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Coverage: 2020-01-01 onwards, with up to 300 results per query from PubMed and Europe PMC and separate limits on other sources. This is a selected index, not a complete literature search.
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Lymphangioleiomyomatosis: Research Summary
Printed from RareWays (rareways.org)
For general awareness only. Not medical advice. Discuss all care options with your healthcare team.
5 Most Recent Research Articles
- 1.
T cell hyporesponsiveness to TSC2null cells suggests bidirectional interactions in LAM.
Xue Chang et al., American journal of respiratory cell and molecular biology (8 October 2026)
https://pubmed.ncbi.nlm.nih.gov/42848521/
- 2.
Clinical Characteristics and Outcomes of Patients with Lymphangioleiomyomatosis.
Yoon Hee-Young et al., Lung (5 October 2026)
https://pubmed.ncbi.nlm.nih.gov/42832099/
- 3.
GPNMB immunohistochemistry distinguishes diagnostically challenging cases of lymphangioleiomyomatosis from other mesenchymal neoplasms presenting with cystic lung metastases.
Ji Selina F et al., Human pathology (1 October 2026)
https://pubmed.ncbi.nlm.nih.gov/42269880/
- 4.
A Multicenter Registry for Rare Interstitial Lung Diseases in Korea: Baseline Characteristics and Clinical Outcomes.
Oh Ju Hyun et al., Tuberculosis and respiratory diseases (1 October 2026)
https://pubmed.ncbi.nlm.nih.gov/42338237/
- 5.
T cell exhaustion in the peripheral blood of patients with sporadic lymphangioleiomyomatosis
Siyi Zhou et al., Respiratory Research (21 August 2026)
https://doi.org/10.1186/s12931-026-03873-2
Source: RareWays research directory. Data from PubMed, Europe PMC, OpenAlex, ClinicalTrials.gov.
Always verify information with your healthcare team before making any decisions about your care.
Lymphangioleiomyomatosis
Lymphangioleiomyomatosis (LAM) is a rare progressive lung disease that almost exclusively affects women, causing cystic lung destruction, breathlessness, and recurrent pneumothorax. It is caused by mutations in TSC genes. The mTOR inhibitor sirolimus slows progression and is the only approved treatment.
This overview is general information, not an individual medical assessment. Research is selected automatically and may include mismatches. How we select and explain research.
Most Recent Research
RATIONALE: Lymphangioleiomyomatosis (LAM) is a rare disease characterized by the proliferation of neoplastic smooth muscle-like cells in women. The role of the immune system in LAM progression is unclear. OBJECTIVES: Investigate the influence of T cells on TSC2null smooth muscle cell behaviour at cellular and transcriptome levels. METHODS: Archival LAM and control lung tissues were compared by gene profiling with NanoString analyses where elevated CD8 T cell accumulation was observed in LAM vs. control tissues. Acknowledging the limited translational potential of animal models, a human T cell-TSC2null smooth muscle cell co-culture system was established and cell phenotype and proliferation over time using flow cytometry was examined. MAIN RESULTS: TSC2null smooth muscle cells adopt cancer-associated fibroblast (CAF)-like phenotypes, which could be regulated by IFN-γ. TSC2null cells suppressed T cell proliferation and induced T cell anergic phenotypes, which could not be reversed by antigen receptor and co-stimulation. TSC2null cells decreased expression of co-stimulatory molecule, CD40, and increased proliferation in the presence of T cells, yet underwent apoptosis in the presence of activated T cells. CONCLUSIONS: Our findings suggest a contact-dependent feedback loop between TSC2null smooth muscle cells and T cells, which may both promote immune dysregulation in LAM and explain the invasive behavior and tissue remodeling observed clinically.
Common Questions
What is Lymphangioleiomyomatosis?
Lymphangioleiomyomatosis (LAM) is a rare progressive lung disease that almost exclusively affects women, causing cystic lung destruction, breathlessness, and recurrent pneumothorax. It is caused by mutations in TSC genes. The mTOR inhibitor sirolimus slows progression and is the only approved treatment.
How many clinical trials are available for Lymphangioleiomyomatosis?
RareWays currently indexes 29 clinical trials for Lymphangioleiomyomatosis, of which 2 are actively recruiting. These counts cover indexed trials across countries, not just your country. An Australian site does not necessarily mean that site is recruiting. Check locations, eligibility and current availability with the study team.
Where does the research data for Lymphangioleiomyomatosis come from?
RareWays aggregates research from PubMed, Europe PMC, OpenAlex, and ClinicalTrials.gov. Data is refreshed automatically every day. All articles and trials link directly to their original sources.
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