# Case series: Interstitial lung disease with IPF pattern presenting with spontaneous pneumothorax—clinical course of two patients

## TL;DR

This case series reports two rare instances of fibrotic lung disease complicated by pneumothorax, subcutaneous emphysema, and pneumomediastinum, highlighting the need for prompt diagnosis and care.

## Contribution

The first reported case series documenting the rare triad of pneumothorax, subcutaneous emphysema, and pneumomediastinum in fibrotic ILD.

## Key findings

- A patient with IPF-pattern ILD presented with pneumothorax, pneumomediastinum, and subcutaneous emphysema and died from respiratory failure.
- Another patient with probable UIP-pattern ILD developed a spontaneous pneumothorax that was successfully managed conservatively.
- The triad of complications in fibrotic ILD is exceedingly rare and requires timely recognition and multidisciplinary care.

## Abstract

Interstitial lung disease (ILD) encompasses a broad spectrum of fibrosing pulmonary conditions. Pneumothorax is a recognized complication of fibrotic ILD, but simultaneous occurrence with subcutaneous emphysema and pneumomediastinum is exceedingly rare.

This case series describes two patients with idiopathic pulmonary fibrosis (IPF)-pattern ILD who experienced complications from spontaneous pneumothorax. In the first case, the patient arrived at the emergency department with worsening shortness of breath. Imaging revealed the presence of spontaneous pneumothorax, pneumomediastinum, and subcutaneous emphysema. The patient’s condition deteriorated rapidly and ultimately succumbed to respiratory failure. The second case involved a female patient who initially presented with chronic respiratory symptoms and was later diagnosed with probable usual interstitial pneumonia (UIP)-pattern ILD. During the follow-up, she exhibited worsening symptoms and was diagnosed with a spontaneous pneumothorax, which was treated successfully with conservative management.

To the best of our knowledge, this is the first reported case series that document the triad of subcutaneous emphysema, pneumomediastinum, and pneumothorax in a patient with fibrotic ILD. The findings underscore the importance of timely recognition and multidisciplinary management for such high-risk patients.