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Usual interstitial pneumonia

Case contributed by Bruno Di Muzio
Diagnosis certain

Presentation

Shortness of breath.

Patient Data

Age: 55 years
Gender: Female

Chest

x-ray

Bilateral peripheral reticular opacities with a basal predominance and association to traction bronchiectasis and, perhaps, a degree of volume loss. Features are those of pulmonary fibrosis. Enlarged heart silhouette with otherwise normal mediastinal contours. 

Chest

ct

Pulmonary architectural distortion due to inter and intralobular septal thickening associated with traction bronchiectasis and peripheral micro and macro honeycombing. There is a craniocaudal gradient of distribution of this pattern. No suspicious lung lesions. Pleural spaces are clear.  

Macroscopy: "Native left lung". 300 g (uninflated weight), 192 x 118 x 75 mm with upper lobe 144 x 70 x 72 mm and lower lobe 160 x 90 x 55 mm. Hilar lymph nodes measure 6-12 mm. The visceral pleural surface is diffusely microcystic. Sectioned on a parasagittal plane to reveal cut surface is tan/brown and partly spongy, with honeycombing, predominantly in the basal aspect of both lobes. There are no masses or endobronchial lesions. The pulmonary vessels appear atherosclerotic and there is no evidence of intravascular thromboembolism.

"Native right lung". 350 g (uninflated weight), 185 x 115 x 120 mm with upper lobe 95 x 85 x 65 mm, middle lobe poorly inflated, 35 x 80 x 45 mm and lower lobe 96 x 96 x 130 mm. Hilar lymph nodes measure 5-14 mm. The visceral pleural surface is diffusely microcystic. Sectioned on a parasagittal plane to reveal cut surface is tan/brown and partly spongy, with honeycombing, predominantly in the basal aspect of the upper and lower lobes. The middle lobe is densely fibrotic with very little residual normal parenchyma, and metal staples laterally, suggestive of previous surgery. There are no masses or endobronchial lesions. The pulmonary vessels appear atherosclerotic and there is no evidence of intravascular thromboembolism.

Microscopy: Multiple sections taken of the lung parenchyma, show a peripheral honeycomb pattern with expanded bronchioles lined by a single layer of columnar epithelium forming cystlike structures with intervening fibrosis, with scattered chronic inflammatory cell clusters and smooth muscle hypertrophy around the expanded bronchiolar walls. Vessels show myxoid degeneration within the wall and some myointimal hyperplasia. There is no evidence of vasculitis. Lymph nodes show sinus histiocytosis and reactive germinal centers.

Conclusion: ​Left and Right native lung–UIP-like pattern of end-stage pulmonary fibrosis. 

Case Discussion

This patient was known from the respiratory clinic with the diagnosis of pulmonary fibrosis for a few years. MDM discussion was for the diagnosis of idiopathic pulmonary fibrosis after all the appropriate workup. Given her progressive deterioration, she was then offered a lung transplant. 

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