Tracheobronchopathia osteochondroplastica and selective IgA deficiency

Abstract

Tracheobronchopathia osteochondroplastica (TO) is a rare large-airway disorder of unknown cause, characterized by the development of multiple submucosal osseous and/or cartilaginous nodules that protrude into the lumen of the trachea and large bronchi. Although most patients are asymptomatic, some may present with nonspecific respiratory symptoms. A chest computed tomographic scan with multiple protruding nodules in the trachea is suggestive but other diagnoses can present with a similar appearance; hence, definitive diagnosis may require bronchoscopic examination. Numerous cases of TO have been reported in association with different diseases, but no definitive causative explanation has been shown with any of the conditions. Chronic airway inflammation is one of the proposed mechanisms to explain the formation of TO nodules. Most of the reported TO patients have a history of frequent airway infections. Patients with IgA deficiency present with frequent sinopulmonary infections. The present case illustrates the first case of TO associated with IgA deficiency.