# A Rare Case of Idiopathic Pleuro-Parenchymal Fibroelastosis Presenting With Bilateral Spontaneous Pneumothoraces

## TL;DR

A 67-year-old woman with a rare lung disease called pleuroparenchymal fibroelastosis presented with breathing difficulties and was managed with supportive care.

## Contribution

This paper presents a rare clinical case of PPFE with bilateral spontaneous pneumothoraces, highlighting diagnostic and management challenges.

## Key findings

- The patient exhibited bilateral spontaneous pneumothoraces on a background of pleural-based consolidation and fibrotic changes.
- High-resolution CT confirmed the diagnosis of pleuroparenchymal fibroelastosis.
- The patient was managed with supportive care due to the advanced stage of the disease.

## Abstract

Pleuroparenchymal fibroelastosis (PPFE) is a rare interstitial lung disease (ILD), characterized by predominantly upper lobe pleural and subjacent sub-pleural parenchymal fibrosis. Its name refers to a combination of fibrosis involving the visceral pleura with fibroelastotic changes, predominantly in the subpleural lung parenchyma.

We describe the case of a 67-year-old lady who presented to the accident and emergency department of Weston General Hospital with worsening shortness of breath (SOB) and cachexia of six to eight months' duration.

The initial imaging studies showed bilateral spontaneous pneumothoraces on a background of pleural-based consolidation and fibrotic changes. A subsequent high-resolution CT (HRCT) chest showed evidence of pleuroparenchymal fibroelastosis in the background. She was not considered for anti-fibrotic medications due to the advanced stage of the disease and was managed with supportive measures, including oxygen support, oral steroids and antibiotics to cover for any infections. After initial management of symptoms and long discussions with the patient, family and the palliative team, she was discharged home with community follow-up.