9586                IDIOPATHIC CYSTIC PULMONARY FIBROSIS

The patient was a woman aged 64 who had been treated for diabetes for 12 years.  Congestive cardiac failure was also present.  She was admitted because of continued vomiting for 6 weeks and a skin rash.  Mercury poisoning from over-use of mercurial diuretic was suspected and the urine contained large amounts of mercury.  She died after a short illness.  At portmortem there was evidence of old myocardial infarction as well as nephrosclerosis.  The lung findings were apparently incidental.

The specimen consists of the right lung sectioned to show a wide band of cystic fibrosis extending beneath the pleura along the posterior and inferior borders o the lower lobe and to a lesser extent in the anterior segment of the upper lobe.  There are many small cystic cavities averaging about 2 mm in diameter but varying up to about 6 mms.  Congested fibrous septa intervene between the cysts.  Medially the process ends sharply in a sinuous serpiginous border, internal to which the lung substance appears essentially normal.  The pleural surface shows the characteristic irregular cobble-stone appearance overlying the diseased cystic lung.  Histology shows cystic spaces lined by cubical to columnar epithelium, fusing in some places to form giant cells.  There is marked interstitial fibrosis.  Small arteries have thick walls.