title: [Chronic atrophic polychondritis. Clinical polymorphism and severity of respiratory lesions].
abstract: The authors report the case of chronic destructive polychondritis (PCA) with extensive and predominant laryngo-tracheal involvement. The respiratory localisation of PCA is apparent in around 50% of cases. In a quarter of patients presenting with this type of localisation, the outcome is fatal. Tracheal disorders as the presenting feature is a rarity, as in the majority of cases it occurs either concomitantly or after the appearance of classic cartilagenous disease in the nose or ear. The diagnosis of the airways disease rests on the interpretation of the flow volume curve and the CT scan of the inspiration and expiration. Taking account of the absence of any correlation between the disturbed respiratory function and the laryngotracheal bronchial lesions, the authors stress the value of an endoscopic assessment with a fibroscope of reduced calibre. The narrowing of the laryngo-tracheal region is due to a thickening of the walls with oedema and inflammatory granulomas, and to collapse of the lumen caused by the destruction of cartilage and by fibrous scarring of the tracheal walls. Either general or local treatment is virtually non-existent and the prognosis of these extensive respiratory forms of the disease is very poor.
authors.affiliation: Service de Pneumologie, Hôpital de la Conception, Marseille.
authors.forename: P
authors.initials: P
authors.lastname: Astoul
authors.forename: V
authors.initials: V
authors.lastname: Quenee
authors.forename: V
authors.initials: V
authors.lastname: Doucet
authors.forename: J P
authors.initials: JP
authors.lastname: Vialette
authors.forename: C
authors.initials: C
authors.lastname: Boutin
mesh_terms: D000368:Aged; D005260:Female; D006801:Humans; D007818:Laryngeal Diseases / Q000175:diagnosis* / Q000628:therapy; D007828:Laryngoscopy; D011081:Polychondritis, Relapsing / Q000175:diagnosis* / Q000628:therapy; D012129:Respiratory Function Tests; D014057:Tomography, X-Ray Computed; D014133:Tracheal Diseases / Q000175:diagnosis* / Q000628:therapy; D014986:Xeroradiography
vernacular_title: Polychondrite chronique atrophiante. Polymorphisme clinique et gravité des localisations respiratoires.