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Tracheobronchopathia osteochondroplastica

ORPHA3348
Synonym(s) Tracheopathia osteoplastica
Prevalence Unknown
Inheritance Unknown
Age of onset Childhood
Adult
Elderly
ICD-10
  • J98.0
OMIM
UMLS -
MeSH -
MedDRA -

Summary

Tracheobronchopathia osteochondroplastica (TO) is an idiopathic and benign disease of the large airways characterized by submucosal osteocartilaginous nodules presenting in the trachea with or without the involvement of the major bronchi.

The prevalence is unknown but approximately 400 cases have been reported worldwide to date. There is a male predominance in TO.

The disease usually presents in the sixth to seventh decade of life but individual cases in children as young as 5 have been reported. The characteristic feature of the disease is the presence of diffuse submucosal nodules comprised of cartilaginous or osseous metaplastic tissue (of 1-3mm in diameter), usually located in the distal two thirds of the trachea and proximal bronchi but potentially anywhere along the anterior and lateral walls of the tracheobronchial tree (but sparing the posterior wall). Manifestations can be secondary to obstruction or ulceration and include hoarseness, dry throat, chronic and often productive cough, dyspnea, wheezing and hemoptysis. Some patients are asymptomatic. Recurrent respiratory infections (such as pneumonia) are also common. The lesions are benign and the disease course usually remains stable when monitored and treated symptomatically. In rare cases obstructive lung collapse has been reported. Significant disease progression has been reported in about 17% of cases

The etiology is unknown but there are theories suggesting that chronic infection, chemical or mechanical irritation, degenerative abnormalities, and genetic predisposition may act as contributing factors.


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