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

Periarteritis nodosa (polyarteritis), or Kussmaul-Mayer disease (from the 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 vascular stacks, and leads to failure of the organs feeding the vessel.

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

The consequence is that small and medium-sized arteries are affected with disruption of the normal structure of the walls (protrusion of the walls). This condition leads to disruption of blood circulation, nutrition of individual organs and tissue areas.

Nodular disease is also referred to as polyarteritis and 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 pathology, which lead to the formation of blood clots, nodules and death of tissues of internal organs.

In most cases, periarteritis nodosa occurs in males in the age group from thirty to fifty years, in children and the elderly.

What is special about the disease?

For the first time, a disease such 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 that this disease affects small arteries in organs of different localizations.

The most commonly affected organs 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 intestinal mesentery. Damage to these vessels can lead to complications incompatible with life;
• Secondarily, the arterial vessels of the skeletal muscles, adrenal glands, pancreas and stomach are affected. Damage to the arteries of these organs is fraught with disturbances in the functionality of the body;
• The last to be affected are the large vessels – the subclavian and carotid arteries. Their defeat occurs extremely rarely.

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

The pathology is a rare one, and is registered in one case per million of the world's population. It is characteristic that males are affected twice as often as females.

Definitive 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 occurs in a chronic form. In the absence of effective treatment, survival rate is thirteen out of one hundred people affected by periarteritis nodosa.

Necrosis of the vascular wall in polyarteritis nodosa

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

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

If you notice the first signs of periarteritis nodosa, you need to contact a dermatologist or infectious diseases doctor.

Further treatment and consultations with other specialists are based on which vessels are affected and which organ they fed.

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

Kidney damage from periarteritis nodosa

Classification

Periarteritis nodosa is classified into three clinical forms and five development options with different life expectancies.

The clinical picture of the disease today involves three forms of periarteritis nodosa, shown in the table below.

Form of the disease	Characteristic
Classic development	This form is characterized by an increase in body temperature, a sharp decrease in weight, and pain in the muscles and joints.
	This form primarily affects the gastrointestinal tract, nervous system, heart and kidneys
Cutaneous form	It is characterized by fevers, weight loss, a sharp feeling of weakness, and pain in the muscles.
	This form is characterized by the formation of nodules under the skin located in the extremities, 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 examination 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 complications, life expectancy is more than ten years from the moment the first signs appear
Relapse type	Relapses occur when the dosage of medications is reduced, infection, hypothermia and colds	If left untreated, life expectancy is about five years in thirteen percent of patients.
		With effective treatment, the survival rate increases to forty percent
Fast-paced	Kidney pathologies and pathological high blood pressure	The patient lives until the renal artery ruptures or completely narrows
Lightning type	Pathological high blood pressure, kidney pathologies, heart failure, intestinal ulcers, thrombosis of intestinal arteries	From 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.

The remaining 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 loss of ability to work, and after that to the definition of disability.

Causes of periarteritis nodosa

The causes of periarteritis nodosa have not been fully determined.

The main versions of the origin of the pathology are:

• Infectious lesions of a bacterial nature;
• Obvious damage to toxins from 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 periarteritis nodosa is the presence of HIV infection, influenza, rubella, hepatitis B and other viral infections in people.

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

There are also some risk factors, including:

• Hypothermia;
• Genetic disposition;
• Irradiation of the body;
• Allergic reactions of the body to certain types of medications;
• Immune complexes deposited on the vascular walls, causing their damage;
• Children with allergic reactions to food, high sensitivity to medications, coronary heart attack, high blood pressure.

Symptoms of periarteritis nodosa

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

Clinical sign	Characteristic
Increase in body temperature	Temperature indicators in periarteritis nodosa are characteristically elevated, which does not decrease with antibiotic treatment
Skin abnormalities	Pale skin, dilation of blood vessels 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 towards activity
Kidney pathologies	Narrowing of the kidney artery, its rapid shrinkage, protein formation, death of kidney tissue and kidney failure
Lung pathologies	Pain in the chest area, heavy breathing, coughing, coughing up blood, death of lung tissue
Pathologies of the eye structure	Protrusions or thickening of the walls of the blood vessels of the eye, deformation of the retina, leading to loss of 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 abdominal wall, death of pancreatic tissue, intestinal ulcers that can rupture
Pathological condition of the arterial vessels of the legs	Insufficient blood supply to the toes, which can lead to gangrene. Vessel protrusions may become deformed with further internal hemorrhage
Endocrine system disorders	Inflammation caused by the production of antibodies to one's own cells by the immune system, localized in the male testicles, malfunction of the adrenal glands and thyroid function
Damage to muscles and joints	Pain, general weakness, muscle atrophy, arteritis of large joints, possibly several at once
Cardiovascular syndrome	An inflammatory process in the vessels of the heart, leading to chest pain, irregular heart rhythms, death of heart muscle tissue, mitral valve insufficiency, and high blood pressure
Pathologies of the nervous system	Damage to one or many nerve endings, which are accompanied by burning pain, a feeling of weakness in the leg, inflammation of the membranes of the brain, seizures

Diagnosis of polyarteritis

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

When the three criteria coincide, periarteritis nodosa is diagnosed.

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

Diagnosis criteria	Characteristic
Hepatitis B	Fixing a given diagnosis in a patient, or antibodies produced against it
Pain in the testicles	What is not caused by injuries or infectious diseases
Weight loss of more than four kilograms in a month	What happens without changing your diet and diet
Increased blood pressure
Livedo reticularis	It is a pathological condition of the skin, in which it has a bluish tint due to translucent vessels filled with blood.
Myalgia	Feeling of weakness and pain in the leg muscles. There is no pain in the shoulders and lower back
Increased levels of urea, or creatine, in the blood	If creatine exceeds more than 133 mmol/l, urea exceeds 14.4 mmol/l. In addition, there is no dehydration or blockage 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.
Angiography abnormalities	When studying vessels with a contrast agent, protrusions or overlaps of small arterial vessels leading to individual organs are recorded. Atherosclerotic deposits are not detected, as are other defects not associated with inflammation
Mononeuritis (polyneuropathy)	Inflammation of one or many nerve endings in the peripheral nervous system is recorded

Treatment of periarteritis nodosa

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

The course is compiled by doctors of different specializations, depending on the location of the affected vessels.

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

The most common medications used for periarteritis nodosa are:

• Glucocorticosteroids in high dosages (Prednisolone, Dexamethasone, Triamcinolone)– drugs have the greatest effect at the initial stages of the disease. Long-term use of them reduces blood pressure and eliminates kidney failure. Medicines help relieve inflammation and suppress the immune system;
• Medicines of the pyrazolone series (Aspirin, Butadione)– used together with non-steroidal anti-inflammatory drugs to enhance the effect of glucocorticosteroids;
• Cytotoxic medications (Cyclophosphamide, Azathioprine)– help to avoid the progression of serious complications;
• When correcting hyperthrombocytosis and preventing disseminated intravascular coagulation (Trental, Curantil)– restore healthy local blood circulation;
• Antibiotics– used for infectious lesions of the skin;
• To eliminate symptoms, use painkillers and blood pressure medications. 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 preventative measures?

• Proper nutrition. The diet should contain a large amount of vitamins and nutrients so that the walls of blood vessels remain elastic;
• Maintaining a daily routine. The working day should contain sufficient 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 at least one hour a day, as well as engage in active sports;
• Treat infectious diseases in a timely manner;
• Carefully study the instructions for medications to avoid allergic reactions;
• Have a preventive examination once a year.

All actions are aimed at maintaining healthy blood vessels.

Video: Polyarteritis nodosa.

What's the forecast?

The prognosis for periarteritis nodosa is unfavorable. Serious vascular lesions, which lead to dysfunction and death of organ tissue, can lead to death. According to statistics, remission is achieved in half of registered patients.

The effectiveness of treatment for periarteritis nodosa depends on the correctness of its prescription, timely diagnosis and compliance with preventive recommendations.

If you notice the slightest symptoms, go to the hospital for a full examination.

Do not self-medicate and be healthy!

Systemic vasculitis includes diseases that are based on widespread necrotic-inflammatory changes in blood vessels. Vasculitis of varying severity can occur in diffuse connective tissue diseases such as rheumatoid arthritis (RA) And systemic lupus erythematosus (SCR), largely determining their course. First of all, independent, “primary” systemic vasculitis is considered, in which inflammatory changes in blood 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 facts such as the possibility of developing severe necrotizing vasculitis in serum sickness and other immune complex diseases. In patients with certain variants of systemic vasculitis, deposits of immunoglobulins and complement are found in the vascular wall. Finally, the basis of treatment for systemic vasculitis is prednisolone and immunosuppressants.

Morphologically, fibrinoid changes and necrosis of the vascular walls are observed in combination with cellular infiltration, spreading 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 a narrowing of the lumen of small vessels and thereby to ischemia of the corresponding organs. The most common localization for most systemic vasculitis is the arterial system, i.e. we are talking primarily about systemic arteritis. In certain forms of vasculitis, other vessels are no less affected.

Classification

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

For example, with polyarteritis nodosa, a patient’s arterioles, small and medium-sized arteries may be affected, but only damage to 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 (intraorgan), 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 includes primarily classic polyarteritis nodosa (periarteritis, as well as arteritis of a similar type in diffuse connective tissue diseases, in particular 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 (Henoch-Schönlein 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 arteritis.

There are transitional forms between classic polyarteritis nodosa and granulomatous arteritis, which justifies their inclusion in one qualification 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 can 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, inflammatory cell infiltration and fibrinoid necrosis of the adventitia, media and endothelium are noted. 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 attracts attention.

In the later stages of the disease, mononuclear cells and possibly a moderate number of eosinophils are also noticeable in the infiltrates. In rare cases, single giant cells are found. When inflammation in a specific area of ​​the vessel is completed, the inflammatory infiltrate disappears, fibrous replacement of the affected area (especially the subendothelial layer) develops with the destruction of the internal elastic membrane. The simultaneous presence of various stages of arterial damage in one patient is typical.

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

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

Etiology and pathogenesis

Views on the pathogenesis of polyarteritis are essentially unanimous- most authors believe that it is based on immune mechanisms. For the first time, this 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 with 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 periarteritis nodosa in experiments on rabbits by sensitizing them with horse serum and sulfadiazine.

A. Rich (1942, 1945) also showed that in some patients the disease develops as an immune reaction to the administration of medicinal serums, sulfonamides and iodide preparations. Subsequently, ideas about the immune pathogenesis of periarteritis nodosa became even stronger. 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 showed that type III immune tissue damage is essential in the pathogenesis of polyarteritis - deposition of immune complexes antigen- antibody in arterial walls. These complexes are capable of activating complement, which may result in direct tissue damage, as well as the formation of chemotactic substances that attract neutrophils to the lesion.

The latter phagocytose deposited immune complexes, resulting in the release of lysosomal enzymes that can destroy the main membrane and the internal 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.

The interaction of immune complexes and neutrophils with endothelial cells is of particular importance. The latter have receptors for the Fc fragment of human IgG and for the first component of complement (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 clotting and promote thrombus formation in conditions of inflammation of the vascular wall.

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

The combination of such results with a decrease in the concentration of complement in the serum and an increase in circulating immune complexes led to the assumption that polyarteritis may be an immune complex disease in which HBs-Ag can be the trigger antigen, i.e., the main etiological factor. At the same time, one should not assume 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 causes the development of the disease, but is by no means the only possible etiological factor.

This is proven by the presence of patients with polyarteritis in whom immune complexes (circulating and in the walls of the arteries) that do not contain HBs-Ag are found. In most of these cases, the specific antigen cannot be identified, but in some patients it is identified. There is a report of 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. There are known individuals with polyarteritis in whom the corresponding antigen was detected in the blood, but immune complexes were not recorded.

In accordance with these data, it is most likely to consider polyarteritis predominantly an immune complex disease caused by various antigens: bacterial, viral, drugs, tumors, 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 very likely that different pathogenetic pathways lead to systemic inflammation of the arteries 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 particularly uncommon. Interestingly, the experiment was able to show the possibility of developing both immune complex viral vasculitis (in mice infected with the 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 influences: high arterial hypertension induced by compression of the renal arteries; administration of deoxycorticosterone acetate together with sodium chloride; prescribing an extract of the anterior lobe of the pituitary gland against the background of unilateral nephrectomy. Apparently, the main common factor is the influence of a sharp increase in arterial tone with possible necrotic changes in their walls.

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

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

The clinical picture of polyarteritis is determined mainly by the localization, extent and extent of vascular damage. The symptoms of the disease themselves are not particularly characteristic, but their combinations and significant diversity have significant diagnostic significance. The onset of the disease is often acute or at least quite distinct. Gradual progression of the disease is less common.

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

More than half of the patients experience significant and rapid weight loss. Pain syndrome of various localizations is very often expressed (primarily severe and prolonged pain in muscles and joints, less often in the abdomen, in the area of ​​the heart, head, etc.). Fever and myalgia are the most important clinical signs for differentiating polyarteritis from rheumatoid and hemorrhagic vasculitis.

Particular manifestations of polyarteritis

Skin lesions occur in approximately ¼ of patients with arthritis, sometimes being one of the initial symptoms of the disease. The predominance of skin changes in some cases led some authors to identify a predominantly “cutaneous form” of polyarteritis. The nature of the skin pathology can be very different: urticaria, erythema multiforme, maculopapular rash, livedo reticularis with a pronounced “marbling” pattern 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 membrane. Necrotic skin changes due to infarction of cutaneous vessels and manifested by ulcerations are relatively typical. Usually they are multiple and small, but in the case of blockage of larger arteries they turn out to be extensive and are combined with peripheral gangrene of the tissues of the extremities. Blistering and bullous rashes are extremely rare.

Skin changes(primarily ulcers, nodules, livedo) with a typical histological picture of polyarteritis are sometimes found without signs of a systemic disease or are combined with moderate muscle and neurological symptoms (but relating 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 and their prognosis is good. There are indications of their possible combination with inflammatory bowel diseases.

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

Joint lesions are also quite common and sometimes the first symptoms of the disease. Arthralgia is common to most patients. True arthritis is also common, which, against the background of a general serious condition and severe muscle pain, may disappear from view. Characterized by reversible arthritis of large joints, 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 can 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 blood vessels of the glomeruli, which occur clinically, as a rule, as glomerulonephritis and, when severe, have an unfavorable prognostic value.

In the initial stages, the main signs of kidney damage are hematuria and proteinuria, including very moderate ones. Swelling is not typical. Hypertension is common, but normal blood pressure does not exclude renal pathology. As 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 rate of patients with polyarteritis from uremia - approximately 20-25% of all fatal cases.

In addition to the glomerular changes characteristic of polyarteritis, others are described that are much less common and are usually associated with damage to larger vessels. Thus, arterial thrombosis can cause kidney infarction with the appearance of severe pain in the lumbar region and massive hematuria. Necrosis of the papillae 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 perinephric abscess, given the high fever characteristic of polyarteritis.

Nephrotic syndrome It is rare and usually results from renal vein thrombosis. Among other lesions of the urinary system, involvement of the vessels of the bladder (clinically manifested by dysuria) and ureters is occasionally noted. In the latter case, using ureterography, it is possible to establish a spasm of the ureters with expansion of the overlying sections. Impaired urine outflow 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 pathological studies, in approximately 70% of patients. As the main cause of death, these lesions occupy second place, second only to renal pathology. The high frequency of involvement of the heart arteries in the process naturally leads to coronary insufficiency, the clinical manifestations of which are not always clear and are sometimes completely absent. This feature of the disease is explained by the predominant damage to small and medium-sized arteries, which in many patients is not accompanied by typical angina pain. With polyarteritis, small, painless myocardial infarctions have been described. In such cases, electrocardiographic examination provides significant assistance.

The most common development is congestive circulatory failure, which is difficult to treat. Various rhythm and conduction disturbances are characteristic, especially supraventricular extrasystoles and tachycardia. Such rhythm disturbances may be a consequence of vascular damage to the sinoatrial node, which is very actively vascularized.

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

In the genesis of circulatory failure, in addition to coronary arteritis, hypertension, which occurs in most patients due to simultaneous kidney damage, is important. The negative impact of high blood pressure is aggravated 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 largely associated with hypertension of renal origin.

Damage to the venous trunks, sometimes occurring as migrating phlebitis, and Raynaud's syndrome are rare manifestations of polyarteritis.

Lung lesions are not very typical for classical polyarteritis, but are characteristic of other vasculitis. However, 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. Damage to the vessels of the digestive tract occurs in almost half of patients and gives pronounced clinical symptoms.

The location of damage is varied; Changes are most often found in the arteries of the small intestine and mesenteric, the stomach suffers less often. Thrombosis and ruptures of the affected vessels are the cause of pain and bleeding (intestinal, less often gastric) that are extremely characteristic of polyarteritis. The combination of these signs 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 common signs of gastrointestinal involvement 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. Angiography is of great diagnostic importance, allowing to detect aneurysms of the abdominal arteries (in particular, intestinal and hepatic) in most patients.

Abdominal pain can be caused by ischemia or microinfarctions of the liver, spleen or mesentery. Damage to liver vessels, in addition to infarctions 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 is enlarged in a small number of patients, and even in people with obvious splenic arteritis, organ enlargement is not always detected. Among the rare abdominal syndromes of polyarteritis, abdominal toad syndrome 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, 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.

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 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.

picture: necrosis of the vascular wall in 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:

1. Syphilitic damage to 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 individuals infected with hepatitis B, HIV, influenza, rubella, cytomegalovirus infection, and 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 heart disease, and hypertension.

Clinic

Among the general symptoms of the disease, the most common and significant are fever, arthralgia, myalgia, and 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, cerebrovascular accident 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 most often occurs among girls of any age. 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 palpated along the course of large vessels. Painful, dense swelling is located in the area of ​​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 of the patient, laboratory tests of blood and urine.

Additional diagnostic methods:

1. Arteriography is a method of examining arteries by injecting a contrast agent and taking a series of x-rays. In patients, arterial aneurysms and their occlusion are detected.
2. A biopsy of the muscles of the lower leg or abdominal wall can reveal characteristic vascular changes.
3. Microscopy of material taken from the patient's skin.
4. A plain X-ray of the lungs shows a clear deformation of the pulmonary pattern.
5. 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 an acute form of the pathology are recommended bed rest or semi-bed rest, gentle nutrition with a maximum of vitamins and microelements. After the exacerbation subsides, walking in the fresh air, adherence to the correct daily routine, and psychotherapy are recommended.

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 in the early stages of the disease. Long-term use of Prednisolone, Dexamethasone, and 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 prolonged use of 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 there are infectious complications on the skin - purulent foci - antibiotic therapy is carried out.
• Symptomatic treatment is painkillers and antihypertensive drugs. Vitamins, antihistamines, diuretics, and cardiac glycosides are administered parenterally.

After the acute inflammation is relieved, they move on to physical therapy, massage, and 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 arteries of the muscular type. The disease belongs to polyetiological, but monopathogenetic pathologies.

Video: periarteritis nodosa, mini-lecture

PERIARTERITIS NODULA (periarteriitis nodosa; Greek peri around, about + arteritis; syn.: Kussmaul-Mayer disease, panarteritis nodosa) 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. u. characterized by damage to small and medium arteries of the muscular type with the formation of vascular aneurysms (“nodules”), which is how the disease got its name. Due to the fact that the inflammatory process is not limited to the outer membrane (adventitia) of the vessel, but involves all layers of the vascular wall, P. at. it would be more correct to call it nodular panarteritis, but in the USSR the name periarteritis nodosa, proposed in 1866 by Kussmaul and R. Maier, is retained. In Russia, a description of the first two cases of P. u. belongs to A.P. Langovoy (1883), who worked in the clinic of prof. A. A. Ostroumova. Lifetime diagnosis of P. u. was first placed in our country 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. u. classified as systemic vascular diseases. In the USA, the Zeek classification has been adopted (R. M. Zeek, 1953), which distinguishes classic P. at., allergic P. at. with bronchial asthma and eosinophilia, hyperergic angiitis with drug and serum sickness. Alrcon-Segovia (D. Alrcon-Segovia, 1977) suggests distinguishing between generalized classic P. at. immune genesis, hypersensitive limited (skin, kidney, etc.) and allergic P. at. (eosinophilic angiitis).

P.u. classified as rare diseases. Engelbert (O. Engelberth, 1962) discovered P. at 41,478 autopsies (1939-1956). in 0.13% of cases. However, there is a clear tendency towards its increase. According to sectional materials from Johns Hopkins University (Baltimore, 1926-1942) based on data from A.R. Rich, an increase in P. at. from 1: 1600 to 1: 137. According to I. V. Vorobyov and V. E. Lyubomudrov, P. u. Mostly men aged 21-60 years are affected.

Etiology and pathogenesis

Etiology of P. 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. The occurrence of P. at is especially common. associated with the effects of various drugs (sulfonamides, penicillin, thiouracil, aminazine, iodine preparations, mercury) and the introduction of foreign serums. Since 1970, the possibility of a viral etiology of P. has been discussed. In this case, crucial importance is attached to 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. Gocke (D. J. Gocke) et al., Gerber (Gerber) et al., described cases of P. 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 is observed.

Pathogenesis of P. u. associated with immunopathological processes. Paronetto and Strauss (F. Paronetto, L. Strauss, 1962), using fluorescent techniques, 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. they received vascular changes characteristic of this disease; Such changes were absent when the animals were administered convalescent blood serum.

Pathological anatomy

Inflammatory changes in P. u. are found in vessels of different levels and different functions, purposes - in 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 one is damage to arteries of the muscular and muscle-elastic type. Inflammatory changes in blood vessels with P. u. 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 acquires a mixed character and their morphology reflects the complexity of the relationship between humoral and cellular allergic reactions. Immunopathological genesis of vasculitis in P. u. confirmed by immunofluorescence (see) and electron microscopy (see). In particular, when studying material obtained from patient P. u. Kidney biopsy showed that exacerbation of the disease is accompanied by fixation on the basement membranes of vascular loops, in the mesangium and parietal layer of the glomeruli of the kidneys of immunoglobulins (IgG, IgA, IgM), the C3-fraction of complement and fibrin, giving a coarse-grained or focally linear glow. Electron microscopy in the renal glomeruli of patients with P. at. subendothelial, mesangial, and occasionally subepithelial deposits of immune complexes, which include fibrin, are found. In inflammatory-changed vessels with P. u. 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).

When gistol, and histochemical, studying biopsy and autopsy material, it was established that morphol, changes in arterial vessels with 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 dystrophy) is caused by the decomposition of dissociating protein-polysaccharide complexes of the main substance of connective tissue with the release of glycosaminoglycans, which leads to an increase in vascular permeability and hydration of the main substance of this tissue. Fibrinoid necrosis (see Fibrinoid transformation) develops following the plasmatic impregnation of arterial walls and is characterized by the loss of amorphous and filamentous masses of fibrin in them.

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

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 membranes of the vessel gives grounds to talk about endo-, meso- and periarteritis. However, often with P. at. it is necessary to state the defeat of all three membranes; in such cases, the process is referred to as panarteritis. Since the disease is characterized by a chronic, relapsing course, mucoid swelling, fibrinoid necrosis, infiltrative and proliferative reactions sometimes occur in sclerotic arteries. The most severe consequence of arteritis with P. at. is a progressive stenosis of the affected arteries. Often, in pathologically altered vessels, especially with aneurysms, fresh, organizing or organized (canalized) blood clots are found (see Thrombus).

Vasculitis with P. u. 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 consequence of arteritis and thrombarteritis, local changes occur in various organs and tissues: hemorrhages, degeneration and atrophy of parenchymal elements, focal necrotic and ulcerative processes, heart attacks and scars after them, sclerotic and cirrhotic phenomena. In 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 degeneration).

Along with the arteritis described above, an important place in P.’s pathology. 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 the 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. u. glucocorticosteroids and immunosuppressants, a predominance of productive forms of vasculitis is noted.

Clinical picture

For P. u. characterized by extreme polymorphism of the wedge, symptoms, which complicates diagnosis. The disease usually begins gradually with general symptoms. The most characteristic of P. at. are fever, progressive weight loss and muscle-joint pain. Among the general symptoms, the first place in frequency (95-100%) is fever (see). Most patients have fever of the wrong type; the temperature does not decrease with the use of antibiotics, but quickly disappears under the influence of glucocorticosteroid hormones. Fever at the onset of the disease is characterized by significant persistence; When organ pathology appears, it, as a rule, does not recur.

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

Common manifestations of intoxication include the following, characteristic of P. symptoms such as tachycardia, which does not decrease when taking glycosides, and sweating.

Sometimes the disease begins with organ lesions, which appear several months and even years before the onset of systemic 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 pathologies characteristic of P. u., there are five most common syndromes that determine the specificity 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 kidney damage usually indicates an advanced process. The most typical sign of kidney damage with P. at. is arterial hypertension (see Arterial hypertension), in most cases stable, persistent, sometimes galloping, with the development of severe retinopathy (see) and loss of vision. Moderate proteinuria (1.0-3.0 g per day) and microhematuria are observed. Gross hematuria occurs rarely. The development of nephrotic syndrome (proteinuria more than 3.0 g per day, peripheral edema) is extremely rare. A rupture of an aneurysmically dilated renal vessel with the formation of a perinephric hematoma is possible. The prognosis of renal syndrome is very serious: it can lead to the development of renal failure within 1-3 years.

Abdominal syndrome is the 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 pain is usually diffuse in nature, it is constant, persistent, and increasing in intensity. Of the dyspeptic disorders, the most pronounced is diarrhea (stool frequency up to 6-10 times a day); There is an admixture of blood and mucus in the stool. Characterized by anorexia (see), sometimes nausea, vomiting. Peritonitis (see) often develops as a result of perforation of ulcers or gangrene of the intestines, sometimes gastrointestinal bleeding occurs (see). Liver damage due to P. u. is observed relatively rarely and is characterized by the development of infarctions and rupture of aneurysms of intrahepatic vessels. Development of hron, hepatitis or cirrhosis of the liver with P. at. is caused by hron, a viral infection (serum hepatitis virus), which is confirmed by serol data, research and intravital organ biopsy. Lesions of the pancreas and gallbladder are detected more often during pathological examination, however, in some patients with systemic manifestations of the disease, symptoms of pancreatitis (see) or cholecystitis (see) may be detected.

Cardiac syndrome is characterized predominantly by coronaritis (see) and occurs in 50-70% of patients. Clinically, it is sometimes difficult to distinguish between cardiac damage caused by P. u. and secondary changes caused by severe arterial hypertension. Coronary disorders are often asymptomatic and are 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. Characteristic is the development of a peculiar angiogenic lesion of the heart of the type of rapidly progressing cardiosclerosis (see) with rhythm disturbances, conduction and heart failure. Heart damage is not often the only cause of death. Possibility of endocardial damage with P. at. is a controversial issue.

Pulmonary syndrome is observed in 30-45% of cases and can manifest itself as 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, the cough is accompanied by the separation of a scant amount of mucous sputum, and occasionally hemoptysis; fever and increasing signs of respiratory failure are noted. X-ray in the lungs shows a sharp increase in the vascular pattern, reminiscent of congestive lung, infiltration of lung tissue mainly in the hilar zones. An important diagnostic sign can be the low effectiveness of antibiotics and the high effectiveness of glucocorticosteroid therapy.

Neurological syndrome (damage to the central and peripheral nervous system) is caused by systemic inflammatory changes in the walls of brain vessels and nerve sheaths. 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 damage to the peripheral nervous system that is the most characteristic and diagnostically important symptom of P. at. Mononeuritis and asymmetrical neuritis are observed (see Neuritis, Polyneuritis). Occasionally, polyneuritis of the type of Landry's ascending paralysis is observed (see Landry's ascending paralysis). Typically, peripheral neurol disorders develop gradually: first, pain and paresthesia appear in the distal parts of the arms and legs, then muscle weakness occurs. Muscle soreness, most often the calf, and sensitivity disorders of the radicular and polyneuritic type are constantly observed.

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

In 15-30% of patients with P. u. There are skin changes characterized by the presence of nodules along the vessels, various sizes of tree-like branching loops of a bluish-red color without peeling - livedo racemosa (see Livedo) or ulcerative-necrotic changes. With P. u. Gangrene of fingers and limbs, necrosis of soft tissues, which are caused by damage to peripheral vessels, may be observed.

Changes in the eyes with P. at. are rare in the form of iridocyclitis (see) or vasculitis of retinal vessels with thrombosis or microaneurysms.

Most often with 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 (coronary disease with diffuse and focal changes on the ECG) with progressive heart failure; pulmonary-cardio-renal, often beginning in the form of hypereosinophilic asthma or pneumonitis; pulmonary polyneuritis, starting with bronchial asthma with the further addition of polyneuritis.

Dominance in the clinic P. u. One or more of these syndromes allows us to identify a number of clinical variants of the disease.

The classic (renal-polyneuritic, or polyvisceral) variant begins, as a rule, with fever, muscle-articular pain, and severe weight loss. In the wedge, kidney damage with arterial hypertension, often malignant, comes to the fore in the picture; coronaritis, the combination of which with arterial hypertension leads to the rapid development of heart failure, as well as abdominal pain and polyneuritis. Lung damage occurs as vascular pneumonia and is not so common. HBsAg is sometimes detected in the blood serum, and with a liver biopsy - signs of hron, active hepatitis or cirrhosis. The presence of immune complexes in blood serum, organs and tissues is possible.

The 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 and 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. Possible fever, accelerated ROE. After 1-5 years, the process generalizes with the development of polyvisceral symptoms characteristic of the classic version of P. at. In half of the cases, the disease occurs without kidney damage, being limited to peripheral neuritis, skin changes or disorders of the gastrointestinal tract. tract. Along with the symptoms of bronchial asthma, eosinophilic infiltrates are often observed in the lungs.

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

The monoorgan variant is extremely rare and manifests itself in damage to one organ (kidneys, appendix, gall bladder). The diagnosis can be made only with histol, examination of a removed organ, or examination of material obtained from an organ biopsy.

Diagnosis

Characteristic lab. There are no tests or pathognomonic symptoms (other than panvasculitis of medium-sized arteries with aneurysm formation detected by tissue biopsy, such as skeletal muscle) to establish the correct diagnosis. With P. u. Neutrophilic leukocytosis, accelerated ROE, and in some cases anemia and eosinophilia are observed in the blood. P.u. other manifestations of nonspecific inflammation are also characteristic, such as dysproteinemia (see Proteinemia), hypergammaglobulinemia (see Dysgammaglobulinemia), the appearance of C-reactive protein (see). These indicators reflect ch. arr. the degree of activity of the process, their diagnostic value is usually low. The main criterion in diagnosis is the typical wedge, symptoms. Attention is drawn to the predominance of middle-aged men among the sick, the typical acute onset of the disease and the combination of several syndromes. Changes in hemomicrocirculation during P. at. can be detected by microscopic examination of the conjunctiva. During the period of exacerbation of the disease, they are manifested by microvascular dystonia, 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 skin or muscle tissue is advisable only in cases of severe myalgia (in the acute phase of the disease) or in cases of skin changes. Negative biopsy results do not contradict the diagnosis of clinically substantiated P. u., since muscle lesions are, as a rule, focal in nature. When assessing 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 internal organs and can also be caused by glucocorticosteroid therapy.

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

Differential diagnosis P.u. It is especially difficult at the beginning of the disease, when there is no organ pathology. Most often, patients are treated for suspected 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), which also occur with fever, myalgia or thrombusitis, weight loss.

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

Patients with abdominal forms of P. at. often end up in surgery or inf. hospital with suspected acute abdomen (see), dysentery (see) or other inf. diseases. 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. Nephritis with arterial hypertension and various concomitant diseases are often mistaken for P. u., not taking into account that in the first stages of P. u., as a rule, it is manifested by fever, weight loss, myalgia and changes in lab data. research, which is unusual for jade.

Treatment

Until the 50s. Only symptomatic therapy was carried out for P. u. In 1949, the first report of the successful use of glucocorticosteroid hormones in the treatment of the disease appeared. However, further observations have shown that the use of glucocorticosteroid hormones for the treatment of patients with P. u., occurring with renal syndrome, can lead to the progression of arterial hypertension and the development of heart and kidney failure. In this regard, with P. at. with kidney damage, glucocorticosteroid hormones in medium doses (prednisolone 30-40 mg per day) are advisable to use 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 this treatment, according to literature data, is achieved in 84% of cases. Indications for the appointment of cytostatics for P. u. are resistance or deterioration of the patient’s condition during treatment with prednisolone, variants of the disease with kidney damage. When choosing treatment, drugs from the group of antimetabolites (azathioprine) or alkylating agents (cyclophosphamide, chlorobutin) 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 subsequent transition to maintenance therapy on an outpatient basis (prednisolone 10-15 mg, azathioprine 50-100 mg per day). If it is well tolerated and there are no adverse reactions, maintenance therapy should be carried out long-term, for several years, increasing the dose of drugs to the therapeutic dose during periods of relapse of the disease.

With the asthmatic variant of P. u. 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 a maintenance dose (5-10 mg per day) and used for several years.

With P. u. without obvious signs of damage to internal organs, prednisolone (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% pyrabutol solution (1.0 ml intramuscularly for 1-2 months). If there are contraindications to treatment with cytostatics, butadione can also be used for visceral forms of P. at. in combination with small doses of glucocorticosteroid hormones. If peripheral vessels are damaged with the development of gangrene, anticoagulants (heparin - 20,000 units intramuscularly) and antispasmodics are prescribed. 4-Aminoquinoline drugs are used only for chronic illness in combination with other drugs. Treatment consists, in addition to the main therapy, in the appointment of adenyl, massage and exercise therapy, see Polyneuritis. Treatment of P. u. is carried out continuously and for a long time.

Prognosis and Prevention

The prognosis is serious with the classic version of the disease, however, due to the use of modern methods of treatment and rational prevention, the life expectancy of patients with P. u. have lengthened significantly. A wedge and remissions for several years are possible, but 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. Forecast for the cutaneous variant of P. at. favorable.

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

Features of periarteritis nodosa in children

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

Pathoanatomical features are due to the unique course of inflammatory and allergic reactions in children, as well as age-related features of the structure of blood vessels and tissues: the abundance of cellular elements and the relative structural immaturity of the vascular walls, rich vascularization of internal organs. A clear picture of necrotizing angiitis is characteristic - panarteritis with the development of multiple aneurysms; thromboangiitis and infarctions of various organs are common.

The clinical picture is basically the same as in adults. The onset is acute, with a pronounced hyperergic component, a strong reaction of the immunocompetent system: there is an increase in lymph nodes, as well as in the spleen (in 1/3 of patients). In the active phase, general symptoms predominate: fever of the wrong type that cannot be treated with antibiotics and antipyretics, increasing weakness, weight loss. Myalgia and arthralgia are typical, asymmetric polyneuritis and arthritis are less common. The most common skin lesions are livedo, capillaritis of the palms and soles, hemorrhagic rashes, skin necrosis, general and localized (mainly on the extremities) dense angioedema. Lesions c. n. With. proceed 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 basic laboratory parameters in children and adults are not fundamentally different. Normochromic anemia, neutrophilic leukocytosis, eosinophilia and bone marrow plasmatization, dysproteinemia with increased levels of gamma globulins, IgM, IgG, and fibrinogen are detected.

Clinical variants of P. u. in adults and children are basically identical. For children, the classic renal polyneuritic or polyvisceral variant is more typical, which, as a rule, is accompanied by symptoms of damage to the mesentery, intestines, and c. n. s., kidney. The cutaneous variant is more typical for school-age children; in this case, isolated damage to small arteries of the muscular type and arterioles predominates. Along with the characteristic features of P. general symptoms in the skin and subcutaneous tissue, along the vessels (usually intercostal and abdominal wall), multiple painful nodules with a diameter of up to 1 cm are palpated. In 1/3 of patients, mainly on the lower extremities (Fig. 3), livedo racemosa appears with a tendency to gradually spread on the body. Trophic disturbances are possible.

Less common in childhood are the asthmatic (eosinophilic) and monoorgan variants of P. at. There is a special, infant variant of P. u., which occurs 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 stool (often with blood), hepatomegaly, erythrocyte riya, leukocyturia, normochromic anemia, neutrophilic leukocytosis.

Current of P. at. in children, as a rule, it is progressive, with damage to internal organs - heart, liver, gastrointestinal tract. tract, kidneys, etc. Richly developed organ vascularization in children contributes to the fact that angiitis with microthrombosis, microinfarction of some internal organs sometimes occurs with little symptoms, without pain.

Lifetime diagnosis of P. u. in children is quite complex due to the multiplicity and variety of combinations of lesions of various organs, which creates a polymorphic wedge picture.

Confirm the diagnosis of P. u. In children, as in adults, muscle and skin biopsies help. In some cases, selective arteriography of the heart, kidneys, and 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 syndrome - hemorrhagic vasculitis (see Schönlein-Henoch disease), Moshkovich disease (see), etc. .

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

Treatment for children and adults is similar. The optimal effective dose of glucocorticosteroids (1.5-3 mg/kg) is prescribed for thromboangia - 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. For 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 blood and the presence of hypercoagulation, heparin is prescribed in combination with corticosteroids and antispasmodics.

All children with P. u. are subject to dispensary observation, which includes ECG monitoring, testing of kidney function, etc. Vaccinations, administration 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 inf. diseases.

Forecast of the classic version of P. at. remains serious in children. Hron, the skin variant tends to last for many years.

Bibliography: Vorobyov I.V. and Lyubomudrov V.E. Periarteritis nodosa, M., 1973, bibliogr.; Lyubomudrov V. E., Basamygina L. Ya. and Mateeva K. M. Periarteritis nodosa in children, Pediatrics, Jvft 8, p. 76, 1960; Semenkova E. N. On the issue of overdiagnosis of periarteritis nodosa, Ter. arkh., t. 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, vol. 6, Jsfb 28, p. 157, 1926; aka, Collagenoses, M., 1965, bibliogr.; Tareev E. M. and Semenkova E. N. Asthmatic variant of periarteritis nodosa, Klin, med., t. 47, “Ns 7, p. 28, 1969; Teodori M.I., Alekseev G.K. and Shnyrenkova O.V. On the classification of systemic vasculitis, Ter. arkh., t. 40, no. 8, p. 22, 1968; Yarygin N. E. et al. Systemic allergic vasculitis, M., 1980, bibliogr.; A 1 d 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 s k e D. J. Extrahepatic manifestations of viral hepatitis, Amer. J. med. Sci., 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. Engl. 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 (pat. an.).

– systemic vasculitis, characterized by inflammatory-necrotic damage to the walls of small and medium-sized visceral and peripheral arteries. The clinical picture of periarteritis nodosa begins with fever, myalgia, arthralgia, which are accompanied by thromboangiitis, skin, neurological, abdominal, cardiac, pulmonary, and renal syndromes. To confirm the diagnosis of periarteritis nodosa, a morphological examination of skin biopsies is performed. Treatment uses corticosteroids, immunosuppressants, and cytostatics. The prognosis of periarteritis nodosa is largely determined by the severity of damage to internal organs.

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

Gastrointestinal lesions in periarteritis nodosa occur with nausea, diarrhea, and epigastric pain. In the complicated version, the development of pancreatic necrosis, jaundice, perforated ulcers of the stomach and 12p is possible. intestines, bleeding. Involvement of the nervous system is manifested by asymmetric polyneuropathy: muscle atrophy, pain in the projection of nerve trunks, paresthesia, paresis, trophic disorders. In case of severe lesions, strokes, meningoencephalitis, and epileptiform seizures are likely to occur.

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

A variant of asthmatic periarteritis nodosa occurs with persistent attacks of bronchial asthma, skin manifestations, fever, arthralgia and myalgia. The dominant manifestations of the cutaneous thrombangitic form of periarteritis nodosa are nodules, livedo and hemorrhagic purpura. Subcutaneous nodules are typically located along the vascular bundles of the extremities. These symptoms develop against the background of myalgia, fever, sweating, and weight loss. Periarteritis nodosa, which occurs as a monoorgan type, is characterized by visceropathy and is established after a histological examination of a biopsy specimen or a removed organ.

Complicated forms of periarteritis nodosa can be accompanied by the development of infarctions and organ sclerosis, rupture of aneurysms, perforation of ulcers, intestinal gangrene, uremia, stroke, and encephalomyelitis.

Diagnosis of periarteritis nodosa

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

To clarify the diagnosis of periarteritis nodosa, a biopsy is performed. A musculocutaneous biopsy of the abdominal wall or leg reveals inflammatory infiltration and necrotic changes in the vascular walls. With periarteritis nodosa, HBsAg or antibodies to it are often detected in the blood. When examining the fundus, aneurysmal changes in the vessels are detected. Ultrasound scanning of renal vessels determines their stenosis. Plain radiography of the lungs reveals an increase in the pulmonary pattern and its deformation. To diagnose cardiopathy, an ECG and ultrasound of the heart are performed.

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

Treatment of periarteritis nodosa

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 jointly by 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.

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

Preventive tasks include taking into account drug intolerance, reasonable and controlled immunization, blood transfusions, and protection against infections.