ArticleOrphanet journal of rare diseases2018
Efficacy of sirolimus for the prevention of recurrent pneumothorax in patients with lymphangioleiomyomatosis: a case series.
Article in Orphanet journal of rare diseases, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 14 papers, 1 of them a synthesis that pooled it.
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Who cites it
14 citing papers in PubMed, 1 synthesis or guideline pooled it, 37 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
- Emerging Concepts in Pathogenesis, Multiomics Applications, and Clinical Research in Lymphangioleiomyomatosis.Seminars in respiratory and critical care medicine · 2025Review
- Prevalence and recurrence rates of spontaneous pneumothorax in patients with diffuse cystic lung diseases in China.Orphanet journal of rare diseases · 2025Article
- Intraoperative mechanical ventilation and incidence of pneumothorax in lymphangioleiomyomatosis.Orphanet journal of rare diseases · 2024Article
- The benefit of indwelling pleural catheter with ambulatory pneumothorax device and autologous blood patch pleurodesis in lymphangioleiomyomatosis with persistent air leak.Respirology case reports · 2023Article
- Impact of Age, Menopause, and Sirolimus on Spontaneous Pneumothoraces in Lymphangioleiomyomatosis.Chest · 2022Article
- Sirolimus reduces the risk of pneumothorax recurrence in patients with lymphangioleiomyomatosis: a historical prospective self-controlled study.Orphanet journal of rare diseases · 2022Article
- Reduced risk of recurrent pneumothorax for sirolimus therapy after surgical pleural covering of entire lung in lymphangioleiomyomatosis.Orphanet journal of rare diseases · 2021Article
- Efficacy, Retention and Tolerability of Everolimus in Patients with Tuberous Sclerosis Complex: A Survey-Based Study on Patients' Perspectives.CNS drugs · 2021Article
- Tuberous sclerosis complex for the pulmonologist.European respiratory review : an official journal of the European Respiratory Society · 2021Article
- RDmap: a map for exploring rare diseases.Orphanet journal of rare diseases · 2021Article
- The effects of total pleural covering on pneumothorax recurrence and pulmonary function in lymphangioleiomyomatosis patients without history of pleurodesis or thoracic surgeries for pneumothorax.Journal of thoracic disease · 2021Article
- Total pleural coverage followed by lung transplantation in patient with lymphangioleiomyomatosis.General thoracic and cardiovascular surgery · 2020Article
- Lymphangioleiomyomatosis: A Case Report and Review of Clinical Features and Management.Cureus · 2020Article
Corrections and comments
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Authors and funding
8 authors at 1 institution in 1 country.
Funding
Abstract
Pneumothorax is one of the most common symptoms in patients with lymphangioleiomyomatosis (LAM). However, current management strategies for patients with LAM who present with recurrent pneumothorax remain inadequate. Here, we describe the successful prevention of recurrent pneumothorax by sirolimus treatment in five women with LAM. Before sirolimus treatment, all patients had received supplemental oxygen support, repeated chest tube drainage, or surgeries for management of the recurrent pneumothorax. Sirolimus treatment was initiated when the pneumothorax was completely resolved, and no patient developed pneumothorax during treatment. Moreover, they exhibited a significantly improved subjective quality of life, increased exercise capacity, and mild adverse effects such as mucositis, irregular menstruation, and delayed wound healing. On discontinuation of sirolimus or in the event that the plasma sirolimus level was markedly low, pneumothorax tended to relapse. The findings from these cases provide valuable insights that will aid in the improvement of treatment strategies for patients with LAM and recurrent pneumothorax.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.