Allergy and Airway |

Tracheobronchopathia Osteochondroplastica in Mixed Connective Tissue Disease FREE TO VIEW

Padmanabhan Premkumar, MD; Mohsin Ehsan, MD; Bimalin Lahiri, MD
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University of Connecticut Health Center, West Hartford, CT

Chest. 2013;144(4_MeetingAbstracts):56A. doi:10.1378/chest.1703759
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SESSION TITLE: Airway Student/Resident Case Report Posters

SESSION TYPE: Medical Student/Resident Case Report

PRESENTED ON: Tuesday, October 29, 2013 at 01:30 PM - 02:30 PM

INTRODUCTION: Tracheobronchopathia osteochondroplastica (TPO) is a rare benign disorder of the endobronchial tree. TPO can be associated with various metabolic, inflammatory or neoplastic disorders but its pathogenesis remains unknown[1]. We report a rare case of TPO in a patient with newly-diagnosed mixed connective tissue disease (MCTD).

CASE PRESENTATION: A 54 year old female presented with progressive shortness of breath and non-productive cough. Her past medical history was significant for asthma, gastroesophageal reflux, hypertension and recurrent episodes of bronchitis. Review of systems was positive for atypical chest pain, orthopnea, occasional dizziness and episodes of epistaxis. She was a lifelong non-smoker from Puerto Rico with no occupational exposures. On examination, she had bilateral sclerodactyly, multiple facial telangectasias, bilateral inspiratory crackles on ascultation and swan neck deformity of her right wrist. Her labs showed normal blood count and chemistries. Rheumatologic evaluation revealed positive anti-nuclear antibody, rheumatoid factor, ribonucleoprotein, anti Smith antibody and anti thyroid antibody. A CT scan of her chest showed multiple tracheal excrescences sparing the posterior tracheal wall with calcifications. Her spirometry was normal with moderately reduced DLCO. A subsequent bronchoscopy showed multiple nodular lesions extending throughout the tracheal surface and bilateral bronchi while sparing the posterior membranous wall. Pathological examination of the bronchial biopsy showed bronchial epithelium with squamous metaplasia and underlying ossified connective tissue. The clinical findings, CT scan and biopsy results confirmed the diagnosis of tracheobronchopathia osteochondroplastica. She was also given a diagnosis of MCTD and started on oral steroids. Patient has been followed up on an outpatient basis with progressive decline in her diffusion capacity.

DISCUSSION: Cartilaginous airway thickening has been described as a known clinical entity for many years. The differential diagnosis usually include TPO, atrophic polychondritis, transbronchial amyloidosis or a granulomatous pathology such as Wegener’s[1]. TPO is a benign process that involves the formation of multiple cartilaginous or bony sub mucosal nodules that project into the transbronchial lumen. There appears to be an association between chronic inflammatory states and TPO. Although some case reports of relapsing polycondritis and TPO exist there are very few cases of association with autoimmmune rheumatologic processes specifically MCTD.

CONCLUSIONS: TPO is a rare clinical entity that should be considered in patients with abnormal CT findings characteristic of cartilaginous tracheal involvement particularly in chronic inflammatory states like MCTD.

Reference #1: 1. Prince JS, Duhamel DR, Levin DL, et al. Nonneoplastic lesions of the tracheobronchial wall: radiologic findings with bronchoscopic correlation. Radiographics 2002; S215-230

DISCLOSURE: The following authors have nothing to disclose: Padmanabhan Premkumar, Mohsin Ehsan, Bimalin Lahiri

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