Tracheobronchopathia Osteochondro-What? A Rare Disorder of the Airway

ABSTRACT

Airway abnormalities may be due to a multitude of conditions. Symptoms do not occur until there is very significant narrowing, and therefore these conditions are often incidentally found. It is important to consider a broad differential.This case is a 47 year old woman with history of mild intermittent asthma and chronic sinusitis who presented to the emergency department with four weeks of malaise, shortness of breath, cough, and two days of hemoptysis. On CT imaging she was found to have tracheal wall thickening, calcifications and projections along the trachea, as well as extensive right upper and middle lobe, and lingular ground glass opacities as seen in figure 1. With hindsight, similar tracheal abnormalities were noted on chest x-ray in 2019. During admission, ENT was consulted and she was noted to have saddle nose deformity. Nasal biopsy was performed and revealed evidence of chronic inflammation, but no other abnormalities. Extensive workup was performed including rheumatologic workup with mildly positive ANA, mildly elevated ESR and CRP, negative ANCAs, MPO, PR3, RF. Bronchoscopy was pursued and direct visualization of nodules were noted along the anterior and lateral aspects of the trachea, as well as narrowing of the right upper lobe segmental bronchi, also seen in figure 1. Bronchoalveolar lavage cultures revealed Citrobacter freundii complex, Group G beta hemolytic streptococcus, Providencia rettgeri, Enterococcus avium, Pseudomonas aeruginosa, Klebsiella pneumoniae, in addition to rhinovirus and COVID-19. Biopsy was not performed due to patient intolerance of the procedure and desaturation. Ultimately, the pulmonary infiltrates were felt to be a separate process from the tracheal nodules. She improved clinically, and was discharged on antibiotic therapy with a plan for close outpatient follow up.Tracheobronchopathia osteochondroplastica (TO) is a rare condition. Imaging is often the first clue, as symptoms come late in disease course. The differential diagnosis of imaging findings consistent with TO include relapsing polychondritis, granulomatosis with polyangiitis, amyloidosis, along with a host of others. Diagnosis of TO often requires multidisciplinary involvement to evaluate other etiologies,and ultimately bronchoscopy for direct visualization. (Table Presented).