Pleuroparenchymal Fibroelastosis With Progressive Bronchiectasis and Apical Pneumothoraces in a Patient With Severe...

Pleuroparenchymal fibroelastosis (PPFE) is a rare condition of progressive interstitial lung disease marked by bilateral upper lobe pneumothoraces and fibrosis. We present the case of a 73-year-old female with a background of bronchiectasis, chronic obstructive pulmonary disease (COPD), and long-standing Pseudomonas colonization, who presented with progressive shortness of breath, chest tightness, and cough. Imaging revealed bilateral apical pneumothoraces and progressive bronchiectatic and fibrotic changes. Sputum cultures grew Escherichia coli, Achromobacter species, and Candida. A diagnosis of PPFE was made based on characteristic clinical and radiographic findings. After discussion in the multidisciplinary team (MDT) and confirmation of the diagnosis, given the non-progressive nature of the pneumothoraces and her poor functional reserve, conservative management was advised. This case highlights the complexity of managing advanced respiratory disease in a severely malnourished patient with chronic infection and multiple comorbidities.