PURPOSE: Relapsing polychondritis (RP) is a rare systemic inflammatory disease affecting cartilaginous structures. The most severe complications involve the tracheobronchial tree and the cardinal symptoms include dyspnea and cough. Life-threatening upper airway obstruction occurs when critical narrowing occurs. Due to rarity of the disease, specific airway complications from RP have not been well described. This study examines the types of airway pathologies in patient with RP.
METHODS: We conducted a retrospective review of patients at our institution with RP seen at rheumatology clinic and referred to the complex airway center for evaluation of suspected airway obstruction. Clinical histories were obtained from the referring rheumatologist. Computed tomography of the trachea with dynamic maneuvers and bronchoscopies were performed to evaluate the extent and severity of airway pathologies.
RESULTS: From 1996 to 2005, 18 patients were seen and evaluated for airway involvements from RP. Diagnoses were based on diagnostic criteria of RP from clinical findings and made by rheumatology. Age ranged from 33 to 80. Sixteen of 18 patients were female. All patients were on medical therapy when seen. Duration of symptoms to diagnosis ranged from 4 months to 19 years. Extra-thoracic manifestations included saddle-nose deformities (10), auricular involvements (4), and dysphagia (1). Four patients had undergone tracheostomies and one patient had a Montgomery T-tube placed previously when initially referred. Two patients had undergone airway interventions including airway stents placement. CT findings included 1) airway stenosis, 2) airway wall thickening with or without calcifications, 3) subglottic stenosis and 4) tracheobronchomalacia. Bronchoscopic evaluations were consistent with CT findings.
CONCLUSION: Patients with relapsing polychondritis with airway involvements have heterogenous spectrum of radiographic and bronchoscopic findings.
CLINICAL IMPLICATIONS: Management of respiratory symptoms in patients with relapsing polychondritis should be specific to airway pathologies.
DISCLOSURE: Arthur Sung, None.