Wegener's granulomatosis: diagnosis

Indications for specialist consultations

When establishing the diagnosis of graylenematosis Wegener is obligatory consultation of a rheumatologist.

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 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. The features are the polymorphism of giant multinucleate cells, differing in cytoplasm size, the number of nuclei, and the presence of necrosis from focal karyorexis in cell infiltrates and small necrotic foci to massive confluent dry coagulation necrosis fields. Morphological differential diagnosis should be conducted between Wegener's granemematosis, tuberculosis, syphilis, middle malignant nasal granuloma, tumors.

Laboratory research

Among laboratory tests, the determination of antineutrophil cytoplasmic antibodies (ANCA) is important for the diagnosis of Wegener's granulomatosis, which is found in 40-99% of patients; more often in patients with an active generalized process, less often during remission with a localized form of the disease. When the kidneys are affected, urine changes are characteristic: microhematuria (more than 5 erythrocytes in the field of vision) or a collection of cells in the urine sediment.

Instrumental research

Bony changes are detected in radiography and CT, which is currently one of the main methods of diagnosing lesions of the nasal cavity and paranasal sinuses. In this case, the skiologic picture of the nasal cavity and paranasal sinuses with Wegener's granulomatosis depends on the terms of the study from the time from the moment of the disease and the nature of its course (acute, post-acute, chronic).

In the initial stages of the disease the skeleton of the nose is not changed, the picture of the nasal cavity and paranasal sinuses is characteristic of the nonspecific inflammatory process. In the acute course of the process after 3-6 months, thinning of the septum of the nose is detected, the bones of the nose are revealed diminished, atrophic, the distal end of them is bent inside, they take the form of a comma. In the chronic course of the disease, the destruction of the bones occurs gradually and with dynamic observation we find out after several years.

• X-ray changes in the septum of the nose. The bone compartment of the septum of the nose on the roentgenogram is defined as thinned, atrophic, in some cases, the "ruffledness" of the contours of the septum of the nose, and sometimes the rupture of the contour (defect), indicating the presence of perforation. In a third of cases there is complete destruction of the nasal septum. In connection with such pronounced changes in the septum in the osseous part, there is a suspicion of syphilis, and with perforation only in the anterior parts of the nose tuberculosis. Elements of the nasal concha of the affected side can be radioliologically detected enlarged and reduced, sometimes completely absent.
• X-ray changes in the paranasal sinuses.
  • The maxillary sinus. The decrease in pneumatization of the sinus affected by the granulomatous process is different in intensity, uniformity and prevalence, which is due to the presence of a granule with the corresponding reaction of the mucosa, the attachment of a secondary infection and destructive changes in the bone walls. The bony walls of the maxillary sinus on the radiographs are determined by the thinned, the intensity of their pattern is reduced. As a rule, the destruction of the medial wall of the maxillary sinus is revealed. Less frequent are destructive changes in the upper wall of the maxillary sinus. More convincingly, bone changes in the sinus are revealed on the direct front tomograms, where the "rupture of the contour" of the medial wall is highly demonstrative. The bone wall is also thinning (or appears blurred) in a limited area of the lower part of the pear-shaped aperture of the nose. To detect changes in the soft tissues of the sinus, it is advisable to perform an X-ray examination with filling it with a contrast agent. The peculiarities of the sciatic features in normal and tomographic studies are the same as in the lesion of the medial sinus wall, but are more clearly visible due to the characteristic anatomical structure and smaller superposition with the surrounding bone formations. Changes in the sinus lower wall are rare, that. Probably, it is connected with its considerable thickness.
  • Lattice labyrinth. Differences in the X-ray picture of the lesions of the latticed labyrinth with Wegener's granulomatosis and the chronic inflammatory process have not been revealed. With this and other diseases, the pattern of intercellular septa is poorly differentiated or missing, the lattice plate is thinned or partially destroyed, the latticed labyrinth is somewhat expanded in comparison with the opposite side.
  • The sphenoid sinus. The radiological picture depends on the degree of decrease in the pneumatization of the sphenoid sinus. The walls of the sphenoid sinus are thinned. The body of the sphenoid bone and its wings on the process side have a less intense pattern. Significant changes occur in the area of the upper and lower orbital gaps: their lumen becomes cloudy, the contours are fuzzy and uneven, sometimes usuric. The defeat of the sphenoid sinus with Wegener's granulomatosis is much less common than the maxillary sinus, however, the possibility of such damage should be remembered.

It is necessary to take into account changes in the lungs that are established during 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, nodular periarteritis, etc.); when there is a perforation in the cartilaginous part of the septum of the nose - from tuberculosis, and in the bone-cartilage department - from syphilis. Further progression of ulcerative-necrotic process in the nasal cavity and paranasal sinuses requires differential diagnosis with malignant neoplasms.

[1], [2], [3], [4], [5], [6], [7], [8]