25670 CHRONIC INTERSTITIAL PULMONARY FIBROSIS
The patient was a woman who presented with dyspnoea when aged 70. On X-ray there were patchy opacities in both lung fields and investigation showed diffuse interstitial pulmonary fibrosis. She also had severe urinary tract infection (Klebsiella) with considerable elevation of blood urea nitrogen. There was no peripheral oedema. She was readmitted 4 years later, at which time the dyspnoea was more severe and frank congestive cardiac failure was present, partly controlled by digoxin and diuretics. Her J.V.P. was elevated 3 cms but there was no peripheral oedema and the liver was not noticeably enlarged. The ESR was 110. She deteriorated steadily and died after a few weeks. At autopsy the right lung weighed 445 grams and the left lung 435 grams.
The specimen consists of half a left lung divided in the sagittal plane. The lung is small and shrunken and the pleural surface is somewhat nodular and shows considerable anthracotic reticulation. On the cut surface there is irregular fibrous scarring throughout the lung, most evident in the apical and sub-apical regions of the upper lobe and alone the posterior border of the lower lobe. There is some congestion in the apical and basal segments of the lower lobe. Histology shows irregular ill-defined stellate areas of fibrosis of varying density and varying apparent age. In some of the scars there is marked bronchialisation of small included air spaces, and in general there is considerable fibrous interstitial thickening of alveolar walls, which are infiltrated by chronic inflammatory cells and in which vessels are inconspicuous and scanty. Proliferation of smooth muscle has occurred in some of these alveolar walls. Some alveolar spaces contain a few macrophages and others a few clusters of cholesterol crystals. There is hypertrophy of the muscle coat of medium-sized arteries in the fibrous tissue. There is squamous metaplasia of some bronchioles. The intervening lung shows moderate diffuse atrophic emphysema.
