CHYLOTHORAX AND SARCOIDOSIS

The association of sarcoidosis and chylothorax is rare. A patient aged 64 presented with mediastinal sarcoidosis. Two years after the diagno sis he developed a right chylothorax. The computed tomographic examina tion showed a retraction of the right hemithorax associated with large mediastinal nodes, without signs of interstitial lung disease. Pulmon ary function tests revealed a restrictive ventilatory defect with a Fo rced Vital Capacity (FVC) of 53% and a Forced Expired Volume in one se cond (FEVI) of 54% from the predicted values; the FEVI/FVC ratio was 7 8%. Steroid therapy was started at 1 mg per kilogram per day for three months then progressively decreased to 15 mg per day. Inspite of the steroid therapy there was as well biological evidence of continued dis ease activity (bronchoalveolar lavage lymphocytosis and increased seru m angiotensin converting enzyme), as persistance of pleural effusion a nd mediastinal adenopathy; the contraction of the right hemithorax inc reased. Restrictive ventilatory defect worsened with a FVC of 44% and an FEVI of 47% of the predicted values. The presence of a significant contraction of the hemithorax and of a severe restrictive ventilatory defect suggested the existence both of pleural fibrosis and of a compr ession of the main lymphatic pathways responsible for the chylothorax.