Wegener's granulomatosis presenting as subglottic stenosis.

Wegener's granulomatosis is an uncommon disease process that causes necrotizing vasculitis predominantly in the lungs, kidneys, and upper air-ways. Subglottic stenosis has been observed in up to 20% of patients with established diagnosis of Wegener's granulomatosis, but it is rarely the initial manifestation of the disease. This case report presents a patient with Wegener's granulomatosis who had subglottic stenosis as predominant initial manifestation, without signs of lung or kidney involvement. The diagnosis was unclear initially due to the absence of damage to these organs and to the nonspecificity of the initial biopsy findings. After stabilizing the patient, an elevated cytoplasmic pattern of antineutrophil cytoplasmic antibodies titer was obtained, which played an important role in the diagnostic process and led to a second biopsy that showed vasculitis. The patient had an excellent response to prednisone, methotrexate, and trimethoprim/sulfamethoxazole, but as with many such cases, she also required tracheo-laryngoplasty.