Nodosa nadasny

Nodosa Nowotny (synonym: polyarteritis nodosa, illness, Kussmaul - Meier) is a systemic disease, which is based giperergicakie the defeat of arteries, mainly muscular type.
Nodosa nadasny considered one of the most rare species of collagen diseases (see). Sick more often men aged 20-50 years.
Nodosa nadasny manifested giperergicakie reaction of the body, particularly cardiovascular system, in response to different allergens, often toxicoinfections origin. There are known cases of development nadeznogo of periarteriit after consumption of various drugs, especially sulfanilamidov, antibiotics, serums, vaccines. Morphologically defeat at nodosum the nodosa is an allergic vasculitis (see will Sekulic).
The clinical picture. The disease often begins acutely - with fever, pains of various localization (in the joints, heart, abdomen, and so on), skin rashes. Characterized by growing drain. In some cases the disease can begin with the defeat of the heart, kidneys, or nervous system.
When the skin lesions are sometimes there is a variety rash: erythematous, urticarnae, maculopapular, and others, as well as the characteristic nadeznogo of periarteriit subcutaneous nodules. Often affects the joints: more frequent arthralgia or arthritis. Articular syndrome is usually accompanied by myalgia, muscle soreness at palpation, convulsions, muscle wasting and weakness.
In case of defeat of coronary arteries and cardiac muscle marked pain in the heart, shortness of breath, tachycardia, cardiac rhythm, changing of borders of heart, muting tone. Coronary artery disease causes breast toad (see) and myocardial infarction (see).
Kidney damage is observed quite frequently. Develops diffuse picture of jade, heart attack, kidney, intra - or perinephral bleeding in connection with the formation of renal artery aneurysms; there hypertension and quickly progressing to renal failure.
With the defeat of vessels of the stomach pain in epigastria, nausea and vomiting with blood, with the defeat of vessels of the small intestine - cramping around the navel, diarrhea, blood in Calais: the localization of lesions in the colon - lower abdominal pain, constipation alternating with diarrhea, blood in the stool, tenesmus.
Lung damage is manifested by symptoms of asthma or pneumonia.
With the defeat of the nervous system occur mono - or polyneuritis. It is also possible the defeat of the Central nervous system (meningoencephalitis) and a change of mentality.
For nadeznogo of periarteriit often long, wavy, with repeated exacerbations and involvement in the process of new organs and systems.
The prognosis is unfavorable. However, in the case of relatively benign disease occasionally observed recovery.
Nodosa nadasny in children is less common than in adults, and is characterized by undulating and progressive course. The prognosis is poor in most cases, the disease duration does not exceed three months. As adults, Nowotny nodosa in children is characterized by a variety of symptoms.
The treatment is carried out in the hospital. Antisense and anti-inflammatory drugs in combination with vitamin.


Nodosa Nowotny (periarteriitis nodosa; synonym: nodosa knotted, or nodular, periarteriit knotted, illness, Kussmaul - Meier) is a common disease, which is based system defeat giperergicakie nature of small arteries and arterioles in different organs.
For a long time nadasny nodosa was considered a rare disease. Currently in the literature cited thousands of cases.
The etiology and pathogenesis. Regarding the nature nadeznogo of periarteriit there were different views. Currently, no single etiology and allergic nature of suffering. For the first time this view was put forward Gruber (G. Gruber), who believed that the P. N. is not etiological outlined by disease, but by the manifestation of giperergicakie reaction segments of the arterial vessels that have acquired increased sensitivity (sensitized by various allergens, often toxicoinfections origin), during the preceding infectious diseases. This view has been widely recognized and confirmed in clinical observations of the development of localized changes arteries with a characteristic pattern P. N. in many infectious diseases without systemic nature of the process. There are known cases of development of P. N. after consumption of various drugs, especially sulfanilamidov, antibiotics, after the introduction of therapeutic sera as a manifestation of allergic reactions to these substances, as well as on the background of bronchial asthma.
Changes in the arteries, characteristic nadeznogo of periarteriit, appear to experiment by local allergic reactions. P. N. as a systemic disease also described some animals (deer, pigs). In connection with the development of the doctrine of collagen diseases (see Collagenoses) P. N. considered as one of the varieties of this group of diseases.
Pathological anatomy. Element of lesions P. N. is giperergicakie the Takayasu or necrotizing of angia.
The most important component it is fibrinoid necrosis (see Fibrinogene transformation), developing mainly in the middle layer of the vessel and covering any part of the perimeter of the vessel, or annular a limited section of the artery. On the affected area, the vessel wall loses its fibration and lamination and turns into homogeneous or fine eosinophilic and Perinatology mass, which at a special colouring can be detected particles of elastic fibers. To fibrinoidnogo necrosis quickly joins inflammatory infiltration of the vessel wall, consisting of neutrophilic and eosinophilic white blood cell and histiocytes in different ratios. Thick cell infiltration permeates all layers of the wall; the most characteristic feature is the localization of the changes in the outer layers and on the periphery of the vessel (hence the name of the disease - nodosa). Inflammatory and necrotic process is often accompanied by the formation of fine-grained blood clot in the affected area. Often the structure of the vessel erased so that all of it on the cross-section has the form of a bundle, consisting of a structureless eosinophilic mass of fibrin and fibrinoid, in which the presence of the elements of the vascular wall is documented only identifying elastic membranes. As the process involves all layers, not only external, it is essentially panarteritis. The normal consequence of the destruction of the walls of arteries - the development of aneurysmal swelling in the affected area with deformation of the vessel and the eccentricity of the lumen. In the further stage of development there is a gradual increase of fibrosing processes with the turning of the vessel in the fibrous nodules with subsequent hyalinosis connective tissue. In this bundle can be detected narrow the lumen of the artery, lying Central or eccentric; sometimes during complete obliteration of this clearance is completely indiscernible. Such an outcome is found in the natural course of a disease, but it is especially relevant in cases where were conducted intensive therapy with corticosteroids.
P. N. a systemic disease, covering different, sometimes many vascular pools of the body. However, there are individual election or preferential localization in certain blood pool, which determines the variety of clinical manifestations P. N. When the generalization of data of numerous observations, we can establish the following number frequency (downwards) organ localization P. N.: heart-kidney liver (especially the bed of the gallbladder)-spleen-pancreas-skin-adrenal - skeletal muscles and peripheral nerves - bowel-light. It is extremely rare changes of cerebral vessels. In addition to these organs, changes characteristic of nadeznogo of periarteriit, are found in the sex glands, bladder, thyroid, etc. of the Defeat of the above mentioned bodies are found in various combinations and sequences. Sometimes affected in isolation only a limited area.
In advanced lesions of the vasa vasorum along the major arterial branches (the coronary arteries of the heart, mesenteric artery and others) last dotted with getcoursename Burling, giving them uzlovatom. Aneurismatic extended with periarterial infiltration and sklerozirovanie small arteries be felt in the form of nodules value to pea. Such knots and felt in the skin when it is defeated. Biopsy them with histological examination are sometimes crucial diagnostic value. Ordinary chronic and wavy course of the disease its display has a variety find at postmortem and pathohistological examination of changes. Along with a complete process in the form of fibrous obliteration vessels meet fresh defeat deathly-inflammatory nature and various transitional morphological phase. In cases of active therapy fresh changes may be missing.
The affected organs nodosum the nodosa, develop changes associated with focal arterial ischemia. In the myocardium found small infarctions different prescription - from fresh to fully healed, respectively duration and wolnoobraznae disease. In the crust of the kidneys find characteristic multiple zapadenia and ingrown scars after heart attacks, giving the surface of the kidney bumpy appearance. In the spleen, heart attacks different prescription and scars after heart attacks. In skeletal muscles - atrophic processes caused by impaired blood circulation and trophic innervation in connection with damage of nerve trunks. In the last picture degeneration with the collapse of myelin and axial cylinders. In the intestines - flat interceptable, often triangular in shape ulcers. In the pancreas, adrenal - atrophic and sclerotic processes.
Clinical course and symptoms. The clinical picture nadeznogo of periarteriit motley, which is largely determined by a variety of localization of vascular lesion, disease duration and sequence of involving in pathological process different areas of the vascular system. It consists of the manifestations of General toxic and allergic nature and symptoms of some organs and systems. Clinic "classic" nadeznogo of periarteriit (diseases of Kussmaul - Meier) is slightly different from P. N. "modern" (with E. M. Tareev)to which a number of researchers relates also nekrotizirutee arteritis hypersensitive origin (medicinal, whey, vaccination, parasitic and other).
Of the common symptoms of P. N. most constantly occur: fever, muscle (less joint or muscle and joint) pain, asthenia, growing drain. Temperature can be subfebrile, wrong remitting with large daily swings, undulating; it generally stable during the treatment by antibacterial remedies.
In subacute and protracted period of nadeznogo of periarteriit there is growing drain, sometimes reaching extreme levels. To the phenomena of a General nature can be related skin changes, hematologic changes and hyperplasia of the reticuloendothelial gistiotitarnaya system with enlarged lymph nodes and spleen. A characteristic skin and subcutaneous nodules in the magnitude of poppy to the millet grain (less large size), located mainly along the vessels; the eruption of them often occurs in acute condition of a disease, they keep for long. Change of blood celebrate anemia, neutrophilic leucocytosis with more or less pronounced nuclear shift to the left, eosinophilia, persistent and significant acceleration ROHE. Often detect a moderate hypergammaglobulinemia due to the increase of alpha - and gamma-globulin fractions.
The heart. The defeat of coronary artery disease and myocardial occupy one of leading places in the pathology P. N. Most persistent signs of heart disease are tachycardia (independent of temperature and is not appropriate) and various non-specific ECG changes, indicating hypertrophy or another Department of the heart (usually the left) with the indication of overload that Department or ischemic changes connected with insufficiency of blood supply to the hypertrophied infarction due to narrowing of the lumen (obliteration, thrombosis) the affected coronary arteries. Only with the development of necrosis or massive myocardial infarction ECG taking a pronounced "infarction". Coronary breaking often asymptomatic or atypical. See also dystrophic and (much less frequently) inflammatory changes infarction with occurrence in the interstitium of cell infiltrates or granulomatous lesions. Cardiac rhythm and conductivity (ekstrasystolichniy arrhythmia, rarely atrial flutter or atrial fibrillation, disorders intraventricular conduction with the blockade feet and others)as heart failure, usually are the result of progressive angiogenic myocardiosclerosis or the condition of the hypertrophied heart. The defeat of the endocardium observed only in separate cases in terminal period of disease on the background izotermicheskoi uremia as warty aseptic thromboangiitis. The defeat of the pericardium (and other serous membranes) as a separate process is not typical for nadeznogo of periarteriit, however, occurs in connection with myocardial infarction or at uremia. The most serious complication is bleeding in the pericardial cavity resulting from the breakage of the aneurysm subepicardial branches of the affected coronary arteries.
Arterial hypertension is common and usually caused by loss of renal vessels.
The most common variant of kidney damage - multiple ischemic heart attacks at different stages, which are shown periodic more or less pronounced mikrogematuria, and sometimes pain in the lumbar region and dysuria, simulating an attack of urolithiasis. See also a focal or diffuse glomerulonephritis with acute or subacute course, rarely malignant nitroanisoles syndrome malignant hypertension, hypertensive retinopathy and rapidly progressive renal failure. Often failure of the kidneys long time runs hidden. Dangerous complication P. N. is ruptured aneurysm branches renal artery education perinephral or subcapsular hematoma, usually accompanied by a sharp pain in the lumbar region (on the affected side), collapse, fall in hemoglobin.
Vascular lesions of the gastrointestinal tract and mesentery very often observed in nodosum the nodosa and seen the picture of acute or chronic diseases of abdominal organs, appendicitis, cholecystitis, pancreatitis, acute intestinal obstruction (mesenteric thrombosis) with abdominal pain, symptoms of peritoneal irritation, temperature reaction, leukocytosis. Vascular lesions of the mucous membrane of the stomach and intestines often leads to the development of ulcero-necrotic processes, complicated by bleeding or perforation of the affected organs. Mesenteric thrombosis of arteries usually results in the formation of heart attacks and necrosis of the intestinal wall.
Vascular lesions of the pancreas is manifested functional disorders and pain, less likely to develop diabetic syndrome; when thrombosis occur infarction and necrosis of pancreatic cancer. Ruptured aneurysm of the arteries feeding the pancreas, accompanied by massive bleeding into the thickness of the pancreas.
Changes in the liver are expressed in the development of small - or krupnooptovyh necrosis, heart attacks, cerebral hemorrhage. Rarely observed diffuse hepatitis with jaundice. The defeat of the cystic artery thrombosis it can cause necrosis of the gallbladder Wall with a picture of acute cholecystitis (often gangrenous) and punching him.
On the grounds of vascular lesion of the lungs (bronchial artery branching pulmonary artery) occur bronchitis, necrosis, heart attacks the lungs with secondary involvement of the pleura, bronhospasticskie syndrome with a picture of a bronchial asthma. When hypersensitive version nadeznogo of periarteriit in the lungs can occur eosinophilic infiltration. The result of all these processes may be developing focal or diffuse pulmonary fibrosis pulmonary-cardiac insufficiency.
The defeat of the ramifications of the splenic artery observed in almost one third of cases of P. N., may be complicated by thrombosis them with the development of heart attacks spleen. Breaks aneurysms branches of the splenic artery lead to massive internal bleeding.
The peripheral nervous system refers to the common manifestations (from 20 to 40% of all cases) P. N. arise mono - or polyneuritis, often symmetrical with a firearm or burning pain in the extremities, paresthesia, progressive atrophy of the muscles of the extremities, lower tendon reflexes, the development of peripheral paresis and paralysis. Typical distal type of lesion.
The Central nervous system occurs by P. N. rarer and appears transient or persistent violations of cerebral circulation due to spasm or thrombosis of the affected vessels, small or large in the subdural hemorrhages, subarachnoid space, or in the substance of the brain. Less frequent are meningoencephalitis with corresponding symptoms.
The defeat of vessels of the eye is observed in 10-20% of cases of P. N. thus meet ptosis, exophthalmos, episclerit, irity, uveitis, horioidit, neuritis and optic nerve atrophy. The most characteristic P. N. thrombosis of the Central retinal artery, aneurysm of the retina, and perivascular nodules (detected when ophthalmoscopy).
From the endocrine glands most often affects the arteries of the testicles, adrenal glands, thyroid, pituitary, ostrovkovogo apparatus pancreas with the development of hemorrhage, necrosis, heart attacks. Clinic endocrine lesion is usually little or marked, or is absorbed diverse symptoms of the disease.
Complications. For nadeznogo of periarteriit often protracted, often relapsing with repeated exacerbations and remissions of various duration, coming spontaneously or with treatment. In some cases the disease for a long time runs hidden or blurred pronounced individual symptoms (subclinical forms) and suddenly manifested sudden catastrophic development of heavy (in most cases fatal) complications in the form of massive internal bleeding, bleeding in the brain, acute abdominal catastrophe (perforation of the stomach or intestines, mesenteric thrombosis of arteries), acute coronary insufficiency with myocardial infarction or without it.
Diagnosis nadeznogo of periarteriit difficult, however, when deployed clinical picture of the disease is recognized in 60-70% of cases. All cases of prolonged fever of unknown Genesis accompanied by symptoms of many organs and systems, polyneuritis and exhaustion must raise suspicion on P. N. extensive diagnostic tool is the detection of subcutaneous nodules and biopsy.
Forecast despite the introduction of new active funds, is still unfavorable.
Treatment. Antisense and anti-inflammatory drugs in combination with symptomatic therapy. Prescribe steroid hormones napochechnikov - prednisone, prednisolone, triamcinolone and dexamethasone. Average daily dose of prednisone (prednisolone) 30-40 mg, which is divided into 3-4 single reception. Duration of treatment is from 4 to 8 weeks and more (depending on the tolerability of treatment), followed by a slow decline and transition to maintenance dose (5-7,5 to 10 mg) for 1-2 months. Treatment with corticosteroids is not shown when expressed kidney failure with significant azotemia, has expressed hypertensive syndrome, the development of thrombohemorrhagic complications. Ineffective corticosteroids and at advanced stages of the disease. From other desensitizing and anti-inflammatory drugs with different effect using aspirin, salicylic sodium, phenylbutazone, probucol, reopirin, diphenhydramine, phenergan. Should avoid the use of sulfonamides, serums, vaccines, protein drugs, antibiotics, it is permissible to designate only with the development of infectious complications. The appropriate use of vitamins C, P and complex, especially B1 (polyneuritis). Hypertension shown antihypertensives. With the development of heart failure by using cardiac glycosides and diuretics.