Nodular periarteritis

Nodular periarteritis - systemic necrotizing vasculitis by the type of segmental lesion with aneurysmic protrusion of arteries of muscle type and smaller caliber.

Pathogenesis. Hyperergic reaction of the body in response to the impact of various factors; Essential role of immune complexes, circulating and fixed in the vessel wall. Mostly men are sick at the age of 30-40 years.

Symptoms, course. The disease begins acutely or gradually with symptoms of a general nature - a rise in body temperature, rapidly growing weight loss, joint pain, muscle, abdomen, skin rashes, signs of damage to the gastrointestinal tract, heart, kidneys or peripheral nervous system. In the course of time, the development of the field-of-interest symptomatology. Almost all patients have glomerulonephritis - from mild nephropathy with transient hypertension and moderate urinary syndrome to diffuse glomerulonephritis with persistent arterial hypertension and rapidly progressing course. Prognostically unfavorable development in patients with the syndrome of malignant hypertension and nephrotic syndrome, characterized by the rapid development of renal failure and poor tolerability of active corticosteroid therapy. In addition, there are infarcts of the kidneys, aneurysms on the soil of the arteritis, defeat of the glomerular apparatus.

Damage to the heart is observed in almost 70% of patients. In view of the significant lesion of the coronary vessels, angina attacks are noted, myocardial infarction without vivid clinical signs may develop. Occasionally, hemopericard is seen as a result of rupture of aneurysm or exudative pericarditis due to the lesion of small vessels. Perhaps the development of Raynaud's syndrome occasionally with gangrene of the fingers; Sometimes there is a picture of migrating phlebitis.

Acute pain in the abdomen is very typical for nodular periarteritis and is associated with a pathological process in the vessels of the abdominal cavity. When gastric vessels are affected, gastritis is observed, with enteritis in the small intestine, colitis is manifested by colitis with constipation, alternating diarrhea with tenesmus and blood in the feces. Can develop acute appendicitis or acute cholecystitis, pancreatitis, intestinal perforation due to necrosis, heart attack, hemorrhages.

The defeat of the nervous system in half of the patients is manifested by characteristic asymmetric multiple neuritis in connection with the pathological process in the vessels feeding this or that nerve. Meningoencephalitis is also possible with speech and hearing impairment, headache with pain and dizziness, convulsions, darkened consciousness and irritation of the meninges, and focal brain lesions in connection with thrombosis, ruptures of aneurysms. When examining the fundus, aneurysms of the arteries, perivascular infiltrates, sometimes retinopathy, plasmorrhagia, central retinal artery thrombosis, etc., are revealed. Eye damage can be one of the earliest symptoms of the disease.

There are arthralgia, less migratory arthritis of large joints, myalgia, various skin lesions (erythematous, spotty-papular, hemorrhagic, urticaric, vesicular and necrotic); Only a small part of the patients can detect subcutaneous nodules, which are very characteristic of the nodular periarteritis, which are vascular aneurysms or granuloma associated with the affected vessel. It should be specially emphasized the rapidly evolving expressed pallor of the patients, which, in combination with depletion, creates a picture of "chlorotic senility". Lesion of the lungs is manifested by bronchial asthma or pneumonitis syndromes. Pulmonary symptoms are associated with vascular damage. Syndrome of bronchial asthma can long before the unfolded picture of nodular periarteritis.

The most common syndromes are: renal-polyneuritis, renal-abdominal-cardiac, pulmonary-cardiac-renal, pulmonary-polyneuritis.

Laboratory data are not typical. Possible leukocytosis with neutrophil shift, eosinophilia, sometimes high, in severe cases - moderate anemia and thrombocytopenia. ESR is usually increased, there is persistent hypergammaglobulinemia and often hyperproteinemia. When biopsies of the muscles from the region of the legs or the abdominal wall, the changes in the vessels characteristic of this disease are revealed.

Treatment. Glucocorticoids are most effective in the early stages of the disease. Prednisolone is used in doses of 60-100 mg / day for 3 to 4 days; When the condition is improved, the dose is slowly reduced. Prolonged use of prednisolone leads to the stabilization of hypertension, the progression of retinopathy and kidney failure. In acute course, it is often observed the paradoxical effect of corticosteroids with the development of multiple infarctions.

In addition, corticosteroids can dramatically worsen the course of syndromes of malignant hypertension, in such cases, hormones are contraindicated. The method of choice are cytotoxic drugs - cyclophosphamide and azathioprine (50 mg2 3-4 times a day) for 2.5-3 months, and then 100-150 mg / day; Treatment lasting with careful monitoring of side effects.

In chronic course with muscular atrophies and neuritis, therapeutic physical exercises are recommended taking into account organ pathology, massage and hydrotherapy, long-term reception of hingamine (delagila) in 0.25 g or plaquenil 0.2 g / day after dinner; In the presence of eosinophilia, the doses of plakvenyl may be increased to 0.2 g 5 times per day (months) followed by a prolonged intake of maintenance doses.