Pleuroparenchymal fibroelastosis

Case contributed by Caitriona Logan
Diagnosis almost certain

Presentation

Asthma symptoms, family history of pulmonary fibrosis.

Patient Data

Age: 45 years
Gender: Male

Chest

ct

Apical pleural thickening, traction bronchiectasis and reticular thickening. Hilar retraction. 

Case Discussion

No history of transplant (this can be seen in CLAD - chronic lung allograft dysfunction). No previous TB, no radiation. Imaging findings are therefore supportive of idiopathic PPFE (pleuroparenchymal fibroelastosis). 

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