ArticleOrphanet journal of rare diseases2022
Sirolimus reduces the risk of pneumothorax recurrence in patients with lymphangioleiomyomatosis: a historical prospective self-controlled study.
Article in Orphanet journal of rare diseases, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 12 papers, 2 of them syntheses that pooled it.
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Who cites it
12 citing papers in PubMed, 2 syntheses or guidelines pooled it, 27 citations in OpenAlex.
- Brazilian Thoracic Association recommendations for the management of lymphangioleiomyomatosis.Jornal brasileiro de pneumologia : publicacao oficial da Sociedade Brasileira de Pneumologia e Tisilogia · 2025Guideline
- Probability of sporadic lymphangioleiomyomatosis in women presenting with spontaneous pneumothorax.Orphanet journal of rare diseases · 2023Pooled it
- Six-minute walk distance predicting the risk of mortality in lymphangioleiomyomatosis patients.Orphanet journal of rare diseases · 2026Article
- Multidisciplinary Management of Lymphangioleiomyomatosis Presenting With Severe Bilateral Pneumothorax During Pregnancy: A Case Report.Case reports in pulmonology · 2026Article
- Surgical strategies for spontaneous pneumothorax: a narrative review.Journal of visualized surgery · 2026Review
- Iron metabolism disorder and artesunate inhibiting tumor growth by inducing ferroptosis in Lymphangioleiomyomatosis.Respiratory research · 2025Article
- Illness cognition, illness perception and related factors in patients with lymphangioleiomyomatosis.Orphanet journal of rare diseases · 2025Article
- Prevalence and recurrence rates of spontaneous pneumothorax in patients with diffuse cystic lung diseases in China.Orphanet journal of rare diseases · 2025Article
- Lymphangioleiomyomatosis and Pregnancy-Do We Have All the Answers for a Woman Who Desires to Conceive?-Literature Review.Cancers · 2025Review
- Neutrophil and neutrophil-lymphocyte ratios as predictors of clinical characteristics in patients with Lymphangioleiomyomatosis.Frontiers in medicine · 2025Article
- Intraoperative mechanical ventilation and incidence of pneumothorax in lymphangioleiomyomatosis.Orphanet journal of rare diseases · 2024Article
- Spontaneous pneumothorax-associated with genetic disorders.Therapeutic advances in respiratory diseaseReview
Corrections and comments
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Authors and funding
10 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundSpontaneous pneumothorax has a high incidence and high rate of recurrence in patients with lymphangioleiomyomatosis (LAM). The risk factors for pneumothorax and the effects of sirolimus on pneumothorax in patients with LAM are unknown. In our study, multivariate logistic regression was applied to a cross-sectional cohort to investigate factors associated with pneumothorax in LAM patients. Kaplan-Meier analysis was applied in the historical prospective self-controlled study to determine whether sirolimus reduces the risk of pneumothorax recurrence in patients with LAM.
resultsOf the 399 patients registered with LAM-CHINA at our center between May 10, 2017 and August 31, 2020, 142 had a history of pneumothorax at registration. High CT grade and age at presentation ≤ 35 years were associated with a higher risk of pneumothorax in patients with LAM. Postmenopausal status was correlated with a lower risk of pneumothorax. In the historical prospective self-controlled study, the 5-year probability of pneumothorax recurrence was 80% lower in the sirolimus group than in the control group (hazard ratio for pneumothorax recurrence, 0.20; 95% CI, 0.14 to 0.30, P < 0.001 by log-rank test).
conclusionSirolimus reduced the risk of pneumothorax recurrence in LAM patients.
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