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Wegener's Granulomatosis Appearing Initially in the Trachea

We treated ten patients with Wegener's granulomatosis whose clinical picture differed only in the stage of the disease at the time of the first visit. All patients were female with a mean age of 30 years and all presented with a picture of high tracheal obstruction. Three died and six have a permanent tracheostomy. Surgical treatment was unsuccessful; corticosteroids provided only transient improvement but cyclophosphamide induced remission. Removal of the stenosis either by open surgery or endoscopically by laser was not successful in accomplishing decannulation.