Presentation
Chronic shortness of breath.
Patient Data
Extensive interstitial lung markings throughout the lungs are consistent with chronic interstitial lung disease with equivocal lung volume loss. No pleural effusion. No pneumothorax. Enlarged heart silhouette.
Extensive pulmonary fibrosis characterised by peripheral inter and intralobular septal thickening, traction bronchiectasis, and micro and macrocytic honeycombing. These demonstrate a relative craniocaudal gradient of distribution. There is a moderate degree of volume loss, most pronounced on the left where there is an elevation of the left main bronchus and hemidiaphragm. No confluent airspace consolidation to suggest an acute infective process.
The heart is enlarged and the pulmonary trunk is dilated in keeping with a degree of pulmonary arterial hypertension. There are prominent mediastinal and hilar nodes, the largest right hilar node measuring up to 15 mm in short axis, unchanged from previous imaging (not shown).
Macroscopy: "Lung transplant Left Native Lung": 366 g, 162 x 145 x 80 mm, comprising upper lobe, 150 x 105 x 85 mm, and lower lobe, 145 x 60 x 75 mm. The pleural surface is brown, with a reticulated pattern of anthracosis pigment, and slight nodularity. The cut section shows peripheral areas of cystic change and dilated airways, up to 15 mm in diameter, which extend to the periphery. The soft brown spongy tissue is replaced by a firm, rubbery tissue, especially peripherally. There are some areas of central sparing, especially in the upper lobe. The areas of change show a predominantly subpleural distribution. No pulmonary emboli or endobronchial lesions are identified. Within the pulmonary arteries, there is a moderate amount of pale yellow atheromatous plaque. "Lung transplant Right Native Lung": 577 g, 185 x 175 x 85 mm, comprising upper lobe, 125 x 130 x 85 mm, middle lobe, 70 x 85 x 80 mm, and lower lobe, 135 x 105 x 85 mm. The pleural surface is brown and smooth, with dark anthracotic pigment. The cut section shows spongy brown parenchyma centrally. Peripherally, in a subpleural distribution, there is firm tan tissue, with cystic change and honeycombing. Cysts in the upper lobe measure up to 20 mm in diameter and filled with a pale yellow material. There is a small amount of atherosclerotic plaque within pulmonary arteries. No pulmonary emboli or endobronchial lesions are identified.
Microscopy: Sections of the lung, show focal areas within which there is cyst formation, with this line in part by respiratory and in part by cuboidal and columnar epithelium with intervening fibrous connective tissue and chronic inflammation focally at the edge of the fibrotic areas, the interstitium shows chronic inflammatory cell clusters with reactive overlying pneumocytes and some areas within which there are plump interstitial fibroblastic plugs. The pulmonary artery shows atherosclerosis. Giant cells are not seen. There is no vasculitis. There is no dysplasia. There is focal evidence for anthracosis pigment. Bronchioles appear patent. There is no evidence of thromboembolic, dysplasia or fungal hyphae.
Conclusion: Bilateral native lungs – chronic interstitial fibrosis with a UIP pattern.
Case Discussion
Pulmonary fibrosis with a characteristic UIP pattern further confirmed on anatomopathology.
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