. with bronchial asthma and eosinophilia, hyperergic angiitis with drug and serum disease. Alrcon-Segovia (D.Alrcon-Segovia, 1977) proposes to distinguish between generalized classical P. at. immune genesis, hypersensitive limited (skin, renal, etc.) and allergic P. at. (eosinophilic angiitis).

P. at. are classified as rare diseases. Engelbert (O. Engelberth, 1962) found P. at 41,478 autopsies (1939-1956). in 0.13% of cases. However, there is a clear upward trend. According to sectional materials-tsy Johns Hop-kins (Baltimore, 1926-1942) on the basis of Rich's data (A. R. Rich), P.'s increase at is noted. from 1: 1600 to 1: 137. According to I. V. Vorobyov and V. E. Lyubomudrov, P. at. predominantly men aged 21-60 years are ill.

Etiology and pathogenesis

P.'s etiology at. not exactly established. The most common and generally accepted is the allergic theory, which explains the origin of the disease by a hyperergic reaction of blood vessels to various antigenic influences. P.'s emergence at is especially frequent. associated with the effects of various drugs (sulfonamides, penicillin, thiouracil, chlorpromazine, iodine, mercury) and the introduction of foreign sera. Since 1970 the question of a possibility of a virus etiology of P. at is discussed. In this case, the formation of immune complexes consisting of the surface antigen of serum hepatitis (HBsAg), antibodies to it and complement, and their deposition in the walls of blood vessels, is of decisive importance. Gouk (D. J. Gocke) et al., Gerber et al., P.'s cases are described at. after suffering HBsAg-positive hepatitis; at the same time, persistence of the antigen was observed, and sometimes immune complexes containing HBsAg were found in the wall of the affected arteries or muscles. According to Gouk, in 30-40% of cases of typical P. at. persistence of HBsAg.

P.'s pathogeny at. associated with immunopathological processes. Paronetto and Strauss (F. Paronetto, L. Strauss, 1962), using a fluorescent technique, established the presence of Y-globulin in the arterioles of a patient with periarteritis nodosa. Roger and Martin (J. Roge, E. Martin, 1965) by administering blood serum to animals from patients in the acute phase of P. at. received from them vascular changes characteristic of this disease; such changes were absent when the animals were administered the blood serum of convalescents.

pathological anatomy

Inflammatory changes at P. at. are found in vessels of different levels and different functions, appointments - in the arteries of all calibers, as well as in small and large veins, which indicates the systemic nature of the process. At the same time, the leading is the defeat of the arteries of the muscular and musculo-elastic type. Inflammatory changes in vessels at P. at. represent a manifestation of immediate or delayed hypersensitivity (see Allergy) with immunocomplex or immunocellular mechanisms. Often there is a combination of them, as a result of which vasculitis becomes mixed and their morphology reflects the complexity of the relationship between humoral and cellular allergic reactions. Immunopathological genesis of vasculitis at P. at. confirmed by immunofluorescence (see) and electron microscopy (see). In particular, at research of the material received from the patient P. at. in a kidney biopsy, it was shown that the exacerbation of the disease is accompanied by fixation on the basement membranes of vascular loops, in the mesangium and the parietal leaf of the glomeruli of the kidneys of immunoglobulins (IgG, IgA, IgM), the C3-fraction of complement and fibrin, giving a large-granular or focal-linear luminescence. Electron microscopically in the renal glomeruli of patients with P. at. subendothelial, mesangial, and occasionally subepithelial deposits of immune complexes, which include fibrin, are found. In the inflammatory and changed vessels at P. at. Immune complexes are detected that contain, along with IgG (Fig. 1, a) and complement, the surface antigen of the hepatitis B virus (Fig. 1, b).

At gistol, and gistokhim, studying of biopsy and autopsy material it is established that morfol, changes in arterial vessels at P. at. develop in a certain sequence: Mucoid swelling of the walls of blood vessels, fibrinoid changes up to necrosis, infiltrative-proliferative phenomena and sclerosis of the affected arteries. Mucoid swelling (see. Mucous degeneration) is caused by the decomposition of dissociating protein-polysaccharide complexes of the main substance of the connective tissue with the release of glycosaminoglycans, which leads to an increase in vascular permeability and to the hydration of the main substance of this tissue. Fibrinoid necrosis (see Fibrinoid transformation) develops after the plasma impregnation of the walls of the arteries and is characterized by the loss of amorphous and filamentous masses of fibrin in them.

Against the background of connective tissue disorganization, an inflammatory cellular reaction occurs, characterized by infiltration of vessel walls and the surrounding connective tissue by lymphocytes, macrophages, neutrophilic and eosinophilic leukocytes (Fig. 2, a) in various quantitative combinations. With great constancy among the cells of the infiltrate with such vasculitis, mast cells are also found. Acute arteritis often ends with the formation of aneurysms (Fig. 2b). As the exudative phenomena subside, the processes of proliferation and transformation of undifferentiated cellular elements of histiogenic and hematogenous origin develop, as a result of which an infiltrate - proliferate is formed in the walls of the affected arteries. Along with lymphocytes and macrophages, epithelioid cells, fibroblasts, and plasmocytes are detected in the infiltrate. With an increase in repair processes, fibroplastic cells become predominant in the infiltrate. As a result, there are sclerosis (see) and hyalinosis (see) of the walls of the arteries and arterioles.

Depending on the ratio of alterative, exudative or proliferative changes in the inflammatory reaction, arteritis can be destructive, destructive-productive and productive. Preferential localization patol. process in one of the shells of the vessel gives reason to talk about endo-, meso- and periarteritis. However, often with P. at. it is necessary to ascertain the defeat of all three shells; in such cases, the process is referred to as panarteritis. Since the disease is characterized by hron, recurrent course, Mucoid swelling, fibrinoid necrosis, infiltrative and proliferative reactions sometimes occur in sclerosed arteries. The heaviest consequence of an arteritis at P. at. is a progressive stenosis of the affected arteries. Quite often in pathologically changed vessels, especially with aneurysms, fresh, organized or organized (canalized) blood clots are found (see Thrombus).

Vasculitis at P. at. develops simultaneously or sequentially in many organs, although the vessels of the kidneys, heart, intestines, brain and nerve sheaths are most often affected. As a result of arteritis and thrombarteritis, local changes occur in various organs and tissues: hemorrhages, dystrophy and atrophy of parenchymal elements, focal necrotic and ulcerative processes, heart attacks and scars after them, sclerotic and cirrhotic phenomena. In the peripheral nerves, due to damage to the vasa sanguinea nervorum, signs of Wallerian degeneration are found with destruction of axons and myelin sheaths in combination with regenerative processes (see Wallerian rebirth).

Along with the arteritis described above, an important place in P.'s pathology at. occupies immune inflammation of microcirculation vessels. Thus, allergic microvasculitis underlies various variants of glomerulonephritis, alveolitis, pneumonitis), polyserositis. Inflammation of microcirculation vessels is essential in the occurrence of necrotic enteritis, severe manifestations of myocarditis, pancreatitis, hepatitis, and especially neuritis and myositis.

The prevalence of vasculitis in P. at. and the severity of secondary changes caused by them in organs and tissues vary significantly, which determines the clinical and anatomical polymorphism of the disease. In connection with the use for the treatment of patients with P. at. glucocorticosteroids and immunosuppressants, there is a predominance of productive forms of vasculitis.

Clinical picture

For P. at. the wedge, the symptoms complicating diagnosis is characteristic extreme polymorphism. The disease begins, as a rule, gradually with general symptoms. The most characteristic for P. at. are fever, progressive weight loss, and musculo-articular pain. Among the general symptoms in the first place in frequency (95-100%) is fever (see). Most patients have a fever of the wrong type, the temperature does not decrease with the use of antibiotics, but quickly disappears under the action of glucocorticosteroid hormones. Fever at the beginning of the disease is characterized by considerable persistence; when organ pathology appears, it, as a rule, does not resume.

Exhaustion is extremely characteristic, almost pathognomonic for P. at. in the acute phase of the disease (Kussmaul-Mayer chlorotic insanity). In some cases, the decrease in body weight reaches catastrophic figures (30-40 kg in a few months), and the degree of cachexia is higher than with oncol diseases.

The general manifestations of intoxication include such typical for P. at. symptoms such as tachycardia, not relieved by glycosides, and sweating.

Sometimes the illness begins with organ defeats, to-rye appear in some months and even years prior to emergence of system manifestations. With such "organ debuts" P. at. there may be bronchial asthma occurring with hypereosinophilia, repeated myocardial infarctions in young people, attacks of abdominal pain in combination with dyspeptic disorders.

Among the organ pathology inherent in P. at., there are five most common syndromes that determine the specifics of the wedge, the picture of the disease - renal, abdominal, cardiac, pulmonary and neurological.

Renal syndrome occurs in 75-90% of patients. The appearance of a wedge, signs of damage to the kidneys usually indicates a far advanced process. The most typical sign of damage to the kidneys in P. at. is arterial hypertension (see Arterial hypertension), in most cases stable, having a persistent course, sometimes galloping, with the development of severe retinopathy (see) and loss of vision. Moderate proteinuria (1.0-3.0 g per day), microhematuria are observed. Rarely, gross hematuria occurs. The development of nephrotic syndrome (proteinuria more than 3.0 g per day, peripheral edema) is extremely rare. Possible rupture of the aneurysmal dilated vessel of the kidney with the formation of a perirenal hematoma. The prognosis of renal syndrome is very serious: it can lead to the development of renal failure within 1-3 years.

Abdominal syndrome - second in frequency and prognostic significance; it is often observed at the onset of the disease. Abdominal syndrome is manifested by pain and dyspeptic disorders. Abdominal pains are, as a rule, diffuse in nature, they are constant, persistent, increasing in intensity. Of the dyspeptic disorders, diarrhea is the most pronounced (stool frequency up to 6-10 times a day); in the feces there is an admixture of blood and mucus. Characterized by anorexia (see), sometimes nausea, vomiting. Often develops peritonitis (see) as a result of perforation of ulcers or gangrene of the intestines, sometimes there is gastrointestinal bleeding (see). Damage of a liver at P. at. observed relatively rarely and is characterized by the development of heart attacks and rupture of aneurysms of intrahepatic vessels. Development hron, hepatitis or liver cirrhosis at P. at. it is caused hron, a virus infection (a virus of a serumal hepatitis) that is confirmed by data serol, a research and an intravital biopsy of body. Lesions of the pancreas and gallbladder are detected more often during post-mortem examination, however, in some patients with systemic manifestations of the disease, symptoms of pancreatitis (see) or cholecystitis (see) may be detected.

The cardiac syndrome is characterized mainly by coronaritis (see) and occurs in 50-70% of patients. Clinically, it is sometimes difficult to distinguish between heart damage caused by P. at., from secondary changes caused by severe arterial hypertension. Coronary disorders are often asymptomatic, not accompanied by anginal pain, even in the case of focal myocardial lesions. Small-focal myocardial infarctions (see) are more common than large-focal ones. Characterized by the development of a kind of angiogenic lesion of the heart according to the type of rapidly progressive cardiosclerosis (see) with rhythm disturbance, conduction and heart failure. The only cause of death is heart failure, which is rare. Possibility of defeat of an endocardium at P. at. is a controversial issue.

Pulmonary syndrome is observed in 30-45% of cases and may present with symptoms of bronchial asthma (see) with hypereosinophilia, eosinophilic pulmonary infiltrates of the Leffler type (see Leffler syndrome), vascular pneumonia, less often interstitial pulmonary fibrosis (see Pneumosclerosis) or pulmonary infarction ( cm.). With vascular pneumonia, cough is accompanied by the separation of a meager amount of mucous sputum, occasionally hemoptysis; fever, increasing signs of respiratory failure. X-ray in the lungs - a sharp increase in the vascular pattern, resembling a congestive lung, infiltration of the lung tissue mainly in the basal zones. An important diagnostic sign can be the low effectiveness of antibiotics and high - glucocorticosteroid therapy.

The neurological syndrome (lesion of the central and peripheral nervous system) is caused by systemic inflammatory changes in the walls of the vessels of the brain and the membranes of the nerves. Vessels c. n. With. are affected, according to sectional data, in 70% of cases, and the peripheral nervous system - in 12-25% of cases. Nevertheless, it is the defeat of the peripheral nervous system that is the most characteristic and diagnostically important symptom of P. at. Mononeuritis and asymmetric neuritis are observed (see Neuritis, Polyneuritis). Polyneuritis is occasionally noted as Landry's ascending paralysis (see Landry's ascending paralysis). Usually peripheral nevrol, disturbances develop gradually: at first there are pains and paresthesias in distal departments of arms and legs, then muscle weakness joins. Soreness of the muscles, more often the calf muscles, sensitivity disorders of the radicular and polyneuritic type are constantly observed.

Wedge, picture of defeat of c. n. With. polymorphic. The main manifestations can develop acutely with the onset of cerebral and focal symptoms of the type of stroke (see). Sometimes, along with focal symptoms, epileptic seizures are observed, occasionally status epilepticus (see Epilepsy), signs of subarachnoid and subdural hemorrhages. In some cases, lesions of the nervous system proceed under the guise of a dynamic violation of cerebral circulation (see Crises) or resemble slowly progressive cerebral atherosclerosis with increasing dementia (see Dementia). The cranial (cranial, T.) nerves are affected relatively rarely, predominantly visual and facial. Observed optic neuritis, transient decrease in visual acuity, narrowing of the retinal arteries, disc edema.

At 15-30% of patients with P. at. there are skin changes characterized by the presence of nodules along the vessels, various sizes of tree-like-branching bluish-red loops without peeling - livedo racemosa (see Livedo) or ulcerative necrotic changes. At P. at. gangrene of fingers and extremities, a necrosis of soft tissues can be observed, to-rye are caused by defeat of peripheral vessels.

Changes from eyes at P. at. meet seldom in the form of an iridocyclitis (see) or a vasculitis of vessels of a retina with thrombosis or microaneurysms.

Most often at P. at. combinations of the following syndromes are observed: renal-polyneuritic - kidney damage with high arterial hypertension in combination with asymmetric motor polyneuritis; renal-abdominal-cardiac - kidney damage with high arterial hypertension, abdominalgia with dyspeptic disorders, heart damage (coronaritis with diffuse and focal ECG changes) with progressive heart failure; pulmonary-cardio-renal, often starting in the form of hypereosinophilic asthma or pneumonitis; pulmonary polyneuritis, starting with bronchial asthma with further addition of polyneuritis.

Dominance in P.'s clinic at. one or more of these syndromes allows you to identify a number of clinical variants of the disease.

The classic (renal-polyneuritic, or polyvisceral) variant begins, as a rule, with fever, muscle and joint pain, and pronounced weight loss. In a wedge, a picture on the foreground defeat of kidneys with arterial hypertension, often malignant act; coronaritis, the combination of which with arterial hypertension leads to the rapid development of heart failure, as well as abdominal pain and polyneuritis. The defeat of the lungs proceeds according to the type of vascular pneumonia and is not so common. In blood serum sometimes find HBsAg, and at a biopsy of a liver - signs hron, active hepatitis or cirrhosis. The presence of immune complexes in the blood serum, organs and tissues is possible.

Asthmatic, or eosinophilic, variant is known abroad as allergic granulomatous angiitis or as Churg-Strauss syndrome (J. Churg, L. Strauss). Women get sick more often. The disease begins with attacks of bronchial asthma, it is often preceded by drug intolerance and other manifestations of allergies. Asthma is accompanied by high eosinophilia (50-85%) with leukocytosis of 20,000-35,000. Fever accelerated by ROE is possible. After 1-5 years, a generalization of the process occurs with the development of polyvisceral symptoms characteristic of the classical variant of P. at. In half of the cases, the disease proceeds without damage to the kidneys, limited to peripheral neuritis, skin changes or disorders from the gastrointestinal tract. tract. Along with the symptoms of bronchial asthma, eosinophilic infiltrates are often observed in the lungs.

The cutaneous variant of periarteritis nodosa is manifested by skin lesions with the formation of typical nodules along vessels the size of millet and lentils, painful on palpation. Skin manifestations are usually accompanied by myalgia, fever, accelerated ESR, anemia, leukocytosis. In some cases, along with nodular formations, livedo (livedo racemosa), necrosis of soft tissues, mucous membranes appear, and gangrene of the extremities develops. Skin P. at. rarely complicated by damage to internal organs.

The monoorganic variant is extremely rare and manifests itself in the defeat of one organ (kidneys, appendix, gallbladder). The diagnosis can be made only at gistol, a research of remote body or at a research of the material received at a biopsy of body.

Diagnosis

Characteristic lab. tests or pathognomonic symptoms (other than panvasculitis of medium-sized arteries with the formation of aneurysms, detected by biopsy of tissues, such as skeletal muscle), allowing a correct diagnosis do not exist. At P. at. neutrophilic leukocytosis, accelerated ESR, in some cases anemia and eosinophilia are observed in the blood. P. at. other manifestations of nonspecific inflammation are also characteristic, such as dysproteinemia (see Proteinemia), hypergammaglobulinemia (see Dysgammaglobulinemia), the appearance of C-reactive protein (see). These figures reflect Ch. arr. the degree of activity of the process, their diagnostic value is usually low. The main criterion in diagnosis is a typical wedge, symptoms. Attention is drawn to the predominance of middle-aged men among the diseased, the typical acute onset of the disease and the combination of several syndromes. Changes in hemomicrocirculation at P. at. can be detected by microscopic examination of the conjunctiva. During the period of exacerbation of the disease, they are manifested by dystonia of microvessels, a decrease in the number of functioning capillaries, a violation of the rheological properties of blood, and an increase in vascular permeability. When examining the vessels of the fundus, nodules and aneurysms can be detected.

A biopsy of the skin or muscle tissue is advisable only in cases of severe myalgia (in the acute phase of the disease) or with skin changes. Negative results of a biopsy do not contradict the diagnosis of clinically substantiated P. at. as defeats of muscles have, as a rule, focal character. When evaluating the results of gistol, studies pay attention to the prevalence, depth and severity of vasculitis, since moderate vascular changes occur in a number of diseases of the internal organs and may also be due to glucocorticosteroid therapy.

In unclear cases, it may be necessary to take a biopsy of an organ. The question in each case is solved individually. Kidney biopsy at P. at. dangerous due to the possibility of bleeding (vascular aneurysms, high blood pressure). Lung biopsy is not always possible due to the severe condition of the patients. In some cases, it is advisable to conduct an arteriographic study with contrasting vessels of the kidneys, heart, etc., which makes it possible to identify aneurysmally dilated vessels, which is pathognomonic for P. at.

Differential Diagnosis P. at. it is especially difficult at the beginning of the disease, when there is no organ pathology. Most often, patients are treated for alleged inf. diseases with large doses of antibiotics, which worsens their condition. Differential diagnosis should be carried out with some forms of tumors, for example, hypernephroma of the kidney (see), pancreatic cancer (see), to-rye also occur with fever, myalgia or thromboangiitis, weight loss.

In the initial period, the clinical picture of P. at. may be similar to prolonged septic endocarditis (see) or Hodgkin's disease (see). For P. at. chills are not characteristic, as in prolonged septic endocarditis, or profuse sweats and itching, as in patients with lymphogranulomatosis.

Patients with abdominal forms of P. at. often get to surgical or inf. hospital with suspected acute abdomen (see), dysentery (see) or other inf. illness. In such cases, it is always possible to identify, in addition to abdominal pain, some other symptomatology: polyneuritis, kidney damage, or bronchial asthma with high eosinophilia. Nephrites with arterial hypertension and various concomitant diseases are often mistaken for P. at., not taking into account that at the first stages of P. at., as a rule, it is manifested by fever, weight loss, myalgia and changes in these lab. research, which is unusual for jade.

Treatment

Until the 50s. only symptomatic therapy of P. at was carried out. In 1949, the first report appeared on the successful use of glucocorticosteroid hormones in the treatment of the disease. However, further observations showed that the use of glucocorticosteroid hormones for the treatment of patients with P. at., flowing with renal syndrome, can lead to the progression of arterial hypertension and the development of heart and kidney failure. In this regard at P. at. with kidney damage, glucocorticosteroid hormones in medium doses (prednisolone 30–40 mg per day) should be used only in the early phase of the disease, before the formation of persistent organ changes and in the absence of arterial hypertension.

Taking into account the immune mechanism of the disease, combined therapy with glucocorticosteroid hormones and cytostatics is used. A positive effect with such treatment, according to the literature, is achieved in 84% of cases. The indication to purpose of cytostatics at P. at. are the resistance or deterioration of the patient's condition in the treatment of prednisone, variants of the disease with kidney damage. When choosing a treatment, drugs from the group of antimetabolites (azathioprine) or alkylating agents (cyclophosphamide, chlorbutine) can be used, in severe cases, a combination of two cytostatics is possible. Azathioprine is most often used at a dose of 150-200 mg per day for 1-2 months. and prednisolone (15-20 mg per day) with a subsequent transition to maintenance therapy on an outpatient basis (prednisolone 10-15 mg, azathioprine 50-100 mg per day). With good tolerance and the absence of adverse reactions, maintenance therapy should be carried out for a long time, for several years, increasing the dose of drugs to therapeutic during periods of relapse of the disease.

At an asthmatic option of P. at. without kidney damage in the acute phase of the disease, higher doses of prednisolone are prescribed (up to 40–50 mg per day), then the dose is reduced to maintenance (5–10 mg per day) and used for several years.

At P. at. without obvious signs of damage to internal organs, prednisone (15-20 mg) should be prescribed only in the acute phase of the disease for a short period (1-2 months).

Good results are obtained by treatment with butadione (0.45 g per day) or 5% solution of pyrabutol (1.0 ml intramuscularly for 1-2 months). With contraindications to treatment with cytostatics, butadion can also be used in visceral forms of P. at. in combination with small doses of glucocorticosteroid hormones. In case of damage to peripheral vessels with the development of gangrene, anticoagulants are prescribed (heparin - 20,000 IU intramuscularly), antispasmodics. 4-Aminoquinoline preparations are used only for hron, the course of the disease in combination with other drugs. Treatment consists, in addition to the main therapy, in the appointment of adenyl ^ massage and exercise therapy, see Polyneuritis. P.'s treatment at. is carried out continuously and for a long time.

Forecast and Prevention

The prognosis is serious in the classical variant of the disease, however, due to the use of modern methods of treatment and rational prevention, the life span of patients with P. at. lengthened considerably. A wedge, remissions for several years are possible, however, patients with renal forms of the disease, as a rule, remain disabled. The prognosis is more favorable for the asthmatic variant of P. at. without kidney damage: the life expectancy of this group of patients is calculated in decades, some of them return to work. The forecast at skin option P. at. favorable.

Prevention. Specific prevention of P. at. not developed. It must be remembered that exacerbation of the disease can be caused by transfusions of blood and plasma and their substitutes, vaccination and the introduction of foreign sera, physiotherapy, insolation.

Features of nodular periarteritis in children

At children of P. at. develops less frequently than in adults. Children of any age are ill, mainly early childhood and school, girls and boys - with the same frequency.

Pathological anatomical features are due to the peculiarity of the course of inflammatory and allergic reactions in children, as well as age-related features of the structure of blood vessels and tissues: an abundance of cellular elements and relative structural immaturity of the vascular walls, rich vascularization of internal organs. Characterized by a bright picture of necrotic angiitis - panarteritis with the development of multiple aneurysms; frequent thromboangiitis, heart attacks of various organs.

The clinical picture is basically the same as in adults. The onset is acute, with a pronounced hyperergic component, a vivid reaction of the immunocompetent system: there is an increase in limf, nodes, and also the spleen (in 1/3 of patients). In the active phase, general symptoms predominate: fever of the wrong type, not amenable to treatment with antibiotics and antipyretics, increasing weakness, weight loss. Myalgia and arthralgia are characteristic, asymmetric polyneuritis, arthritis are less common. Of the skin lesions, the most common are livedo, capillaritis of the palms and soles, hemorrhagic rashes, skin necrosis, general and localized (mainly on the extremities) dense angioedema. Defeats c. n. With. occur, as in adults, aseptic serous meningitis occurs more often (without changes in the protein and sugar content in the cerebrospinal fluid). Pulmonary syndrome develops less frequently. Abdominal syndrome is most pronounced in young children and is usually accompanied by intestinal bleeding. Arterial hypertension is observed in 1/4 of patients. Cardiac, renal, neurological syndromes, as well as the main laboratory parameters in children and adults do not have fundamental differences. Normochromic anemia, neutrophilic leukocytosis, eosinophilia and bone marrow plasmatization, dysproteinemia with increased levels of gamma globulins, IgM, IgG, fibrinogen are detected.

Clinical variants of P. at. in adults and children are basically identical. For children, the classic renal-polyneuritic yali is a more typical polyvisceral variant, which, as a rule, is accompanied by symptoms of damage to the mesentery, intestines, c. n. s., kidneys. The cutaneous variant is more typical for school-age children; in this case, isolated lesions of small arteries of the muscular type and arterioles predominate. Along with characteristic for P. at. common symptoms in the skin and subcutaneous tissue, along the vessels (usually intercostal and abdominal walls), multiple painful nodules up to 1 cm in diameter are palpated. on the body. Trophic disturbances are possible.

Asthmatic (eosinophilic) and monoorganic variants of P. are less characteristic of childhood. There is a special, infantile variant of P. at., which proceeds with prolonged fever of the wrong type, catarrhal changes in the mucous membrane of the upper respiratory tract, polymorphic skin rashes, dense angioedema, arthralgia, myalgia, tachycardia, signs of coronaritis, increased ADH, abdominal pain, vomiting , enterocolitic stools (often with blood), hepatomegaly, erythrocyturia, leukocyturia, normochromic anemia, neutrophilic leukocytosis.

P.'s current at. in children, as a rule, progressive, with damage to internal organs - the heart, liver, went. - kish. tract, kidneys, etc. Richly developed organ vascularization in children contributes to the fact that angiitis with microthrombosis, microinfarctions of some internal organs sometimes proceeds with few symptoms, without pain.

Intravital diagnosis of P. at. in children it is rather complicated due to the multiplicity and variety of combinations of lesions of various organs, which creates a polymorphic wedge, a picture.

To confirm the diagnosis P. at. in children, as in adults, muscle and skin biopsy data help. In some cases, selective arteriography of the heart, kidneys, mesenteric vessels is performed.

Differential diagnosis of P. at. in children includes a wide range of diseases: lymphogranulomatosis (see), acute leukemia (see), sepsis (see), viral and bacterial infections, collagenoses - systemic lupus erythematosus (see), systemic scleroderma (see), dermatomyositis ( see), as well as rheumatoid arthritis (see), Wegener's granulomatosis (see Wegener's granulomatosis), diseases complicated by the development of widespread intravascular coagulation - hemorrhagic vasculitis (see Shenlein-Genoch disease), Moshkovich disease (see), etc. .

Considerable difficulties are caused by differential diagnosis of an abdominal syndrome at P. at. with intussusception, widespread intravascular coagulation syndrome with hypoxic necrotizing enterocolitis, intestinal infections, hepatitis.

Treatment in children and adults is similar. An optimally effective dose of glucocorticosteroids (1.5-3 mg / kg) is prescribed for thromboangiitis - up to 5-7 mg / kg per day. After 4-6 weeks. the dose is gradually reduced to an individual maintenance dose, which is canceled only in the phase of stable clinical and laboratory remission. With abdominal, neurological, renal syndrome with hypertension, glucocorticosteroids are ineffective. It is recommended to combine them with cytostatic drugs (azathioprine, cyclophosphamide). In case of changes in the rheological properties of the blood and the presence of hypercoagulability, heparin is prescribed in combination with corticosteroids and antispasmodics.

All children with P. at. are subject to dispensary observation, a cut includes control of an ECG, a research of functions of kidneys, etc. Vaccinations, introduction of serums and other possible allergenic factors are excluded. Preventive measures should be aimed at preventing the development of allergic reactions, reducing the frequency and severity of the course of inf. diseases.

The forecast of a classical option of P. at. children remain serious. Hron, skin option has a tendency to a long-term course.

Bibliography: Vorobyov I. V. and Lyubomudrov V. E. Nodular periarteritis, M., 1973, bibliogr.; Lyubomudrov V. E., Basamygina L. Ya. and Mateeva K. M. Nodular periarteritis in children, Pediatrics, Jvft 8, p. 76, 1960; Semenkova E. N. To the question of overdiagnosis of periarteritis nodosa, Ter. arch., v. 47, no. 4, p. 122, 1975; Strukov A. I. and Beglaryan A. G. Pathological anatomy and pathogenesis of collagen diseases, M., 1963; Tareev E. M. To the clinic of periarteritis nodosa, Rus. clinic, v. 6, Jsfb 28, p. 157, 1926; he, Collagenoses, M., 1965, bibliogr.; Tareev E. M. and Semenkova E. N. Asthmatic variant of periarteritis nodosa, Klin, medical, t. 47, “Ns 7, p. 28, 1969; Teodori M. I., Alekseev G. K. and Shnyrenkova O. V. O classification of systemic vasculitis, Ter. arch., v. 40, no. 8, p. 22, 1968; Yarygin H. E. et al. Systemic allergic vasculitis, M., 1980, bibliogr.; A 1 g c b n-S e g o v i a D. The necrotizing vasculitides, Med. Clin. N. Amer., v. 61, p. 241, 1977, bibliogr.; Churg J.a. Strauss L. Allergic granulomatosis, allergic angiitis and periarteritis nodosa, Amer. J. Path., v. 27, p. 277, 1951; E t t linger R. E. a. o. Poliarteritis nodosa in childhood, Arthr. Rheum., v. 22, p. 820, 1979; G o c k e D. J. Extrahepatic manifestations of viral hepatitis, Amer. J. med. Sc., v. 270, p. 49, 1975; Kussmaul A. u. M a i e r R. tiber eine bisher nicht beschriebene eigen-thiimliche Arterienerkrankung (Periarterii-tis nodosa), Dtsch. Arch. klin. Med., Bd 1, S. 484, 1866; Michalak T. Immune complexes of hepatitis B surface antigen in the pathogenesis of periarteritis nodosa, Amer. J. Path., v. 90, p. 619, 1978; Z e e k P. M. Periarteritis nodosa and other forms of necrotizing angiitis, New. English J. Med., v. 248, p. 764, 1953, bibliogr.

E. M. Tareev, E. H. Semenkova; S. V. Levitskaya, O. G. Solomatina (ped.), JI. M. Popova (neur.), H. E. Yarygin (stalemate. An.).

- systemic vasculitis, characterized by inflammatory-necrotic lesions of the walls of small and medium visceral and peripheral arteries. The clinic of nodular periarteritis begins with fever, myalgia, arthralgia, which are joined by thromboangiitis, skin, neurological, abdominal, cardiac, pulmonary, renal syndromes. To confirm the diagnosis of nodular periarteritis, a morphological study of skin biopsy specimens is performed. The treatment uses corticosteroids, immunosuppressants, cytostatics. The prognosis of nodular periarteritis is largely determined by the severity of damage to internal organs.

The syndrome of cardiovascular insufficiency includes the development of coronaritis leading to angina pectoris and myocardial infarction, myocarditis, cardiosclerosis, conduction disorders, arrhythmias, mitral valve insufficiency. Arterial hypertension is a characteristic cardiovascular manifestation of periarteritis nodosa. When the lungs are affected, pulmonary vasculitis and interstitial pneumonia develop, manifested by cough, shortness of breath, hemoptysis, thoracalgia, respiratory noises and wheezing, pulmonary infarctions.

Lesions of the gastrointestinal tract with nodular periarteritis proceeds with nausea, diarrhea, pain in the epigastrium. In a complicated variant, the development of pancreatic necrosis, jaundice, perforated stomach ulcers and 12p is possible. intestines, bleeding. The involvement of the nervous system is manifested by asymmetric polyneuropathy: muscle atrophy, pain in the projection of the nerve trunks, paresthesia, paresis, trophic disorders. In case of severe lesions, the occurrence of strokes, meningoencephalitis, epileptiform seizures is likely.

Visual disturbances in periarteritis nodosa are expressed by malignant retinopathy, aneurysmal dilations of the fundus vessels. Violations of the peripheral blood supply to the extremities cause ischemia and gangrene of the fingers. With lesions of the endocrine apparatus, orchitis and epididymitis, dysfunction of the adrenal glands and the thyroid gland are noted.

A variant of asthmatic nodular periarteritis occurs with persistent attacks of bronchial asthma, skin manifestations, fever, arthralgia and myalgia. The dominant manifestations of the cutaneous thromboangitic form of periarteritis nodosa are nodules, livedo and hemorrhagic purpura. Subcutaneous nodules are characterized by location along the vascular bundles of the extremities. This symptomatology develops against the background of myalgia, fever, sweating, weight loss. Periarteritis nodosa, proceeding according to the monoorganic type, is characterized by visceropathy and is established after a histological examination of a biopsy specimen or a remote organ.

Complicated forms of periarteritis nodosa may be accompanied by the development of heart attacks and sclerosis of organs, rupture of aneurysms, perforation of ulcers, intestinal gangrene, uremia, stroke, encephalomyelitis.

Diagnosis of nodular periarteritis

In the general clinical analysis of urine, microhematuria, proteinuria and cylindruria are determined; in the blood - signs of neutrophilic leukocytosis, hyperthrombocytosis, anemia. Changes in the biochemical blood picture in periarteritis nodosa are characterized by an increase in the fractions of γ- and α2-globulins, sialic acids, fibrin, seromucoid, CRP.

To clarify the diagnosis with periarteritis nodosa, a biopsy is performed. In the skin-muscle biopsy of the abdominal wall or lower leg, inflammatory infiltration and necrotic changes in the vascular walls are detected. With periarteritis nodosa, HBsAg or antibodies to it are often determined in the blood. When examining the fundus, aneurysmal changes in the vessels are detected. Ultrasound of the kidney vessels determines their stenosis. Plain radiography of the lungs shows an increase in the pulmonary pattern and its deformation. For the diagnosis of cardiopathy, an ECG, ultrasound of the heart is performed.

Large diagnostic criteria for periarteritis nodosa include the presence of kidney damage, abdominal syndrome, coronaritis, polyneuritis, bronchial asthma with eosinophilia. Additional (small) criteria are myalgia, fever, weight loss. When diagnosing nodular periarteritis, three major and two minor criteria are taken into account.

Treatment of nodular periarteritis

The therapy is characterized by continuity and duration (up to 2-3 years), complexity and individual selection of funds. Taking into account the form of the disease, it is carried out by the joint efforts of a rheumatologist, cardiologist, nephrologist, pulmonologist and other specialists. The course of early and uncomplicated forms of periarteritis nodosa can be corrected by corticosteroid therapy with prednisone, repeated courses 2-3 times a year. In the interval between corticosteroid courses, drugs of the pyrazolone series (butadione) or acetylsalicylic acid are prescribed.

With periarteritis nodosa complicated by malignant hypertension or nephrotic syndrome, immunosuppressive cytostatics (azathioprine, cyclophosphamide) are prescribed. Correction of the DIC syndrome, thrombosis, perforation complications, etc.) can be fatal. Remission and arrest of progression of periarteritis nodosa is achieved in 50% of patients.

Preventive tasks include accounting for drug intolerance, reasonable and controlled immunization, transfusion of blood products, protection against infections.