15652              DIFFUSE IDIOPATHIC INTERSTITIAL FIBROSIS

The patient was a man aged 72 who had had several operations for peptic ulcer including a partial gastroectomy and a subsequent operation for adhesions.  Despite this haemoptysis continued.  Two years before his death, chest X-ray showed cardiac enlargement and diffuse interstitial pulmonary fibrosis.  He was treated with small doses of steroids.  He was then found to be diabetic and tolbutamide treatment was begun.  He remained moderately well, but exertional dyspnoea gradually worsened.  A week before his last admission he vomited 600 ml of blood.  On admission there was congestive cardiac failure with J.V.P. elevated 8 cms.  Barium meal showed a large stomal ulcer.  After resuscitation and transfusion lung function tests showed gross arterial oxygen desaturation with reduced CO2 tension.  The serum bicarbonate was low and the blood pH was high.  He died quite suddenly from a combination of cardiac and pulmonary failure with supervening extra-renal uraemia apparently precipitated by the haematemesis.  At postmortem the right side of the heart was grossly enlarged and there was chronic passive venous congestion of organs.  A stomal ulcer was found.

The specimen consists of the lungs sectioned to show nodular areas of idiopathic fibrosis with formation of small cystic cavities up to about 3 mms in diameter.  These areas have ill-defined margins and are most evident beneath the pleura, particularly of the lower lobes.  The intervening lung shows a little acute pneumonia especially in the left lower lobe.  Hilar glands are anthracotic but not grossly enlarged.  The major air passages appear normal.  Histology shows nodular and reticular masses of interstitial fibrosis with bronchialization of small cystic cavities, many of which contain purulent exudate.  Bands of smooth muscle are present in the fibrous tissue and small branches of the pulmonary arteries show some medial thickening.