Definition and Epidemiology
Wegener granulomatosis is a chronic, granulomatous vasculitis mainly affecting the respiratory tract and
Fig. 6.21a-c. Acute fulminant fungal rhinosinusitis. TSE T2 axial plane (a), Gd-DTPA SE T1 axial plane (b), VIBE coronal plane (c). Inhomogenous inflammatory material centered at the level of vertical process of maxillary bone and along medial wall of maxillary sinus. Note the absence of contrast uptake, related to ischemic necrosis (invasion and obliteration of vessels). Extensive bone destruction is detected, involving nasal septum (1), infiltration of premaxillary subcutaneous fat tissue (2), middle turbinate (3), hard palate and alveolar process of maxillary bone (4). The lesion contacts the ethmoid roof (5) with no signs on intracranial extension b a c a
Fig. 6.22a-c. Chronic invasive fungal rhinosinusitis. Coronal Gd-DTPA SE T1 (a), coronal Gd-DTPA VIBE (b), reformatted sagittal Gd-DTPA VIBE (c). A hypointense fungus ball is retained within the sphenoid sinus. At the level of the sinus roof both the hyperintense mucosa and the hypointense cortical bone are focally interrupted (arrows). A plaque-like inhomo-geneous lesion is demonstrated along the intracranial surface of the planum sphenoidale invading the right superior orbital fissure (arrowheads)
b a c the kidneys. More rarely, other organs and systems may be involved. A sinonasal tract localization is present in up to 91% of patients with systemic Wegener granulomatosis (Yucel et al. 2002). An isolated form of involvement, without other localizations, has also been described (Lloyd et al. 2002).
The etiology of the disease is still unknown; however, the hypothesis of an autoimmune process is at present the most favored.
The incidence ranges between 0.5 and 8 new cases per 1,000,000 inhabitants per year (Takwoingi and Dempster 2003). The age at presentation is extremely variable, varying from 9 to 78 years (Hoffman et al. 1992; Takwoingi and Dempster 2003), thus confirming that the observation in the pediatric age is not exceptional. Moreover, there is a moderate predominance in male patients.
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