Polychondriatis

Few advances in our understanding of the polychondritic disease process have been made within the past year. Clinical studies have emphasized pulmonary manifestations and newer means of evaluation of functional and anatomic upper and lower airway disease. Esophageal dysfunction has been described. Further associations have been established with Sjögren's syndrome and with malignancy. Active disease during pregnancy was not associated with neonatal transmission. Although lacking diagnostic specificity, preliminary studies suggest that serum quantitation of a noncollagenous cartilage matrix protein may correlate with disease activity. Differential diagnosis must now include a recently described form of hereditary chondropathy. Segmental tracheal resection and splinting techniques including use of stents have been advocated for therapeutic management of severe tracheobronchial disease.