Nodular periarteritis refers to pathologies with an unclear etiology. The role of a viral infection (including hepatitis B) in the development of nodular periarteritis is considered; With the starting factors may be vaccination, the introduction of sera, drugs, insolation or hypothermia. In response to these factors, a hyperergic reaction develops with the formation of immune complexes, which are fixed in the walls of the vessels and cause autoimmune inflammation in them. These processes are accompanied by the isolation of endothelium of damaged vessels by factors of increased coagulation and thrombus formation. Usually periarteritis is predominantly male for 30 to 50 years.
There is a classic (with renal-visceral or renal-polyneuritis symptoms), asthmatic, skin-trombangiotic and monoorganic variants of the clinical development of nodular periarteritis. Benign development of nodular periarteritis is noted with cutaneous form without visceropathy. Patients are physically and socially preserved; Remissions are stable, however, exacerbations of vasculitis are possible.
Slow progression is characteristic of the thrombangiotic variant of nodular periarteritis. In this case, there may be arterial hypertension, peripheral neuritis, microcirculatory disorders in the limbs. Recurrent nodular periarteritis is provoked by cancellation or reduction of dosage of cytostatics, glucocorticoids, drug allergy, infection, cooling.
Rapid progression of nodular periarteritis is associated with kidney damage and malignant form of arterial hypertension. In rare cases, the disease develops at lightning speed, leading to the death of the patient after 5-12 months. In the clinic of nodular periarteritis, an active, inactive and sclerotic phase is isolated.
With nodular periarteritis, there is a high wave-like fever, decreasing in response to taking glucocorticoids or aspirin, losing weight right up to cachexia, adynamia, weakness. For the skin is characterized by pallor, marble shade, the appearance of reticulated livedo, skin rashes (erythematous, patchy-papular, hemorrhagic, necrotic), subcutaneous nodules in the region of the forearms, shins, thighs.
Musculo-articular manifestations with nodular periarteritis include myalgia, weakness, soreness, muscle atrophy; Polyarthralgia, migratory arthritis of large joints. The renal symptom complex in 70-97% of patients with nodular periarteritis occurs with vascular nephropathy: microhematuria, proteinuria, cylindruria, rapid development of renal failure. Possible outcomes are ruptures of renal vascular aneurysms, kidney infarction.
The syndrome of cardio-vascular insufficiency includes the development of coronary arteries leading to angina and myocardial infarction, myocarditis, cardiosclerosis, conduction disorders, arrhythmias, mitral valve insufficiency. The characteristic cardio-vascular manifestation of nodular periarteritis is hypertension. When lung lesions develop pulmonary vasculitis and interstitial pneumonia, manifested by coughing, shortness of breath, hemoptysis, thoracalgia, respiratory noises and wheezing, lung infarcts.
Lesions of the gastrointestinal tract with nodular periarteritis occur with nausea, diarrhea, epigastric pain. With a complicated version, it is possible to develop pancreonecrosis, jaundice, perforated stomach ulcers and 12p. Guts, bleeding. Involvement of the nervous system is manifested by asymmetric polyneuropathy: muscular atrophy, soreness in the projection of nerve trunks, paresthesia, paresis, trophic disorders. In the case of severe lesions, the occurrence of strokes, meningoencephalitis, epileptiform seizures is likely.
Visual disorders with nodular periarteritis are expressed by malignant retinopathy, aneurysmal dilations of the vessels of the fundus. Violations of the peripheral blood supply of the limbs cause ischemia and gangrene of the fingers. When the endocrine apparatus is damaged, orchites and epididymitis, dysfunctions of the adrenal and thyroid gland are noted.
The variant of an asthmatic nodular periarteritis proceeds with persistent attacks of bronchial asthma, skin manifestations, fever, arthralgia and myalgia. The dominant manifestations of the cutaneous-thrombangic form of the nodular periarteritis are nodules, livedo and hemorrhagic purpura. The subcutaneous nodules are characterized by an arrangement along the vascular bundles of the extremities. This symptomatology develops against the background of myalgia, fever, sweating, weight loss. Nodular periarteritis, flowing in a mono-organ type, is characterized by visceropathies and is established after a histological examination of a biopsy or a remote organ. Complicated forms of nodular periarteritis may be accompanied by the development of infarctions and sclerosis of organs, rupture of aneurysms, perforation of ulcers, gangrene of the intestine, uremia, stroke, encephalomyelitis.
In the general clinical analysis of urine, microhematuria, proteinuria and cylindruria are determined; In the blood - signs of neutrophilic leukocytosis, hyperthrombocytosis, anemia. Changes in the biochemical picture of blood with nodular periarteritis are characterized by an increase in the fractions of γ- and α2-globulins, sialic acids, fibrin, seromucoid, SRP.
To clarify the diagnosis with nodular periarteritis, a biopsy is performed. In the musculoskeletal biopsy of the abdominal wall or shin, inflammatory infiltration and necrotic changes in the vascular walls are revealed. With nodular periarteritis in the blood often determines HBsAg or antibodies to it. An examination of the fundus reveals aneurysmal changes in the vessels. UZDG of kidney vessels determines their stenosis. With an overview of the radiography of the lungs, there is an increase in the pulmonary pattern and its deformation. For diagnostics of cardiopathies, ECG, ultrasound of the heart is performed.
The large diagnostic criteria of nodular periarteritis include the presence of lesions of the kidneys, abdominal syndrome, coronaryitis, polyneuritis, bronchial asthma with eosinophilia. Additional (small) criteria are myalgia, fever, weight loss. When diagnosing nodular periarteritis, three large and two small criteria are taken into account.
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 nodular periarteritis can be corrected by corticosteroid therapy with prednisolone with a repetition of courses 2-3 times a year. In the interval between corticosteroid courses, prescribe drugs pyrazolone series (butadione) or acetylsalicylic k-tu.
With nodular periarteritis complicated by malignant hypertension or nephrotic syndrome, immunosuppressive agents-cytostatics (azathioprine, cyclophosphamide) are administered. Correction of the syndrome of ICE and hyperthrombocytosis includes therapy with heparin, pentoxifylline, dipyridamole. Biopreparations blocking TNF (infliximab, ethanercept), can quickly reduce inflammation.
With chronic nodular periarteritis, which occurs with muscle atrophy or neuritis, LFK, hydrotherapy, and massage are performed. Methods of extracorporal hemocorrection (plasmapheresis, hemosorption, cryoapheresis) reduce the severity of autoimmune reactions and blood viscosity due to the removal of the CEC, autoantibodies, and excessive factors of thrombus formation from the bloodstream.
The course of nodular periarteritis is unfavorable in terms of prognosis. Severe vascular lesions (a syndrome of renal failure, arterial hypertension, cerebral disorders, thrombosis, perforated complications, etc.) can lead to death. Remission and arrest of progression of nodular periarteritis is achieved in 50% of patients. The preventive tasks include the account of drug intolerance, reasonable and controlled immunization, transfusion of blood products, protection against infections.