Nodular periarteritis

Description:

Nodular periarteritis (polyarteritis) - a system necrotizing vasculitis with preferential damage of arteries of muscular type of average and small caliber and secondary changes of bodies and systems.

It is more correct to call a disease of a panarteritis in connection with involvement in pathological process of all layers of a vascular wall. Inflammatory cellular infiltration and a fibrinous necrosis of an adventitia, mussel and endothelium are histologically noted. In an active stage of a disease as a part of cellular infiltrate neutrophils prevail, mononuclear cells (monocytes, lymphocytes), a small amount of eosinophils appear further. After the end of inflammatory process in a vascular wall fibrosis with destruction of an inner elastic membrane develops.

Defeat of a wall of vessels leads also to formation of blood clots, formation of aneurisms, frequent heart attacks of bodies. Perivascular small knots from which there was a name of a disease a nodular periarteritis are observed seldom.

Symptoms of the Nodular periarteritis:

Men at the age of 30-60 years are ill preferential.
Fever of various type, long, not coming under influence of antibiotics.
General syndrome: weight loss, weakness, adynamia.
Changes of skin: pallor, peculiar mramornost of extremities and trunk; mesh livedo; skin rashes - erythematic, spotty and papular, hemorrhagic, urtikarny, are more rare - necrotic ulcerations with the subsequent pigmentation; at 20% of patients small painful small knots (aneurism of vessels or a granuloma) in skin or hypodermic cellulose on the course of neurovascular trunks of shins, forearms, hips are palpated.
Muscular and articulate syndrome: megalgias in muscles (it is especially frequent in sural), weakness and an atrophy of muscles, morbidity at a palpation; polyarthralgias, are more rare the polyarthritises - migrating, not deforming with preferential damage of one or several large joints (knee, talocrural, humeral, elbow).
Cardiovascular syndrome: coronaritises with development of stenocardia or myocardial infarction; "mute heart attacks" without characteristic clinical signs, only with ECG symptomatology are possible; myocardites, cardioscleroses, various disturbances of a rhythm, blockade, at 10% of patients - insufficiency of the mitral valve can develop. The most characteristic symptom - arterial hypertension.
Damage of kidneys in the form of a vascular nephropathy (at 70-97% of patients): a proteinuria, a microhematuria (the gross hematuria is more rare), a cylindruria, bystry development of a renal failure, development of a heart attack of a kidney owing to thrombosis of a renal artery is possible (severe pain in a waist, the hamaturia, is palpated a kidney, body temperature increases). Doppler ultrasonography reveals change of renal vessels at 60% of patients, and the stenozirovaniye is found in a half of them. In rare instances the rupture of aneurism of renal vessels with formation of pararenal hematomas is found.
Damage of lungs in the form of a pulmonary vasculitis or intersticial pneumonia: cough, short wind, stethalgias, pneumorrhagia, strengthening and deformation of the pulmonary drawing, various respiratory noise and rattles; heart attacks of lungs.
Damage of a digestive tract: the expressed pains in various departments of a stomach, tension of a front abdominal wall, an eructation, nausea, vomiting, a frequent liquid chair with impurity of blood and slime is frequent, considerable gastrointestinal bleedings, development of a pancreatonecrosis, perforation of ulcers of intestines, jaundice (damage of a liver) are possible.
Defeat of a nervous system: asymmetric mono - and polyneurites (thermalgias in extremities, sensitivity disturbance, paresis), strokes, meningoentsefalita, epileptiform attacks.
Damage of eyes: a malignant retinopathy, aneurysmal expansions or thickenings on the course of vessels of an eyeground.
At a part of patients damage of peripheral arteries of extremities develops that leads to ischemia of fingers up to their gangrene. Ruptures of aneurisms of arteries of legs, an inflammation of superficial branches of a temporal artery are possible.
Defeat of endocrine system: defeat of testicles (an orchitis, an epididymite) is observed at 80% of patients and more often happens against the background of infection with a hepatitis B virus; dysfunction of a thyroid gland and adrenal glands.
Features of a clinical current of a nodular periarteritis against the background of infection with a hepatitis B virus: defeats of testicles, kidneys and arterial hypertension are more often observed. At infection with a virus of hepatitis C damage of skin and liver and decrease in level of a complement in blood are more often noted.
Clinical options

The first symptoms of a classical nodular periarteritis - fever, muscle and joints pain, skin rashes, weight loss. Main clinical syndromes of this form: damage of kidneys (the isolated uric syndrome, an uric syndrome with unstable arterial hypertension, persistent arterial hypertension, malignant arterial hypertension, a nephrotic syndrome at fibrinferment of a renal vein); abdominal syndrome (vascular ulcers, ischemia, thrombosis of arterial trunks); defeat of a peripheral nervous system (neuritis, mialgiya, hypertensia, motive disturbances, atrophy of muscles, brushes and feet); damage of heart (a coronaritisis, a melkoochagovy myocardial infarction, the progressing heart failure, arrhythmias); damage of lungs (a vasculitis, the progressing fibrosis, a lung heart attack, pleurisy); defeat of TsNS (mental disorders, epipripadka, hemiparesis).

Skin тромбангитический the option of a nodular periarteritis is shown by small knots, livedo reticularis, a hemorrhagic purpura. Skin or hypodermic small knots are located on the course of a vascular bundle on extremities. Viscerites can be absent. Sometimes small knots are combined with livedo, tendency to development of necroses and ulcers. Fever, mialgiya, weakness, perspiration, weight loss are characteristic.

Mean the changes of vessels typical for a nodular periarteritis established at a patogistologichesky research by a monoorgan nodular periarteritis removed at operation or a biopsy of body.

Allocate the following options of a current of a nodular periarteritis: favorable (high-quality), slowly progressing, recuring, quickly progressing and acute, or fulminant.

The high-quality current is observed at patients with a skin nodular periarteritis without viscerites. A recurrence of a skin vasculitis with remissions to 3-5 years is possible. Patients somatic and socially sokhranna.

Slowly progressing current of a nodular periarteritis is observed at a half of patients with trombangitichesky option of a nodular periarteritis. At them the residual phenomena of peripheral neuritis and disturbance of blood circulation in extremities prevail for a number of years. At an uncomplicated current duration of a disease makes up to 10 years and more.
The recurrent current is followed by aggravations at cancellation of glucocorticoids and tsitostatik or decrease in their dose, at an intercurrent infection, a medicinal allergy, cooling. Emergence of new organ defeats worsens the forecast.

Quickly progressing current is observed at severe damage of kidneys with malignant arterial hypertension. The forecast of a nodular periarteritis is defined by vascular complications.

Acute, or fulminant, forms with life expectancy of 5-12 months are observed rather seldom. Damage of kidneys with development of a chronic renal failure, heavy arterial hypertension, cerebral frustration, heart failure, perforation of ulcers and fibrinferments of mezenterialny arteries are a cause of death.

Reasons of the Nodular periarteritis:

The etiology is finally not found out. The virus nature of a disease, in particular, a hepatitis B virus role (in blood of patients HBsAg and antibodies to it as a part of the CEC are found) is discussed.

The contributing factors - the postponed infections, intoxications, introduction of vaccines, serums, drug intake, overcooling, insolation.

The pathogeny comes down to giperergichesky reaction of an organism in response to etiological factors, autoimmune reaction antigen antibody (including to a vascular wall), to formation of cell-bound immune complexes, their adjournment in a vascular wall and to development in it an immune inflammation.

Cell-bound immune complexes activate a complement owing to what there is a direct damage of vessels and formation of hemotaksichesky substances which attract neutrophils in the defeat center.

They englobe cell-bound immune complexes, at the same time in a large number lizosomalny proteolytic enzymes which damage structures of a vascular wall are emitted. Development of pathological processes in a vascular wall is promoted as well that circumstance that endothelial cells have receptors for a Fc-fragment of IgG and the first fraction of a complement Clq that considerably facilitates interaction of cell-bound immune complexes with a vascular wall. The important pathogenetic point is also ability of neutrophils to stick to an endothelium and to allocate the activated oxygen radicals aggravating damage of a vessel in the presence of a complement. Besides, allocation by an endothelium of the factors promoting a blood coagulation and a thrombogenesis in the inflamed vessel amplifies.

Treatment of the Nodular periarteritis:

Treatment of a nodular periarteritis comes down to purpose of powerful antiinflammatory therapy; as a rule, use hormonal drugs on the basis of corticosteroids.