CHRONIC DESTRUCTIVE POLYCHONDRITIS - CLINICAL POLYMORPHISM AND SEVERITY IN CASES WITH RESPIRATORY LOCALIZATION

The authors report the case of chronic destructive polychondritis (PCA ) with extensive and predominant laryngo-tracheal involvement. The res piratory localisation of PCA is apparent in around 50% of cases. In a quarter of patients presenting with this type of localisation, the out come is fatal. Tracheal disorders as the presenting feature is a rarit y, as in the majority of cases it occurs either concomitantly or after the appearance of classic cartilagenous disease in the nose or ear. T he 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 laryngo-tracheal 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 d ue to a thickening of the walls with oedema and inflammatory granuloma s, and to collapse of the lumen caused by the destruction of cartilage and by fibrous scarring of the tracheal walls. Either general or loca l treatment is virtually non-existent and the prognosis of these exten sive respiratory forms of the disease is very poor.