课件：ANCA相关性小血管炎.ppt

* Here is a series of CT scans over 3 years from a patient with Wegener’s. This patient had no invasive procedures performed during this time. As mentioned earlier, the avascular necrosis as a result of acute or chronic inflammatory cell infiltrates within the vessel wall or the presence of large epithelioid granulomas with obliterations of adjacent small arteries. The resulting avascular necrosis accounts for the bone destruction which is the most striking change on these CT scans and is seen against a background of generalized mucosal thickening in the nose and sinuses. The destructive process is located initially in the mid-line, affecting the septum and turbinates and typically spreads symmetrically to involve the antra and the rest of the sinuses. Results of this retrospective study of CT scans of28 Wegener’s patients found that 85% had non-specific mucosal thickening in the nasal cavity or paranasal sinuses or other evidence of infection such as fluid levels; 75% had bony destruction The end result of this series is a large single cavity with disappearance of the antral walls, the ethmoid septa, laminae papyracea and cribriform plate, but characteristically sparing the hard palate. I omitted the fourth CT scan depicting this, in favor of showing you all Wegener’s original description of his necroscopy findings. Lund V. Rhinologic changes in WG. The Journal of Laryngology Otology. July 2002, Vol. 116, pp. 565-69 * Figure 2. Hematoxylin eosin stain (magnification x 400) of sinus mucosa revealing chronic inflammation and granuloma formation within a small vessel wall typically seen in WG. Maxillary sinus tissue taken during endoscopic middle turbinectomy and maxillary antrostomy . Final pathology of the sinus contents confirmed acute necrotizing vasculitis with focal granulomatmatous inflammation consistent with WG. Keni Altman (Northwestern). American Journal of Otolaryngology. Skull-base WG resulting in multiple cranial neuropathies. 2005; 26,146-9.