ArticleJornal brasileiro de pneumologia : publicacao oficial da Sociedade Brasileira de Pneumologia e Tisilogia
Use of sirolimus in the treatment of lymphangioleiomyomatosis: favorable responses in patients with different extrapulmonary manifestations.
Article in Jornal brasileiro de pneumologia : publicacao oficial da Sociedade Brasileira de Pneumologia e Tisilogia. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 18 papers, 1 of them a synthesis that pooled it.
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Who cites it
18 citing papers in PubMed, 1 synthesis or guideline pooled it, 29 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
- A case description: lymphangioleiomyomatosis mimicking ovarian cyst.Quantitative imaging in medicine and surgery · 2026Article
- Delayed TSC Diagnosis Presenting as End-Stage Renal Disease With Renal and Hepatic Angiomyolipoma: Case Report and Review.Clinical case reports · 2025Article
- Clinical, tomographic and functional comparison of sporadic and tuberous sclerosis complex-associated forms of lymphangioleiomyomatosis: a retrospective cohort study.ERJ open research · 2024Article
- Review
- A Case of Uterine Lymphangioleiomyomatosis Complicated by Tuberous Sclerosis Complex.Case reports in obstetrics and gynecology · 2022Article
- Sporadic uterine Lymphangioleiomyomatosis (LAM): Report of a unique case arising in the lower uterine segment with short review.Gynecologic oncology reports · 2021Article
- Clinical characteristics in lymphangioleiomyomatosis-related pulmonary hypertension: an observation on 50 patients.Frontiers of medicine · 2019Observational
- Effects of Sirolimus on Lung function in patients with Lymphangioleiomyomatosis.Caspian journal of internal medicine · 2019Article
- Functional improvements in patients with lymphangioleiomyomatosis after sirolimus: an observational study.Orphanet journal of rare diseases · 2018Observational
- Lymphangioleiomyomatosis: a case report and review of diagnosis and treatment.OncoTargets and therapy · 2018Article
- Article
- Pelvic lymphangioleiomyomatosis treated successfully with everolimus: Two case reports with literature review.Medicine · 2017Review
- Is mTOR Inhibitor Good Enough for Treatment All Tumors in TSC Patients?Journal of Cancer · 2016Review
- JBP and bibliometric índices.Jornal brasileiro de pneumologia : publicacao oficial da Sociedade Brasileira de Pneumologia e TisilogiaArticle
- The need for a balance between highly prevalent diseases and neglected diseases.Jornal brasileiro de pneumologia : publicacao oficial da Sociedade Brasileira de Pneumologia e TisilogiaArticle
- Low-dose sirolimus in retroperitoneal lymphangioleiomyomas.Lung India : official organ of Indian Chest SocietyArticle
- Treatment of lymphangioleiomyomatosis and Camões.Jornal brasileiro de pneumologia : publicacao oficial da Sociedade Brasileira de Pneumologia e TisilogiaArticle
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Authors and funding
6 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
objectiveLymphangioleiomyomatosis (LAM) is a rare disease that is currently considered a low-grade neoplasm with metastatic potential and variable progression. Mammalian target of rapamycin (mTOR) inhibitors, such as sirolimus and everolimus, have recently become a treatment option for LAM patients, especially those with extrapulmonary manifestations. The objective of the present study was to describe a case series of four patients with LAM in Brazil who showed significant improvement, particularly in their extrapulmonary manifestations, after treatment with sirolimus (at 1-4 mg/day).
methodsWe describe four cases of LAM patients with different extrapulmonary manifestations who were treated with sirolimus.
resultsAfter treatment with sirolimus for 12 months, one patient presented resolution of severe chylothorax; one had a significant reduction in renal angiomyolipoma volume; and one showed significant regression of retroperitoneal lymphangioleiomyomas and abdominal lymph node enlargement. After treatment with sirolimus for 6 months, the remaining patient had a significant reduction in the volume of a massive retroperitoneal lymphangioleiomyoma.
conclusionsOur findings confirm that mTOR inhibitors are beneficial for patients with LAM, especially those with extrapulmonary manifestations, such as renal angiomyolipoma, lymphangioleiomyomas, and chylous effusions. However, certain aspects, such as the optimal dose, duration of treatment, and long-term adverse effects, have yet to be sufficiently clarified for mTOR inhibitors to be incorporated into LAM management protocols.
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