[An atypical case of Wegener's disease]

The authors report on a case of Wegener's syndrome diagnosed fortuitously by the discovery of a perforation of the nasal septum during a bronchial fibroscopy for monitoring of a pulmonary fibrosis. In addition to the late appearance of the nasal perforation, the clinical picture includes classic polyvisceral impairment with pulmonary fibrosis, polyarthralgias and renal insufficiency. After 21 months of treatment, a stabilization of the visceral lesions was recorded, with the all but total disappearance of the E.N.T. lesions.