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Wegener's Granulomatosis - Diagnosis
Last reviewed: 04.07.2025
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Physical examination
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 should 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 the vessels that do not have a specific orientation. The features are the polymorphism of giant multinucleated cells, differing in the size of the cytoplasm, the number of nuclei, and the presence of necrosis - from focal karyorrhexis in cellular infiltrates and small necrotic foci to massive merging fields of dry coagulation necrosis. Morphological differential diagnostics should be carried out between Wegener's granudomatosis, tuberculosis, syphilis, median malignant granuloma of the nose, tumors.
Laboratory research
Among laboratory tests, the determination of antineutrophil cytoplasmic antibodies (ANCA) is of great importance for the diagnosis of Wegener's granulomatosis. They are found in 40-99% of patients; more often in patients with an active generalized process, less often - during remission in the localized form of the disease. In case of kidney damage, changes in urine are characteristic: microhematuria (more than 5 erythrocytes in the field of vision) or accumulations of erythrocytes in the urine sediment.
Instrumental research
Bone changes are detected by X-ray and CT, which is currently one of the main methods for diagnosing lesions of the nasal cavity and paranasal sinuses. At the same time, the skeletal picture of the nasal cavity and paranasal sinuses in Wegener's granulomatosis depends on the timing of the study, the time since the onset of the disease, and the nature of its course (acute, post-acute, chronic).
In the initial stages of the disease, the bone skeleton of the nose is not changed, the picture of the nasal cavity and paranasal sinuses is characteristic of a non-specific inflammatory process. In the acute course of the process, after 3-6 months, thinning of the nasal septum is detected, the nasal bones are revealed to be reduced, atrophic, their distal end is curved inward, they acquire the shape of a comma. In the chronic course of the disease, the destruction of bones occurs gradually and is detected during dynamic observation even after several years.
- X-ray changes in the nasal septum. The bone section of the nasal septum on the radiograph is determined to be thinned, atrophic, in some cases the contours of the nasal septum are "shaggy", and sometimes there is a rupture of the contour (defect), indicating the presence of perforation. In a third of cases, there is complete destruction of the nasal septum. Due to such pronounced changes in the bone section of the septum, there is a suspicion of syphilis, and with perforation only in the anterior parts of the nose, tuberculosis. The elements of the nasal turbinates of the affected side can be radiologically detected enlarged and reduced, sometimes completely absent.
- Radiological changes in the paranasal sinuses.
- Maxillary sinus. The decrease in pneumatization of the sinus affected by the granulomatous process varies in intensity, uniformity and prevalence, which is due to the presence of granulomas with the corresponding reaction of the mucous membrane, the addition of a secondary infection and destructive changes in the bone walls. The bone walls of the maxillary sinus are determined on radiographs as thinned, the intensity of their pattern is reduced. As a rule, destruction of the medial wall of the maxillary sinus is revealed. Destructive changes in the upper wall of the maxillary sinus are observed less often. Bone changes in the sinus are more convincingly revealed on direct anterior tomograms, where the "contour break" of the medial wall is very demonstrative. The bone wall also becomes thinner (or the pattern becomes unclear) in a limited area of the lower part of the piriform opening of the nose. To identify changes in the soft tissues of the sinus, it is advisable to conduct an X-ray examination with filling it with a contrast agent. Skialogic features in conventional and tomographic examination are the same as in lesions of the medial wall of the sinus, but are more clearly visible due to the characteristic anatomical structure and lesser superposition with surrounding bone formations. Changes in the lower wall of the sinus are rare, which is possibly due to its significant thickness.
- Ethmoid labyrinth. No differences in the radiographic picture of ethmoid labyrinth lesions in Wegener's granulomatosis and chronic inflammatory process have been identified. In both diseases, the pattern of intercellular septa is poorly differentiated or absent, the ethmoid plate is thinned or partially destroyed, the ethmoid labyrinth is somewhat widened compared to the opposite side.
- Sphenoid sinus. The radiographic picture depends on the degree of reduction of pneumatization of the sphenoid sinus. The walls of the sphenoid sinus are thinned. The body of the sphenoid bone and its wings on the side of the process have a less intense pattern. Significant changes occur in the area of the upper and lower orbital fissures: their lumen becomes cloudy, the contours are unclear and uneven, in places eroded. Lesions of the sphenoid sinus in Wegener's granulomatosis are much less common than those of the maxillary sinus, but the possibility of such a lesion should be remembered.
It is necessary to take into account changes in the lungs, established by radiography: nodules, pulmonary infiltrates or cavities.
Differential diagnostics
Wegener's granulomatosis must be differentiated from diseases related to systemic allergic vasculitis (systemic lupus erythematosus, hemorrhagic vasculitis, periarteritis nodosa, etc.); if perforation occurs in the cartilaginous part of the nasal septum - from tuberculosis, and in the bone-cartilaginous part - from syphilis. Further progression of the ulcerative-necrotic process in the nasal cavity and paranasal sinuses requires differential diagnosis with malignant neoplasms.
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