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Lesion of ENT organs with Wegener's granulomatosis
Last reviewed: 23.04.2024
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Wegener's granulomatosis is a severe general disease from the group of systemic vasculitides. Which is characterized by granulomatous lesions, in the first place, the upper respiratory tract, lungs and kidneys.
Synonyms
Necrotizing respiratory granulomatosis with vasculitis, respiratory-renal form of nodular periarteritis, Wegener's disease, necrotic granuloma of the upper respiratory tract with nephritis.
ICD-10 code
M31.3 Wegener's granulomatosis.
Causes of Wegener's granulomatosis
To date, the etiology of Wegener's granulomatosis remains unclear, but most authors are inclined to believe that this is an autoimmune disease. In the development of the disease, the etiological role of viruses (cytomegalovirus, herpes viruses), as well as the genetic peculiarity of immunity, is assumed. Factors provoking the onset of Wegener's granulomatosis, first of all, testify to exogenous sensitization of the organism; they are not etiological, as they can constantly act on a large contingent of people, but only in single cases lead to the development of Wegener's granulomatosis. Thus, we can assume the existence of a certain genetic predisposition. In favor of this is a significant increase in the blood of patients with Wegener's granulomatosis, the content of HLA-A8 antigen, responsible for the genetic predisposition to autoimmune diseases.
Pathogenesis of Wegener's granulomatosis
In the pathogenesis of Wegener's granulomatosis, immunological disorders are of primary importance, in particular, the deposition of immune complexes in the walls of blood vessels and disorders of cellular immunity. The morphological picture is characterized by necrotic vasculitis of medium and small-sized arteries and the formation of polymorphous-cell granules containing giant cells.
Symptoms of Wegener's granulomatosis
To the otorhinolaryngologist, patients usually turn in the initial stage of the disease, when the rhino and pharyngoscopic picture is regarded as a banal inflammatory process (atrophic, catarrhal rhinitis, pharyngitis), which is why precious time is wasted to start treatment. It is important to recognize in a timely manner the characteristic signs of nasal and paranasal sinus lesions with Wegener's granulomatosis.
The first complaints of patients who turn to the otorhinolaryngologist are usually reduced to nasal congestion (usually one-sided), dryness, poor mucous secretions, which soon become purulent, and then blood-purulent. Some patients complain of bleeding due to the development of granulations in the nasal cavity or destruction of the septum of the nose. However, nosebleeds are not a pathognomonic symptom, since they are relatively rare. A more permanent symptom of the defeat of the nasal mucosa, which occurs in the earliest stages of Wegener's granulomatosis, is the formation of purulent-bloody crusts.
Classification
There are localized and generalized forms of Wegener's granulomatosis. With a localized form, ulcerative necrotic lesions of the ENT organs, granulomatosis of the orbit or a combination of processes develop. In generalized form, along with the granulomatosis of the upper respiratory tract or eyes, glomerulonephritis develops in all patients, and it is also possible to involve the cardiovascular system, the lungs and the skin.
Wegener's granulomatosis can proceed acutely, subacute and chronically, and the primary localization of the process does not determine the further course of the disease. In case of acute variant the disease proceeds malignantly, with subacute - it is relatively malignant, and chronic development is characterized by slow development and prolonged course of the disease.
Screening
In 2/3 of cases, the disease begins with the defeat of the upper respiratory tract. Further visceral organs are involved in the process. In this regard, for the early diagnosis and prolongation of the life of the patient, it is extremely important to know the clinical symptoms (purulent or spotting from the nose, ulcers in the mouth). In the initial stage of the disease, ulcerative necrotic changes can be observed only in one organ, then the lesion spreads to surrounding tissues, eventually the lungs and kidneys are involved in the pathological process. The disease is based on generalized vascular lesion and necrotizing granulomatosis. The second variant of the onset of the disease is less common, it is associated with the lesion of the lower respiratory tract (trachea, bronchi, lungs) and is called "headless Wegener's granulomatosis."
Diagnosis of Wegener's granulomatosis
In the diagnosis of Wegener's granulomatosis, especially in the early stages of the disease, an adequate assessment of changes in the upper respiratory tract, especially the nose and paranasal sinuses, is extremely important. This determines the leading role of the otorhinolaryngologist in the early diagnosis of the disease. The upper respiratory tract is available for examination and taking a biopsy, which can confirm or disprove the diagnosis of Wegener's granulomatosis.
Biopsy of the mucous membrane should be taken aiming, capturing both the epicenter and the border zone of the lesion. The basis of the morphological features of the process is the granulomatous nature of the productive inflammatory reaction with the presence of giant multinucleate cells such as Pirogov-Langhans or gigantic multinuclear cells of foreign bodies. Cells are concentrated around vessels that do not have a specific orientation.
What do need to examine?
What tests are needed?
Treatment of Wegener's granulomatosis
The purpose of the therapy is to achieve remission and exacerbation of the disease and then to maintain it. The main principles of treatment tactics are possible early and timely initiation of treatment, individual selection of the drug, doses, and the timing of treatment for exacerbation, long-term treatment taking into account the progressive nature of the disease.
The basis of treatment is the use of glucocorticoids in combination with cytostatics. As a rule, the initial suppressive dose of glucocorticoids is 6080 mg of prednisolone per day orally (1-1.5 mg / kg of body weight per day), with insufficient effectiveness, the dose is increased to 100-120 mg. If in this case it is not possible to control the disease, so-called pulse therapy is used-prescribe 1000 mg of methylprednisodone for 3 consecutive days, the drug is injected intravenously calve in a 0.9% solution of sodium chloride.