Medical expert of the article
New publications
Lesions of the ENT organs in Wegener's granulomatosis
Last reviewed: 12.07.2025
All iLive content is medically reviewed or fact checked to ensure as much factual accuracy as possible.
We have strict sourcing guidelines and only link to reputable media sites, academic research institutions and, whenever possible, medically peer reviewed studies. Note that the numbers in parentheses ([1], [2], etc.) are clickable links to these studies.
If you feel that any of our content is inaccurate, out-of-date, or otherwise questionable, please select it and press Ctrl + Enter.
Wegener's granulomatosis is a severe general disease from the group of systemic vasculitis, which is characterized by granulomatous lesions, primarily of the upper respiratory tract, lungs and kidneys.
Synonyms
Necrotizing respiratory granulomatosis with vasculitis, respiratory-renal form of nodular periarteritis, Wegener's disease, necrotizing granuloma of the upper respiratory tract with nephritis.
ICD-10 code
M31.3 Wegener's granulomatosis.
Epidemiology
The epidemiology of Wegener's granulomatosis has not been studied, as it is a relatively rare disease that can occur at any age (on average, at 40 years). About 15% of patients are under 19 years old, children rarely get sick. Men get sick somewhat more often.
[ Causes of Wegener's granulomatosis
To date, the etiology of Wegener's granulomatosis remains unclear, but most authors are inclined to believe that it is an autoimmune disease. The etiological role of viruses (cytomegalovirus, herpes viruses) and genetic features of immunity are assumed in the development of the disease. Factors that provoke the onset of Wegener's granulomatosis primarily indicate exogenous sensitization of the body; they are not etiological, since they can constantly affect large groups of people, but only in isolated cases lead to the development of Wegener's granulomatosis. Thus, it is possible to assume the existence of a certain genetic predisposition. This is evidenced by a significant increase in the content of the HLA-A8 antigen in the blood of patients with Wegener's granulomatosis, which is responsible for the genetic predisposition to autoimmune diseases.
Pathogenesis of Wegener's granulomatosis
In the pathogenesis of Wegener's granulomatosis, the main significance is given to immunological disorders, in particular, deposits 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 caliber arteries and the formation of polymorphic cell granulomas containing giant cells.
Symptoms of Wegener's granulomatosis
Patients usually consult an otolaryngologist at the initial stage of the disease, when the rhino- and pharyngoscopic picture is assessed as a banal inflammatory process (atrophic, catarrhal rhinitis, pharyngitis), due to which precious time for the beginning of treatment is lost. It is important to promptly recognize the characteristic signs of damage to the nose and paranasal sinuses in Wegener's granulomatosis.
The first complaints of patients who consult an otolaryngologist usually boil down to nasal congestion (usually one-sided), dryness, scanty mucous discharge, which soon becomes purulent, and then bloody-purulent. Some patients complain of bleeding caused by the development of granulation in the nasal cavity or the destruction of the nasal septum. However, nosebleeds are not a pathognomonic symptom, since they occur relatively rarely. A more constant symptom of damage to the mucous membrane of the nasal cavity, which occurs in the earliest stages of Wegener's granulomatosis, is the formation of purulent-bloody crusts.
Classification
Localized and generalized forms of Wegener's granulomatosis are distinguished. In the localized form, ulcerative-necrotic lesions of the ENT organs, orbital granulomatosis, or a combination of processes develop. In the generalized form, along with granulomatosis of the upper respiratory tract or eyes, all patients develop glomerulonephritis, and involvement of the cardiovascular system, lungs, and skin is also possible.
Wegener's granulomatosis can be acute, subacute and chronic, and the primary localization of the process does not determine the further course of the disease. In the acute variant, the disease is malignant, in the subacute variant - relatively malignant, and the chronic variant is characterized by slow development and a long course of the disease.
Screening
In 2/3 of cases, the disease begins with damage to the upper respiratory tract. Later, visceral organs are involved in the process. In this regard, knowledge of clinical symptoms (purulent or bloody discharge from the nose, ulcers in the oral cavity) is extremely important for early diagnosis and prolongation of the patient's life. In the initial stage of the disease, ulcerative-necrotic changes can be observed only in one organ, then the lesion spreads to the surrounding tissues, and over time, the lungs and kidneys are involved in the pathological process. The disease is based on generalized vascular damage and necrotizing granulomatosis. The second variant of the onset of the disease is less common, it is associated with damage to the lower respiratory tract (trachea, bronchi, lungs) and is called "decapitated 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 otolaryngologist in the early diagnosis of the disease. The upper respiratory tract is available for examination and biopsy, which can confirm or refute the diagnosis of Wegener's granulomatosis.
A biopsy of the mucous membrane must be taken with precision, covering 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 multinucleated cells of the Pirogov-Langhans type or giant multinucleated cells of foreign bodies. The 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 goal of the therapy is to achieve remission during an exacerbation of the disease and then maintain it. The main principles of treatment tactics are the earliest and most timely initiation of treatment, individual selection of the drug, doses, and duration of treatment during an 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), if insufficient effectiveness, the dose is increased to 100-120 mg. If in this case it is not possible to achieve control over the disease, so-called pulse therapy is used - 1000 mg of methylprednisodone is prescribed for 3 days in a row, the drug is administered intravenously by injection in a 0.9% sodium chloride solution.