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

TL;DR

A 73-year-old woman with COPD and chronic infections developed a rare lung disease called PPFE, marked by pneumothoraces and fibrosis.

Contribution

This case report adds to the limited literature on PPFE in patients with complex respiratory comorbidities and chronic bacterial colonization.

Findings

PPFE was diagnosed based on clinical and radiographic features in a patient with COPD and bronchiectasis.

Conservative management was chosen due to non-progressive pneumothoraces and poor functional reserve.

Chronic Pseudomonas colonization and additional infections were present alongside PPFE.

Abstract

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.

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