Clinical and Endoscopic Findings
Wegener granulomatosis is characterized by a great variety of presentations, including systemic and local manifestations. General symptoms may be weakness, arthralgia, neurologic deficits, unexplained fever, malaise, and weight loss. Otitis media and hearing loss, subglottic stenosis, and oropharyngeal lesions, such as ulcers, are common presentations in the otolaryngologic district. However, the sinonasal tract is by far the most frequently involved area, with
85% and 68% of patients suffering from sinusitis and rhinitis, respectively. The most common complaints are epistaxis, crusting, and nasal obstruction. Nasal mucosa appears covered by crusts, with superficial hemorrhages. Especially in advanced stages and/or in the presence of an aggressive form of the disease, it is possible to identify necrosis and resorption of the septal cartilage, turbinates, and lateral bony walls of the nasal cavities; mucosa is even more friable and inclined to hemorrhage (Fig. 6.23). Patients with an extensive septal perforation may present a typical deformity of the nasal pyramid called "saddle nose". When the granuloma-tous process directly involves the lacrimal pathway or the presence of abundant scar tissue obstructs the nasolacrimal duct, chronic dacryocystitis with epiphora may be observed.
Orbital involvement may occur in different forms. Exophthalmos and diplopia may be related to intraorbital pseudotumor, cranial nerve palsy, or lacrimal gland enlargement; visual impairment due to compression of the optic nerve is also possible. Direct ocular globe involvement may present with uveitis, episcleritis, conjunctivitis, or retinitis (Provenzale et al. 1996).
Diagnosis is based on clinical, serological, histo-logical, and imaging data. The clinical picture may sometimes be slightly nonspecific; however, nasal crusting and otitis media resistant to common antibiotic therapy must arouse the suspicion of Wegener granulomatosis. Among laboratory tests, "antineu-trophil cytoplasmic antibodies" with a cytoplasmic pattern (c-ANCA) positivity is the most accurate indicator for Wegener granulomatosis. In the systemic form, serologic titer of this antibody is sensitive and specific, with values reaching 92% and 100%, respectively (Yang et al. 2001). In localized Wegener granulomatosis, however, this test lacks sensitivity (Lloyd et al. 2002). The erythrocyte sedimentation rate is elevated in most patients with active disease; it may be used as a predictor of treatment outcome and therefore as a prognostic index (Takwoingi and Dempster 2003). In patients without c-ANCA posi-tivity, it is otherwise crucial to obtain a biopsy at the level of involved areas. At histology, characteristic elements for Wegener granulomatosis are necrotizing vasculitis, with fibrinoid necrosis of small and medium vessels wall (Lloyd et al. 2002).
Differential diagnosis includes all the conditions that can present with a midline destructive lesion: lymphoproliferative disorders (T-cell lymphoma and angiocentric natural killer T-cell lymphoma), sar-coidosis, tuberculosis, and cocaine induced lesions.
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