Endoscopic manifestations of Wegener disease.

The diagnostic criteria for upper airway necrotizing diseases such as Wegener's granulomatosis (WG) are well defined. However, the differentiation with other diseases of unknown aetiology remains difficult. Establishing an early diagnosis and prompt treatment is very important to minimize loss of function and cosmetic deformity. In the last fifteen years, we observed and treated eight cases of WG. In six patients, WG appeared primarily in the nose area. One patient presented primarily middle ear and secondary nose involvement, whereas in one case, the inner ear was affected too. One patient did not present nasal involvement although the middle ear and several cranial nerves were involved. For these patients, the pathognomonic diagnosis had not been confirmed by biopsy at the early stage and we introduced an immunosuppressive therapy on the basis of clinical and histological findings only. For the eight patients, the treatment stopped the disease process but for one patient, we abandoned drugs because of side effects. Finally, only four of the cases only had a pathognomonic histological diagnosis of WG. In four cases, serologically anticytoplasmic antibodies against neutrophil granulocytes (Anca) were observed early. Both clinical and histological findings and determination of these antibodies give the possibility to solve difficulties in WG differential diagnosis.