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Severe Dynamic Airway Collapse: A Rare Initial Presentation of Relapsing Polychondritis FREE TO VIEW

Sarah Robison, MD; Andres Borja Alvarez, MD
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Mayo Clinic Florida, Jacksonville Beach, FL

Chest. 2015;148(4_MeetingAbstracts):876A. doi:10.1378/chest.2245663
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SESSION TITLE: Pulmonary Manifestations of Systemic Disease Student/Resident Case Report Posters I

SESSION TYPE: Student/Resident Case Report Poster

PRESENTED ON: Tuesday, October 27, 2015 at 01:30 PM - 02:30 PM

INTRODUCTION: Relapsing polychondritis can present with a variety of symptoms, making diagnosis difficult. Although up to 50% of patients will eventual have pulmonary manifestations, airway involvement as a presenting symptom is very rare. Airway involvement contributes significantly to the mortality of the disease.

CASE PRESENTATION: A 62-year-old man was evaluated in pulmonary clinic for voice hoarseness, dyspnea on exertion, and dry cough that had been increasing in severity over the last 6 months. Pulmonary function tests showed severe obstruction. A CT scan of the chest and neck showed thickening and calcification of the anterior trachea with sparing of the posterior wall. Soon after, the patient was admitted to an outside facility for respiratory distress, intubated and placed on positive pressure mechanical ventilation. Mechanical ventilation was complicated by high peak pressures and pneumomediastinum. He was transferred to our facility for further management. A bronchoscopy showed a stenotic trachea with severe dynamic expiratory collapse. High dose systemic corticosteroids were started. Airway intervention involved tracheostomy and ultraflex uncovered stents placed in the trachea, the left main stem bronchus and in the bronchus intermedius. A biopsy of the tracheal cartilage was obtained during one of the procedures. Pathology revealed cartilage with focal necrosis, granulation tissue and acute and chronic inflammation. The patient was diagnosed with relapsing polychondritis. He had no history of autoimmune or connective tissue diseases and no history of inflammation of the nose, eyes, ears, or joints. He improved and was eventually discharged on supplemental oxygen by trach collar.

DISCUSSION: Relapsing polychondritis is characterized by recurrent episodes of inflammation of cartilaginous structures. The disease typically involves auricular or nasal chondritis, arthritis, or ocular inflammation. However, it can involve the laryngeal, tracheal, or bronchial cartilage. This patient had no prior symptoms to suggest an underlying inflammatory disease process. Airway involvement is rarely the presenting symptom. The diagnosis was made in our patient by the expiratory obstruction, characteristic findings on imaging, and pathology of the tracheal cartilage.

CONCLUSIONS: In relapsing polychondritis, structural compromise of the major airways contributes significantly to the mortality of the disease. Patient’s with airway involvement are at risk for sudden unexplained death due to airway collapse. There is no curative treatment, but timely medical treatment and airway intervention may improve survival.

Reference #1: Kent PD, Michet CJ Jr, Luthra HS: Relapsing Polychondritis. Curr Opin Rheumatol 2003, 16:56-61.

Reference #2: Rafeq S, Trentham D, Ernst A: Pulmonary Manifestations of Relapsing Polychondritis. Clin Chest Med 2010, 31:513-5-18.

Reference #3: Sarodia BD, Dasgupta A, Mehta AC: Management of Airway Manifestations of Relapsing Polychondritis. Chest 1999, 116:1669-1675.

DISCLOSURE: The following authors have nothing to disclose: Sarah Robison, Andres Borja Alvarez

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