ArticleOrphanet journal of rare diseases2025
Prevalence and recurrence rates of spontaneous pneumothorax in patients with diffuse cystic lung diseases in China.
Article in Orphanet journal of rare diseases, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 3 papers.
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Who cites it
3 citing papers in PubMed.
- A Multicenter Registry for Rare Interstitial Lung Diseases in Korea: Baseline Characteristics and Clinical Outcomes.Tuberculosis and respiratory diseases · 2026Article
- Diffuse cystic lung diseases-a primer for radiologists.BJR open · 2026Review
- Multidisciplinary Management of Lymphangioleiomyomatosis Presenting With Severe Bilateral Pneumothorax During Pregnancy: A Case Report.Case reports in pulmonology · 2026Article
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8 authors.
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Abstract
objectivesTo investigate the prevalence and recurrence rates of spontaneous pneumothorax (SP) in patients with diffuse cystic lung diseases (DCLDs).
methodsWe 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.
resultsA 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.
conclusionThe 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.
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