Usual interstitial pneumonia - unilateral lung transplant

Case contributed by Dr Bruno Di Muzio

Presentation

Progressive shortness of breath.

Patient Data

Age: 65 years
Gender: Male
X-ray

Chest radiographs

The right lung is small and has diffuse reticular opacities, predominantly seen in the lower zones. The left lung has normal appearances. No pleural effusion. 

X-ray

Chest radiograph (5 years ago)

The lungs have the appearances of reduced volume and peripheral reticular opacities predominantly seen on the left. No pleural effusion.  

The left transplanted lung shows a mild mosaic pattern of attenuation that correlates with some air-trapping on expiration. There is no significant bronchiectasis. Lung herniation along the intercostal space between the 5th and 6th ribs on the left. 

The right lung has marked reduced volume and fibrosis characterized by peripheral septal thickening, honeycombing, and bronchiectasis. A craniocaudal gradient is noted. 

The pleural spaces are clear. 

No lymphadenopathy. Left thyroid lobe is enlarged and dives into the superior mediastinum. Liver low attenuation suggesting steatosis. 

 

Here is the anatomopathological report for the removed native left lung: 

Macroscopy:  A.  Labelled "Left native lung".  The specimen is an uninflated left pneumonectomy, weighing 889g post fixation.  The visceral pleural surfaces are firm and have a diffusely micronodular appearance. Thin fibrous septa connect the left upper and lower lobes.  A large air-filled bulla measuring protrudes from the base of the left lower lobe.  Serial slicing reveals almost solid lung parenchyma, with greenish grey fibrosis present diffusely throughout both lobes.  There are no honeycomb-like changes seen in any of the sections.  The hilar lymph nodes are enlarged and anthracotic. There is relative sparing of the apical portions of the left upper lobe, which shows diffuse dark red congestion.  In this area, the fibrosis is limited to the perivascular and peribronchial spaces.  The pulmonary arterial branches show atherosclerosis distally. B.  Labelled "Station six lymph node".  An enlarged, firm rubbery grey lymph node, 42 x 24 x 8mm.

Microscopy:  A.  Sections of lung show extensive change extending from the pleural surface within which variably sized bronchiolised spaces, are present with intervening areas of smooth muscle hypertrophy, fibrosis and chronic inflammatory cells. Within some foci, plump reactive fibroblastic plugs are still seen, deep to bronchial alveoli.  More medially, the changes appear less marked but there is still increased interstitial fibrosis.  There is no evidence of any in situ or any invasive malignancy.  Pulmonary thromboemboli are not seen.  Vessels show myointimal thickening.  There is no active infection. B.  The sections of lymph node, show reactive dermal features, with central sinus histiocytosis, with histiocytes containing increased carbon and some fine silica particles also seen.   No evidence of any metastatic malignancy.

Conclusion:  A.  Left native lung - endstage pulmonary fibrosis, with features supportive of a diagnosis of usual interstitial pneumonia (UIP).  No malignancy, no evidence of pulmonary thromboemboli. B.  Station six lymph node - anthrasilicosis and sinus histiocytosis with reactive germinal centres.  No evidence of malignancy.

Case Discussion

This patient suffered from a progressive shortness of breath related to pulmonary fibrosis, which has an imaging pattern supportive of usual interstitial pneumonia (UIP). He was submitted to a single left lung transplantation with subsequent improvement of symptoms and quality of life. Although the bronchi were unremarkable, his last CT has demonstrated some left lung air-trapping, which is of concern for bronchiolitis obliterans if clinically supported. 

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Case information

rID: 62155
Published: 5th Aug 2018
Last edited: 6th Aug 2018
System: Chest
Inclusion in quiz mode: Included

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