Recurrent Wegener's granulomatosis with subglottic granulation

While following the course of patients with Wegener's granulomatosis (WG) in which it had been possible to achieve a remission, we encountered a patient in whom we observed granuloma formation in the subglottis. A tracheotomy was performed because of the risk of respiratory distress, and after that there was a recrudescence of the patient's WG. During the course of the above, the patient remained cANCA (anti-neutrophil cytoplasmis antibody with cytoplasmic pattern)-negative. When these facts were considered, it appeared that when the lesions are localized and progressing gradually, cANCA positivity does not develop. While cANCA test has gradually become a powerful tool for the diagnosis of WG, a comprehensive physical examination to identify localized lesions, together with biopsy and histopathological examination, should allow the diagnosis of WG.