Unusual case of pharyngeal-laryngeal Wegener's granulomatosis

Wegener's granulomatosis is characterized by necrotizing granulomas with vasculitis in the upper and lower respiratory tracts, systemic vasculitis and focal necrotizing glomerulonephritis. Along with this so-called classic form, a new clinical entity has been added in recent years: granulomatosis located in a single site without any form of systemic manifestation. In otorhinolaryngology the disease is manifest at the outset in 72% of the cases while it appears later in an extremely high percentage of the cases (92-100%). The nasal sinus site is the region most often affected while the areas least affected are the larynx (16% with only 2% at onset) and oropharynx (10% with only 2.5% at onset). The authors present a clinical case of pharyngeal-laryngeal localization and list the most important otorhinolaryngological sites for onset of this disorder. They discuss the diagnostic aspects of this pathology: clinical history, histopathological findings, serological results and, in particular, the positive testing of anticytoplasmatic antibodies vs. neutrophil polymorphonucleate granules (c-ANCA). In this regard, emphasis is placed on the fact that c-ANCA are highly specific being positive in 97% of the cases. Sensitivity appears to depend on activity and extension of the disease with values fluctuating from 60% for locoregional forms to 93% for generalized forms. The authors indicate that the location found in the present case--at the laryngeal-pharyngeal region boundary--is important for the clinical history, histopathological data and response to specific therapy (cyclophosphamide and desamethasone) for a diagnosis of localized Wegener granulomatosis, even when c-ANCA do not test positive.