[Chronic atrophic polychondritis. Clinical polymorphism and severity of respiratory lesions]

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.