PERIARTERITIS NODULAR (periarteriitis nodosa; Greek peri around, near + arteritis; synonym: Kussmaul's disease — Maier, a nodular panarteritis) — a disease of the allergic nature from group of system vasculites with secondary angiogenic defeat of various bodies and systems and heavy vascular complications.
For P. at. damage of small and average arteries of muscular type with formation of vascular aneurisms («small knots») is characteristic thanks to what the disease and received the name. Because inflammatory process is not limited to an outside cover (adventitia) of a vessel, and takes all layers of a vascular wall, P. at. it is more correct to call a nodular panarteritis, however in the USSR the name a nodular periarteritis offered in 1866 by Kus-smaulem and Maier remains (A. Kussmaul, R. Maier). In Russia the description of the two first cases of P. at. belongs to A. P. Langovy (1883) working in clinic of the prof. A. A. Ostroumov. The intravital diagnosis of P. at. for the first time in our country E is put in 1926. M. Tareev at a biopsy of a hypodermic small knot.
The standard classification of P. at. does not exist. In the WHO classification (1980) P. at. it is carried to general vascular diseases. In the USA classification Zeke (R. M. of Zeek, 1953) is accepted in which allocate classical P. at., allergic P. at. with bronchial asthma and an eosinophilia, a giperergichesky angiitis at a drug and serum disease. Alrkon-Segovia (D.Alrcon-Segovia, 1977) suggests to distinguish generalized classical P. at. immune genesis, gipersensi-tivny limited (skin, renal, etc.) and allergic P. at. (eosinophilic angiitis).
The item at. carry to rare diseases. Engelbert (O. of Engelberth, 1962) on 41 478 openings (1939 — 1956) found P. at. in 0,13% of cases. However there is an accurate tendency to its increase. On section materials-tsy of Jones Gop-kinsa (Baltimore, 1926 — 1942) on the basis of A. R. Rich's data, P.'s increase is noted at. with 1: 1600 to 1: 137. By data I. V. Vorobyova and V. E. Lyubomudrova, P. at. men at the age of 21 — 60 years are ill preferential.
An etiology and a pathogeny
P.'s Aetiology at. it is definitely not established. The most widespread and conventional is the allergic theory explaining an origin of a disease with giperergichesky reaction of vessels to various antigenic influences. P.'s emergence is especially frequent at. connect with influence of various pharmaceuticals (streptocides, penicillin, thiouracil, aminazine, drugs of iodine, mercury) and administration of alien serums. Since 1970 the question of a possibility of a virus etiology of P. is discussed at. At the same time crucial importance is attached to formation of the cell-bound immune complexes consisting of a surface antigen of serumal hepatitis (HBsAg), antibodies to it and a complement and their adjournment in walls of vessels. D. J. Gocke et al., Gerber with soavt, described P.'s cases at. after the postponed HBsAg-positive hepatitis; at the same time the persistirovaniye of antigen was observed, and the cell-bound immune complexes supporting HBsAg in a wall of the affected arteries or muscles sometimes were found. According to D. J. Gocke, in 30 — 40% of cases of typical P. at. the persistirovaniye of HBsAg is observed.
P.'s pathogeny at. connect with immunopathological processes. Paronetto and Strauss (F. Paronetto, L. Strauss, 1962), applying a fluorescent technique, established availability of Y-globulin in arterioles of the patient with a nodular periarteritis. Roget and Marten (J. Roge, E. Martin, 1965) by introduction by an animal of blood serum from patients in an acute phase P. at. received at them the changes of vessels characteristic of this disease; similar changes were absent at introduction by an animal of blood serum of convalescents.
Inflammatory changes at P. at. appointments — in arteries of all calibers, and also in small and large veins are found in vessels of different level and various funkts, that testifies to the system nature of process. At the same time damage of arteries of muscular and myshechnoelastichesky type is leading. Inflammatory changes in vessels at P. at. represent manifestation of the immediate or slowed-down hypersensitivity (see. Allergy ) with immunocomplex or immunokle-exact mechanisms. Quite often their combination takes place owing to what vasculites are got by the mixed character and their morphology reflects all complexity of relationship of humoral and cellular allergic reactions. Immunopathological genesis of vasculites at P. at. is confirmed immunofluorescence (see) and submicroscopy (see). In particular, at a research of the material received from sick P. at. at a biopsy of a kidney, it is shown that the exacerbation of a disease is followed by fixing on basal membranes of vascular loops, in a mezangiya and a parietal layer of balls of kidneys of immunoglobulins (IgG, IgA, IgM), SZ-fractions of a complement and the fibrin giving a krupnogranulyarny or focal and linear luminescence. Electronic microscopically in renal balls of sick P. at. also subepiteli-alny deposits of cell-bound immune complexes which part fibrin is are found subendothelial, mezan-gialny, and occasionally. In the inflammatory changed vessels at P. at. the cell-bound immune complexes containing along with IgG (fig. 1, a) and a complement a surface antigen of a virus of hepatitis B come to light (fig. 1, b).
At gistol, and gistokhy, studying of biopsy and autopsiyny material it is established that morfol, changes in arterial vessels at P. at. develop in a certain sequence: Mucoid swelling of walls of vessels, fibrinoid changes up to a necrosis, the infiltrative and proliferative phenomena and a sclerosis of the affected arteries. Mucoid swelling (see. Mucous dystrophy ) it is caused by decomposition of the complexes of the main substance of connecting fabric dissociating proteinaceous polisakharidnykh with release of glikozaminoglikan that leads to increase in vascular permeability and to hydration of the main substance of this fabric. A fibrinoid necrosis (see. Fibrinoid transformation ) develops after plasmatic treatment of walls of arteries and it is characterized by loss in them the amorphous and filamentous mass of fibrin.
Against the background of disorganization of connecting fabric there is an inflammatory cellular reaction which is characterized by infiltration of walls of vessels and the connecting fabric surrounding them lymphocytes, macrophages, neutrophilic and eosinophilic granulocytes (fig. 2, a) in various quantitative combinations. With big constancy among cells of infiltrate at such vasculites also mast cells are found. Acute arteritis quite often comes to the end with formation of aneurisms (fig. 2, b). On мере^ subsiding of the exudative phenomena develop processes of proliferation and transformation of undifferentiated cellular elements of a gistiogenny and hematogenous origin therefore in walls of the affected arteries infiltrate — proliferat is formed. Along with lymphocytes and macrophages in infiltrate epithelial cells, fibroblasts, plasmocytes come to light. At increase of processes of a reparation prevailing in infiltrate there are cells of a fibroplastic row. In the outcome arise sclerosis (see) and hyalinosis (see) walls of arteries and arterioles.
Depending on a ratio in inflammatory reaction of altera-tivny, exudative or proliferative changes arteritis can be destructive, destruktivnoproduktivny and productive. Preferential localization patol. process in one of covers of a vessel gives the grounds to speak about endo-, meso - and a periarteritis. However it is frequent at P. at. it is necessary to state defeat of all three covers; in similar cases process is designated as a panarteritis. As of a disease it is characteristic hron, the recurrent current, Mucoid swelling, a fibrinoid necrosis, infiltrative and proliferative reactions sometimes arise also in the sclerosed arteries. The most serious consequence of arteritis at P. at. the progressing stenozirovaniye of the affected arteries is. Quite often in patholologically the changed vessels, especially with aneurisms, the fresh, organized or organized (channeled) blood clots are found (see. Blood clot ).
A vasculitis at P. at. develops at the same time or consistently in many bodies though vessels of kidneys, hearts, intestines, a brain and covers of nerves most often are surprised. As a result of arteritis and trombarteriit in various bodies and fabrics there are local changes: hemorrhages, dystrophy and an atrophy of parenchymatous elements, focal and necrotic and ulcer processes, heart attacks and hems after them, the sclerous and cirrhotic phenomena. In peripheral nerves owing to defeat of vasa sanguinea nervorum signs of a vallerovsky degeneration with destruction of axons and myelin covers in a combination with regenerative processes are found (see. Valera regeneration ).
Along with the arteritis described above the important place in P.'s pathology at. occupies an immune inflammation of vessels of microcirculation. So, allergic microvasculites are the cornerstone of various options of a glomerulonephritis, an alveolitis of a pneumonitis), a polyserositis. The inflammation of vessels of microcirculation has essential value in developing of necrotic enteritis, heavy displays of myocarditis, pancreatitis, hepatitis and especially neuritis and a miositis.
Prevalence of vasculites at P. at. and weight of the secondary changes in bodies and fabrics caused by them is varied considerably that causes kliniko-anatomic polymorphism of a disease. Due to the use for sick P.' treatment at. glucocorticosteroids and immunodepressants dominance of productive forms of a vasculitis is noted.
A clinical picture
For P. at. the wedge, symptoms complicating diagnosis is characteristic extreme polymorphism. The disease begins, as a rule, gradually with the general symptoms. The most characteristic of P. at. fever, the progressing weight loss and muscular and joint pains are. Among the general symptoms on the frequency (95 — 100%) is on the first place fever (see). At most of patients fever of the wrong type, temperature does not decrease at use of antibiotics, but quickly disappears under the influence of glucocorticosteroid hormones. Fever at the beginning of a disease differs in considerable persistence; at emergence of organ pathology it, as a rule, does not renew.
Exhaustion is extremely characteristic, almost patognomonichno of P. at. in an acute phase of a disease (chlorotic marasmus of Kussmaul — Maier). In some cases reduction of body weight reaches catastrophic figures (30 — 40 kg for several months), and degree of a cachexia is higher, than at onkol, diseases.
Mialgiya (see) and to a lesser extent arthralgias (see) occur at most of patients at the beginning of a disease. Gastrocnemius muscle and large joints pains are characteristic. There are permanent changes in joints similar externally much less often with pseudorheumatism (see).
Carry to the general displays of intoxication such characteristic of P. at. symptoms as the tachycardia which is not decreasing at reception of glycosides and perspiration.
Sometimes the disease begins with organ defeats, to-rye appear for several months and even years before emergence of system manifestations. Such «organ debuts» of P. at. can be the bronchial asthma proceeding with a hypereosinophilia, repeated myocardial infarctions at persons of young age, attacks of abdominal pains in combination with dispeptic frustration.
Among organ pathology, inherent P. at., allocate five most often found syndromes defining specifics a wedge, pictures of a disease — renal, abdominal, cardial, pulmonary and neurologic.
The renal syndrome occurs at 75 — 90% of patients. Emergence a wedge, signs of damage of kidneys demonstrates usually far come process. The most typical sign of damage of kidneys at P. at. arterial hypertension is (see. arterial hypertension ), in most cases stable, having a persistent current, sometimes galloping, with development of heavy retinopathies (see) and with loss of sight. Are observed a moderate proteinuria (1,0 — 3,0 g a day), a microhematuria. Occasionally the gross hematuria meets. Development of a nephrotic syndrome (the proteinuria is more than 3,0 g in days, peripheral hypostases) meets extremely seldom. The rupture aneurysmally of an expanded vessel of a kidney with formation of a pararenal hematoma is possible. The forecast of a renal syndrome is very serious: it can lead to development of a renal failure within 1 — 3 years.
An abdominal syndrome — the second io to frequency and the predictive importance; it is quite often observed at the beginning of a disease. The abdominal syndrome is shown by pains and dispeptic frustration. Abdominal pains have, as a rule, diffuse character, they constant, persistent, accruing on intensity. From dispeptic frustration diarrhea is most expressed (frequency of a chair to 6 — 10 times a day); in Calais impurity of blood and slime is noted. It is characteristic anorexia (see), sometimes nausea, vomiting. Quite often develops peritonitis (see) as a result of perforation of ulcers or gangrene of guts, sometimes arises gastrointestinal bleeding (see). Damage of a liver at P. at. it is observed rather seldom and characterized by development of heart attacks and a rupture of aneurisms of intra hepatic vessels. Development hron, hepatitis or cirrhosis at P. at. it is caused hron, a viral infection (a virus of serumal hepatitis) that is confirmed by data serol, a research and an intravital biopsy of body. Damages of a pancreas and a gall bladder come to light more often at a pathoanatomical research, however symptoms can be found in certain patients with system displays of a disease pancreatitis (see) or cholecystitis (see).
The cardial syndrome is characterized preferential coronaritisis (see) also occurs at 50 — 70% of patients. Clinically it is sometimes difficult to differentiate the damage of heart caused by P. at., from the secondary changes caused by heavy arterial hypertension. Coronary disturbances often proceed asymptomatically, are not followed by anginous pains even in case of focal damages of a myocardium. Melkoochagovye myocardial infarctions (see) meet more often, than macrofocal. Development of a peculiar angiogenic damage of heart as quickly progressing is characteristic cardiosclerosis (see) with disturbance of a rhythm, conductivity and heart failure. The only cause of death damage of heart happens not often. A possibility of defeat of an endocardium at P. at. is a controversial issue.
The pulmonary syndrome is observed in 30 — 45% of cases and can be shown by symptoms bronchial asthma (see) with a hypereosinophilia, eosinophilic pulmonary infiltrates like Leffler (see. Lefflera syndrome ), vascular pneumonia, is more rare than intersticial pulmonary fibrosis (see. Pneumosclerosis ) or heart attack lung (see). At vascular pneumonia cough is followed by department of scanty quantity of a mucous phlegm, occasionally a pneumorrhagia; fever, the accruing signs of respiratory insufficiency are noted. Radiological in lungs — the sharp strengthening of the vascular drawing reminding a congestive lung infiltration of pulmonary fabric is preferential in radical zones. Small efficiency of antibiotics and high — glucocorticosteroid therapy can be an important diagnostic character.
The neurologic syndrome (defeat of the central and peripheral nervous system) is caused by system inflammatory changes of walls of vessels of a brain and covers of nerves. Vessels of c. N of page are surprised, according to section data, in 70% of cases, and a peripheral nervous system — in 12 — 25% of cases. Nevertheless defeat of a peripheral nervous system is the most characteristic and diagnostically an important symptom of P. at. Mononeurites and asymmetric neuritis are observed (see. Neuritis , Polyneuritis ). The polyneuritis as ascending Landry's paralysis is occasionally noted (see. Landry the ascending paralysis ). Usually peripheral nevrol, disturbances develop gradually: at first there are pains and paresthesias in distal departments of hands and legs, then muscular weakness joins. Morbidity of muscles is constantly observed, disorders of sensitivity on radicular and polyneuritic type are more often sural.
Wedge, picture of defeat of c. N of page of a polimorfn. The main manifestations can sharply develop with emergence of all-brain and focal symptoms on type stroke (see). Sometimes along with focal symptoms epileptic seizures, occasionally epileptic status are observed (see. Epilepsy ), symptoms of subarachnoidal and subdural hemorrhages. In some cases defeats of a nervous system proceed under the guise of dynamic disturbance of cerebral circulation (see. Crises ) or remind slowly progressing cerebral atherosclerosis with the accruing dementia (see. Weak-mindedness ). Cherepnomozgovye (cranial, T.) nerves are surprised rather seldom, preferential visual and front. Neuritis of optic nerves, passing decrease in visual acuity, narrowing of arteries of a retina, hypostasis of disks are observed.
At 15 — 30% of sick P. at. the skin changes which are characterized by existence of small knots on the course of vessels, various size a tree - the visible branching loops of cyanotic-red color without peeling — livedo racemosa meet (see. Livedo ) or yazvennonekrotichesky 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 shape 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 and polyneuritic — damage of kidneys with high arterial hypertension in combination with an asymmetric motive polyneuritis; renal abdominalnoserdechnogo — damage of kidneys with high arterial hypertension, abdominalgiya with dispeptic frustration, damage of heart (a coronaritisis with diffusion and focal changes on an ECG) with the progressing heart failure; pulmonary and cordial and renal, often beginning in the form of gi-pereozinofilny asthma or a pneumonitis; pulmonary polyneuritic go, the polyneuritis beginning with bronchial asthma with further accession.
Domination in P.'s clinic at. or several specified syndromes a number of clinical options of a disease allows to allocate one.
Classical (pochech-but - polyneuritic, or polyvisceral) the option begins, as a rule, with fever, muscular and joint pains, the expressed weight loss. In a wedge, a picture into the forefront act damage of kidneys with arterial hypertension, often malignant; a coronaritisis which combination to arterial hypertension leads to bystry development of heart failure, and also an abdominal pain and a polyneuritis. Damage of lungs proceeds as vascular pneumonia and meets not so often. In blood serum sometimes find HBsAg, and at a biopsy of a liver — signs hron, active hepatitis or cirrhosis. Presence of cell-bound immune complexes at blood serum, bodies and fabrics is possible.
Asthmatic, or eosinophilic, the option is abroad known as an allergic granulematozny angiitis or as a syndrome of Cherga — Strauss (J. Churg, L. Strauss). More often women are ill. The disease begins with attacks of bronchial asthma, it is often preceded by intolerance of pharmaceuticals and other displays of an allergy. Asthma is followed by a high eosinophilia (50 — 85%) at a leukocytosis of 20 Ltd companies — 35 Ltd companies. Are possible the fever accelerated by ROE. In 1 — 5 years there occurs generalization of process with development of the polyvisceral symptomatology inherent to classical option P. at. In half of cases the disease proceeds without damage of kidneys, being limited to peripheral neuritis, change of skin or disturbances from outside went. - kish. path. Along with symptoms of bronchial asthma quite often in lungs eosinophilic infiltrates are observed.
The skin option of a nodular periarteritis is shown by damage of skin with formation of typical small knots on the course of vessels of the size of prosyany grain and lentil, painful at a palpation. Skin manifestations are accompanied, as a rule, by mialgiya, fever, the accelerated ROE, anemia, a leukocytosis. In certain cases along with nodular educations there is a livedo (livedo racemosa), necroses of soft tissues, mucous membranes, and also gangrene of extremities develops. Skin P. at. seldom is complicated by damage of internals.
The monoorgan option meets extremely seldom and is shown by defeat of one body (kidneys, an appendix, a gall bladder). 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.
Characteristic lab. tests or pathognomonic symptoms (except a pan-vasculitis of arteries of the average caliber with formation of aneurisms revealed at a biopsy of fabrics, for example skeletal muscles) allowing to establish the correct diagnosis do not exist. At P. at. in blood the neutrophylic leukocytosis, the accelerated ROE, in certain cases anemia and an eosinophilia are observed. The item at. also other manifestations of a nonspecific inflammation, such as disproteinemia are inherent (see. Proteinemia ), a hypergammaglobulinemia (see. Dysgammaglobulinemia ), emergence C-reactive protein (see). These indicators reflect hl. obr. the degree of activity of process, their diagnostic value is usually small. The main criterion in diagnosis is typical the wedge, symptomatology. The attention to dominance among sick men of middle age, the typical acute beginning of a disease and a combination of several syndromes is paid. Changes in haemo microcirculation at P. at. can be revealed by means of microscopic examination of a conjunctiva. In the period of an exacerbation of a disease they are shown by dystonia of microvessels, reduction of number of the functioning capillaries, disturbance of rheological properties of blood, increase in vascular permeability. At survey of vessels of an eyeground small knots and aneurisms can be revealed.
The biopsy of skin or muscular tissue is reasonable only in cases of the expressed mialgiya (in an acute phase of a disease) or at changes of skin. Negative takes of a biopsy do not contradict clinically reasonable P.'s diagnosis at., as damages of muscles have, as a rule, focal character. At assessment of results gistol, researches pay attention to prevalence, depth and weight of vasculites as moderate changes of vessels meet at a number of diseases of internals and can be caused by also glucocorticosteroid therapy.
In not clear cases there can be a need of a biopsy - whom - or body. The issue in each case is resolved individually. A biopsy of a kidney at P. at. it is dangerous in connection with a possibility of bleeding (aneurism of vessels, high the ABP). The biopsy of a lung is not always possible because of serious condition of patients. The carrying out an arteriogra-fichesky research with contrasting of vessels of kidneys, hearts etc. allowing to reveal aneurysmally expanded vessels that patognomonichio for P. at is in some cases reasonable.
Differential diagnosis The item at. it is especially difficult at the beginning of a disease when there is no organ pathology. Most often patients are treated concerning assumed inf. diseases high doses of antibiotics that worsens their state. The differential diagnosis should be carried out with some forms of tumors, napr, a hypernephroma kidneys (see), cancer pancreas (see), to-rye also proceed with fever, mialgiya or a thromboangitis, weight loss.
In an initial stage a clinical picture P. at. it can be similar to long septic endocarditis (see) or lymphogranulomatosis (see). For P. at. oznoba, as are not characteristic at a long septic endocarditis, or profuse sweats and an itch, as at patients with a lymphogranulomatosis.
Patients with abdominal forms P. at. often get in surgical or inf. a hospital with suspicion on acute abdomen (see), dysentery (see) or others inf. diseases. In similar cases it is always possible to reveal, in addition to abdominal pains, any other symptomatology: a polyneuritis, damage of kidneys or bronchial asthma with a high eosinophilia. Nephrites with arterial hypertension and various associated diseases quite often take for P. at., disregarding that at the first stages P. at. the lab is, as a rule, shown by fever, weight loss, mialgiya and changes in data. researches that is unusual for nephrite.
Till 50th only symptomatic therapy of P. was carried out at. In 1949 the first message on successful use in treatment of a disease of glyukokortiko-steroid hormones appeared. However further observations showed that use of glucocorticosteroid hormones for sick P.' treatment at., proceeding with a renal syndrome, can lead to progressing of arterial hypertension and development of a heart and renal failure. In this regard at P. at. with damage of kidneys it is reasonable to apply glucocorticosteroid hormones in average doses (Prednisolonum of 30 —-40 mg a day) only in an early phase of a disease, before formation of permanent organ changes and in the absence of arterial hypertension.
In view of the immune mechanism of a disease, apply the combined therapy by glucocorticosteroid hormones and cytostatics. The positive effect at such treatment, according to literary data, is reached in 84% of cases. The indication to purpose of tsitostatik at P. at. resistance or an aggravation of symptoms of the patient at treatment are Prednisolonum, options of a disease with damage of kidneys. At the choice of treatment drugs from group of antimetabolites (Azathioprinum) or the alkylating means (Cyclophosphanum, Chlorbutinum) can be used, in hard cases the combination of two tsitostatik is possible. Apply Azathioprinum in a dose of 150 — 200 mg a day within 1 — 2 month and Prednisolonum more often (15 — 20 mg a day) with transition in the subsequent to a maintenance therapy in out-patient conditions (Prednisolonum of 10 — 15 mg, Azathioprinum of 50 — 100 mg a day). At good tolerance and lack of side reactions the maintenance therapy should be carried out it is long, within several years, raising a dose of drugs to therapeutic during the periods of a recurrence of a disease.
At asthmatic option P. at. without damage of kidneys in an acute phase of a disease appoint higher doses of Prednisolonum (to 40 — 50 mg a day), then reduce a dose to supporting (5 — 10 mg a day) and apply within several years.
At P. at. without strong indications of damage of internals it is necessary to appoint Prednisolonum (15 — 20 mg) only in an acute phase of a disease to short term (1 — 2 month).
Good results are yielded by treatment by Butadionum (0,45 g a day) or 5% solution of Pyrabutolum (on 1,0 ml intramusculary within 1 — 2 month). At contraindications to treatment by cytostatics Butadionum can be used also at visceral forms P. at. in combination with small doses a glucocorticosteroid-nykh of hormones. At defeat of peripheral vessels with a gangrenosis appoint antikoagulyai-you (heparin — 20 000 PIECES intramusculary), spasmolysants. 4-Aminokhinolinovye drugs are used only at hron, disease in combination with other drugs. Treatment consists, in addition to the main therapy, in appointment аденила^ performing massage and LFK of cm the Polyneuritis. P.'s treatment at. Provo - * ditsya continuously also is long.
The forecast and Prevention
the Forecast is serious at classical option of a disease, however in connection with use of modern methods of treatment and rational prevention terms of life of sick P. at. were considerably extended. The wedge, remissions within several years are possible, however patients with renal forms of a disease, as a rule, remain disabled. The forecast at asthmatic option P. is more favorable at. without damage of kidneys: longevity of this group of patients is estimated decades, a part them is returned to work. The forecast at skin option P. at. favorable.
Prevention. Specific prevention of P. at. it is not developed. It is necessary to remember that hemotransfusions and plasma and their substitutes, vaccination and administration of alien serums, physiotherapeutic procedures, insolation can cause an exacerbation of a disease.
Features of a nodular periarteritis at children
At P. children at. develops less than at adults. Children of any age, preferential early children's and school, girls and boys — with an identical frequency are ill.
Pathoanatomical features are caused by an originality of a current at children of inflammatory and allergic reactions, and also age features of a structure of vessels and fabrics: abundance of cellular elements and relative structural immaturity of vascular walls, rich vascularization of internals. The bright picture of a necrotic angiitis — a panarteritis with development of multiple aneurisms is characteristic; thromboangites, heart attacks of various bodies are frequent.
A clinical picture generally same, as at adults. The beginning is acute, with the expressed giperergichesky component, bright reaction of immunocompetent system: increase limf, nodes, and also a spleen is noted (at 1/3 patients). In an active phase the general symptoms prevail: the fever of the wrong type which is not giving in to treatment by antibiotics and antipyretics, the increasing weakness, falling of weight. Mialgiya and arthralgias are characteristic, the asymmetric polyneuritis, arthritises are less often noted. From skin defeats capillarites of palms and soles, hemorrhagic rashes, necroses of skin, the general and localized (it is preferential on extremities) dense Quincke's diseases are most frequent a livedo. Defeats of c. N of page proceed, as well as adults, have an aseptic serous meningitis more often (without changes of protein content and sugar in cerebrospinal liquid). The pulmonary syndrome develops less often. The abdominal syndrome is most expressed at children of early age and, as a rule, is followed by intestinal bleeding. Arterial hypertension is observed at 1/4 patients. Cardial, renal, neurologic syndromes, and also the main laboratory indicators at children and at adults have no fundamental differences. Normokhromny anemia, a neutrophylic leukocytosis, an eosinophilia and plasmatization of marrow, a disproteinemia with increase in level of gamma-globulins, IgM, IgG, fibrinogen comes to light.
Clinical options P. at. at adults and children are generally identical. The polyvisceral option which, as a rule, is followed by symptoms of defeat of a mesentery, intestines, c is more typical for children classical renal and polyneuritic a yawl. N of page, kidneys. The skin option is more characteristic of children of school age; in this case the isolated damage of small arteries of muscular type and arterioles prevails. Along with characteristic of P. at. the general symptoms in skin and hypodermic cellulose, on the course of vessels (is more often intercostal and an abdominal wall) palpate multiple painful small knots to dia, to 1 cm. 1/3 patients preferential on the lower extremities (fig. 3) have livedo racemosa with a tendency to gradual distribution on a trunk. Trophic disturbances are possible.
Asthmatic (eosinophilic) and monoorgan options P. are less inherent to children's age at. Allocate special, infantile option P. at., which proceeds with long fever of the wrong type, catarral changes of a mucous membrane of upper respiratory tracts, polymorphic skin rashes, dense Quincke's diseases, arthralgias, mialgiya, tachycardia, signs of a coronaritisis, increase in ADG abdominal pains, vomiting, an enterokolitichesky chair (it is frequent with blood), a hepatomegalia, to an erythrocyte a yard, a leukocyturia, normo-hromny anemia, a neutrophylic leukocytosis.
P.'s current at. at children, as a rule, progressing, with damage of internals — hearts, a liver, went. - kish. path, kidneys, etc. Richly developed organ vascularization at children promotes that the angiitis with microfibrinferments, microheart attacks of some internals sometimes proceeds malosimp-languidly, without pain.
The intravital diagnosis of P. at. at children it is quite difficult because of plurality and a variety of combinations of defeats of various bodies that creates polymorphic a wedge, a picture.
To confirm P.'s diagnosis at. at children, as well as at adults, these biopsies of muscles, skin help. In some cases carry out the selection arteriography of heart, kidneys, mesenteric vessels.
Differential diagnosis of P. at. at children includes a wide range of diseases: lymphogranulomatosis (see), acute leukosis (see), sepsis (see), viral and bacterial infections, collagenoses — system lupus erythematosus (see), system scleroderma (see), dermatomyositis (see), and also pseudorheumatism (see), Wegener's granulomatosis (see. Wegener granulomatosis ), the diseases complicated by development of a syndrome of widespread intravascular coagulation — a hemorrhagic vasculitis (see. Shenleyna — Genokh a disease ), Moshkovich disease (see), etc.
Considerable difficulties are caused by differential diagnosis of an abdominal syndrome at P. at. with invagination, a syndrome of widespread intravascular coagulation with a hypoxemic necrotic coloenteritis, intestinal infections, hepatitis.
Treatment at children and adults is similar. Optimum effective dose of glucocorticosteroids (1,5 — 3 mg/kg) at is appointed at thromboangites — to 5 — 7 mg/kg a day. Later 4 — 6 weeks the dose is gradually reduced to individual supporting which is cancelled only in a phase of permanent clinical laboratory remission. At an abdominal, neurologic, renal syndrome with hypertensia glucocorticosteroids are ineffective. It is recommended to combine them with cytostatic drugs (Azathioprinum, Cyclophosphanum). In case of change of rheological properties of blood and existence of hypercoagulation appoint heparin in a combination with corticosteroids and spasmolysants.
All children with P. at. are subject to dispensary observation, a cut includes control of an ECG, a research of functions of kidneys, etc. Inoculations, administration of serums and other possible allergenic factors are excluded. Preventive actions shall be directed to the prevention of development of allergic reactions, reduction of frequency and weight of a current inf. diseases.
The forecast of classical option P. at. at children remains serious. Hron, skin option tends to a long-term current.
See also Vasculitis .
Bibliography: Vorobyov I. V. and Lyubomudrov V. E. Nodular periarteritis, M., 1973, bibliogr.; JI yu about m at the Dr. about in V. E., Basamygin JI. I. and Mateeva K. M. A nodular periarteritis at children, Pediatrics, Jvft 8, page 76, 1960; Semenkova E. N. K to a question of hyper diagnosis of a nodular periarteritis, Rubbed. arkh., t. 47, No. 4, page 122, 1975; With t r at-kov A. I. and Beglaryan A. G. Pathological anatomy and pathogeny of collagenic diseases, M., 1963; To Tara-e in E. M. K to clinic of a nodular periarteritis, Russian clinic, t. 6, Jsfb 28, page 157, 1926; it, Collagenoses, M., 1965, bibliogr.; Tareev E. M and Sam of the Tax Code about in an E. H. Asthmatic option of a nodular periarteritis, Klin, medical, t. 47, «Ns 7, page 28, 1969; Teodori M. I., Alekseev G. K. and Shnyrenko-v and O. V. O of classification of system vasculites, Rubbed. arkh., t. 40, No. 8, page 22, 1968; I r y and N of H. E. and d river. System allergic vasculites, M., 1980, bibliogr.; And 1 g with n-S e g about v i a D. The necrotizing vasculitides, Med. Clin. N. Amer., v, 61, p. 241, 1977, bibliogr.; Churg J. Strauss L. Allergic granulomatosis, allergic angiitis and periarteritis nodosa, Amer. J. Path., v. 27, p. 277, 1951; E t t linger R. E. and. o. Poliarteritis nodosa in childhood, Arthr. Rheum., v. 22, p. 820, 1979; G o with k e D. J. Extrahepatic manifestations of viral hepatitis, Amer. J. med. Sci., v. 270, p. 49, 1975; Kussmaul A. u. M an 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. Solomatin (ped.), JI. M. Popova (not BP.), H. E. Yarygin (stalemate. An.).