title: Critical airway involvement in relapsing polychondritis.
abstract: Relapsing polychondritis (RP) is a rare multisystem disease characterised by progressive inflammation and destruction of cartilaginous structures. Airway involvement in RP occurs in approximately 50% of cases. We present a 70-year-old woman with a diagnosis of early adult onset asthma. She required multiple hospital admissions for recurrent episodes of acute severe dyspnoea for 1 year. These were treated as asthma. Months later she developed saddle nose deformity and hoarseness of voice. CT revealed tracheal/bronchial wall thickening and luminal narrowing. Based on these findings, RP was diagnosed. Insertion of a tracheobronchial stent was necessary due to severe tracheobronchomalacia. This intervention improved the patient's dyspnoea immediately. This case is reported to raise awareness of airway involvement in RP and discuss its current management. Early diagnosis of RP is essential to allow prompt treatment and to decrease the risk of life-threatening airway collapse.
authors.affiliation: Medical School, University of Bristol, Bristol, UK.
authors.forename: Camilla
authors.initials: C
authors.lastname: Gorard
authors.affiliation: Department of Respiratory Medicine, Gloucestershire Royal Hospital, Gloucester, UK.
authors.forename: Saleheen
authors.initials: S
authors.lastname: Kadri
mesh_terms: D000368:Aged; D001249:Asthma / Q000150:complications / Q000188:drug therapy; D003937:Diagnosis, Differential; D004417:Dyspnea / Q000209:etiology* / Q000628:therapy; D005260:Female; D006801:Humans; D009667:Nose Deformities, Acquired / Q000209:etiology; D011081:Polychondritis, Relapsing / Q000150:complications* / Q000000981:diagnostic imaging*; D012008:Recurrence; D015607:Stents; D014057:Tomography, X-Ray Computed; D055089:Tracheobronchomalacia / Q000209:etiology* / Q000601:surgery