# Prevalence and recurrence rates of spontaneous pneumothorax in patients with diffuse cystic lung diseases in China

## TL;DR

This study examines how often spontaneous pneumothorax occurs in patients with different types of cystic lung diseases in China and finds that surgical treatment reduces recurrence.

## Contribution

The study provides new insights into the recurrence rates of spontaneous pneumothorax in specific diffuse cystic lung diseases and the effectiveness of surgical intervention.

## Key findings

- Patients with Birt-Hogg-Dubé syndrome had the highest rate of spontaneous pneumothorax at 59%.
- Surgical treatment significantly reduced recurrence rates compared to conservative management in BHD and LAM patients.
- Sirolimus therapy may help prevent recurrence of pneumothorax in lymphangioleiomyomatosis patients.

## Abstract

To investigate the prevalence and recurrence rates of spontaneous pneumothorax (SP) in patients with diffuse cystic lung diseases (DCLDs).

We retrospectively identified and analyzed medical records of patients with DCLDs encountered at the First Affiliated Hospital of University of Science and Technology of China from Jan 1, 2017 to December 31, 2023.

A total of 289 patients were identified with DCLDs; 212 females and 77 males, with a median age of 48 years (range, 18–81 years). Among them, 89 (31%) patients had experienced SP; 59% among 115 patients with Birt-Hogg-Dubé (BHD), 34% of 41 patients with lymphangioleiomyomatosis (LAM, all women), 36% of 11 patients with pulmonary Langerhans cell histiocytosis (PLCH), none of 57 patients with Sjögren’s syndrome-associated diffuse cystic lung disease (SS-DCLD), and 5% of 65 patients with no identifiable underlying disease (χ² = 90.585, P < 0.001). The overall recurrence rate of SP was higher with observation or chest tube placement strategy compared to surgical intervention, 59% vs. 11% (P < 0.001, 95% CI [0.1, -0.4]), respectively. The recurrence rate after surgical management was significantly lower compared to conservative management in patients with BHD (10% vs. 69%, P < 0.001, 95% CI [0.1, 0.3]) and LAM (20% vs. 57%, P = 0.322, 95% CI [0.1, 2.1]). Among patients with BHD, LAM, and PLCH, those who had pneumothorax as the initial presentation were diagnosed of their underlying disease at a significantly younger age (42.2 ± 13.0 years) compared to those without pneumothorax (48.1 ± 11.8 years) (P = 0.032, 95% CI [-8.24, -0.36]). Notably, eight of LAM patients who were treated with sirolimus after the initial SP did not experience recurrence of SP.

The risk of SP secondary to DCLDs was highest in patients with BHD, followed by those with PLCH and LAM. It was extremely low in SS-DCLD. Pneumothorax as the initial presentation often facilitated diagnosis of the underlying disease. Surgical treatment was associated with a lower recurrence rate of SP compared to nonsurgical management. In addition, sirolimus therapy may reduce the risk of pneumothorax recurrence in patients with LAM.

The online version contains supplementary material available at 10.1186/s13023-025-03587-6.