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Wegener's disease

At present, Wegener's disease (granulomatosis) is considered as an autoimmune disease that is part of the group of systemic rheumatoid diseases. It has a clearly defined clinical picture with a primary primary lesion of the upper respiratory tract and subsequent involvement of the visceral organs in the process. The disease was first described in 1936-1939. F.Wegener, in whose honor it was later named. Wegener's disease until relatively recently was a "white spot" in otorhinolaryngology and belonged to clinical casuistry. However, systematic publications in domestic and foreign literature indicate that this disease is not so rare. One of the essential pathomorphological and clinical features b. Wegener is the primary lesion of the nose and paranasal sinuses, which determines the leading role of ENT specialists in the diagnosis, clinical observation and treatment of these patients.

Etiology b. Wegener remains largely obscure. However, there is sufficient reason to link the development of the disease with allergization of the respiratory tract to microbial antigens, in particular Staphylococcus aureus, which sensitization is detected in these patients (Hawell SB, Epstein WV, 1976). It is believed that bacterial toxins and certain drugs under certain conditions can cause pathological changes in the walls of blood vessels, contributing to the formation of autoallergens. In response to the appearance of the latter, antibodies are produced not only to altered, but also to normal protein components. It was noted that a relapse of the disease usually occurs after hypothermia, acute infection, trauma, insolation, vaccination and other effects, which are a kind of starting point (Dainyak L.B., Mincin R.A., Bykova V.P., 1987).

According to modern concepts, the basis of pathogenesis b. Wegener has a complex of immune mechanisms, hypersensitivity of a delayed type of bacterial, drug and other origin. The main substrate of the disease is an increase in vascular membrane permeability, associated with the deposition of the antigen-antibody-complement complex, followed by a productive cellular reaction around the vessels and the formation of granulosa nodules. In this case, a genetic predisposition is possible.

The main pathological manifestations of the disease are: 1) generalized allergic vasculitis; 2) necrotizing granulomatosis; 3) kidney damage in the form of glomerulitis and glomerulonephritis and lung damage in the form of diffuse and focal bronchopneumonia or destructive panbronchitis. It is emphasized that with b. Wegener has a peculiar productive inflammation with a pronounced angioplastic reaction and the concentration of inflammatory infiltrate cells around the vessels (arterial and venous small caliber), which creates a peculiar picture of granulomatosis. In histological terms, granulosa nodules covering vessels such as couplings are distinguished by cellular polymorphism. Along with lymphoid, epithelioid and plasma cells, there are histiocytes, eosinophils and neutrophils. An important difference between these granular nodules is the giant multinucleated cells of the Pirogov-Lanhgans type, located randomly around the periphery. Their peculiarity is also a tendency to the development of ischemic necrosis.

Clinic. The multisystem nature of the lesion and involvement of individual organs in the pathological process at different times determines the polysyndromism and polymorphism of clinical manifestations, which complicates the diagnosis b. Wegener. The disease often affects men and can be observed at any age, but prevails in the third and fourth decades of life. There are three clinical options b. Wegener: malignant, relatively malignant and chronic (Mincin R.A., 1976). These options can also be considered as an acute, subacute and chronic course of the disease (Dainyak LB et al., 1987). The main, almost constant, symptom complex b. Wegener is the pathology of the upper respiratory tract, oropharynx and, to a lesser extent, the ears, which determines the particular importance of the participation of otorhinolaryngologists in the treatment of these patients.

The first complaints of nasal lesions usually come down to respiratory failure, as a rule, of one half of the nose, dryness, scarcity of mucous secretions, which soon become purulent, and then blood-purulent. The most persistent symptom of damage to the nasal mucosa in b. Wegener is the formation of purulent-bloody crusts at an early stage of the disease. Crusts are brown-brown in color and are removed from the nasal cavity in the form of casts. Initial manifestations b. Wegeners can be regarded as atrophic rhinitis or ozena. However, with a lake, the crusts have a dirty green color and have a characteristic unpleasant odor. After removing the crusts in patients with Wegener's granulomatosis, the mucous membrane has a rather characteristic appearance: it is thinned, red-cyanotic in color, some of its sections can be covered with granulations that violate the nasal obstruction. The similarity of granulations with a tumor is enhanced due to their increased bleeding.

A feature of clinical manifestations in the nasal cavity with b. Wegener is the presence of an ulcerated mucosa in the anterior nasal septum. With the development of necrosis, perforation of the septum occurs, which is first localized in the cartilage. Gradually progressing, the process captures its bony section.
It should be noted that with b. Wegener's destructive process from the nasal septum does not pass to the hard palate, which is observed with the lethal median granuloma of Stuart. In connection with the destruction of the septum, the nose becomes saddle-shaped. The paranasal sinuses are also involved, most often one of the maxillary sinuses on the affected side of the nose. The spread of the process to other sinuses and polysinitis is less common. The bone wall between the nasal cavity and the maxillary sinus, including the nasal concha, undergoes destruction and forms a single cavity of the sinus and the corresponding half of the nose. With the destruction of the nasal septum and the involvement of the other half of the nose, the cavity increases. However, with b. Wegener (again, in contrast to Stuart's granuloma), there were no cases of damage to the external bone walls. The resulting pathological cavity is covered with a necrotic mucous membrane with a large number of crusts. X-ray can be revealed destructive processes of the bone walls.

According to L. B. Dainiak et al. (1987), lesions of the nasal cavity and paranasal sinuses, manifested in the form of ulcerative necrotic rhinitis with partial or complete destruction of the nasal septum, unilateral lesion of the bony base of the nasal concha, as well as destructive monosynitis - are one of the most pathognomonic signs b. Wegener.

Along with rhinological symptoms, an ophthalmic pathology can be detected at the very beginning of the disease, which is explained by the common blood supply to the paranasal sinuses and orbits. One of the most frequent ophthalmic manifestations b. Wegener is a lesion of the cornea (keratitis), as well as uveitis. Unilateral exophthalmos is less common.

Ulcerative necrotic lesions of the pharynx and larynx are observed relatively rarely. Damage to the middle and inner ear is possible.

Purulent otitis media is usually accompanied by paresis of the facial nerve, which indicates deep destructive processes in the temporal bone. Confirmations of cochlear sensorineural hearing loss can be obtained audimetrically.

Lesions of the lungs and kidneys, along with damage to the upper respiratory tract, are characteristic clinical signs b. Wegener and, according to various authors, are found in 50 - 95% of cases.

Clinical pathology of the lungs in patients with Wegener's granulomatosis manifests itself in the form of diffuse or focal bronchopneumonia with a tendency to abscess and hemoptysis. Sometimes, with vascular erosion, pulmonary bleeding is possible. Glomerulitis and glomerulonephritis develop in the kidneys, which is manifested by proteinuria, hematuria and hyalinuria. Another organopathology is also possible.

General symptoms with b. Wegeners manifest as prolonged malaise, subfebrile or higher body temperature. The progression of the disease is accompanied by the appearance of clinical symptoms characteristic of lesions of the upper respiratory tract (nose, paranasal sinuses), lungs, kidneys, and other organs involved in the pathological process.

Patients with Wegener's granulomatosis die from azotemic uremia with increasing manifestations of pulmonary failure, septic complications.

Diagnostics b. Wegener, especially in the initial period of the disease, remains complex and requires a known clinical knowledge from the doctor. Any specific laboratory diagnostic tests specific to b. Wegener, no. In the diagnosis of this disease, it is important to be able to assess the complex of the main clinical manifestations of Wegener's granulomatosis (pathology of the respiratory tract - nose and paranasal sinuses, lungs and kidneys), as well as conducting (sometimes repeated) targeted morphological studies of biopsy material.

A differential diagnosis is made with a group of diseases related to systemic allergic vasculitis (nodous periarteritis, systemic lupus erythematosus) with the appearance of perforation of the nasal septum in the cartilaginous department with tuberculosis, and in the bone-cartilaginous department with syphilis. Further progression of the ulcerative-necrotic process in the nasal cavity and paranasal sinuses requires differential diagnosis with malignant neoplasms and a lethal median gangrenous granuloma of Stuart. Recently, it has been considered as malignant lymphoma. A characteristic feature of Stuart's granuloma is a progressive destructive process of the middle part of the face, capturing the bone structures of the nose, upper jaws, and sharply destroying the facial skeleton. A distinctive feature of Stuart's granuloma is not only severe ulceration of the nose and tissues of the middle part of the face, but also the absence of generalized vasculitis, which is so characteristic of Wegener's granulomatosis. It should be added that Stuart's granuloma is radiosensitive.

Treatment. Until the 50s of the 20th century b. Wegener was an absolutely fatal disease. Antibiotic treatment was unsuccessful. Evolution of views on etiology and pathogenesis b. Wegener allowed to achieve significant success due to the use of corticosteroids and immunosuppressants. This allowed for timely treatment, when the disease has not yet passed into a generalized form, as well as in the chronic course of the disease, to extend persistent remission, and with it the life expectancy of doomed patients for many years.
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Wegener's disease

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