Pleuroparenchymal Fibroelastosis With Progressive Bronchiectasis and Apical Pneumothoraces in a Patient With Severe Chronic Obstructive Pulmonary Disease and Chronic Pseudomonas Colonization: A Case Report

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 <i>Pseudomonas</i> 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 <i>Escherichia coli</i>, <i>Achromobacter</i> species, and <i>Candida</i>. 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.