ArticleThorax2004
Survival and disease progression in UK patients with lymphangioleiomyomatosis.
Article in Thorax, 2004. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 46 papers, 4 of them syntheses that pooled it.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
46 citing papers in PubMed, 4 syntheses or guidelines pooled it.
- Brazilian Thoracic Association recommendations for the management of lymphangioleiomyomatosis.Jornal brasileiro de pneumologia : publicacao oficial da Sociedade Brasileira de Pneumologia e Tisilogia · 2025Guideline
- A Systematic Review of Lymphangioleiomyomatosis on Diagnosis and Molecular Mechanism.BioMed research international · 2021Pooled it
- The efficacy and safety of pharmacological treatments for lymphangioleiomyomatosis.Respiratory research · 2020Pooled it
- Official American Thoracic Society/Japanese Respiratory Society Clinical Practice Guidelines: Lymphangioleiomyomatosis Diagnosis and Management.American journal of respiratory and critical care medicine · 2016Guideline
- Efficacy and safety of sirolimus in lymphangioleiomyomatosis.The New England journal of medicine · 2011Trial
- Article
- Small airways morphological alterations associated with functional impairment in lymphangioleiomyomatosis.BMC pulmonary medicine · 2024Article
- An Evidence-Based Update on the Potential Association between Rheumatoid Arthritis and Lymphangioleiomyomatosis.Journal of personalized medicine · 2023Review
- A case report of lymphangioleiomyomatosis with retroperitoneal masses in pregnancy.Frontiers in medicine · 2023Article
- A pulmonary lymphangioleiomyomatosis with multi-site angiomyolipoma: a case report.Translational cancer research · 2022Article
- Long-term clinical course and outcomes in patients with lymphangioleiomyomatosis.Respiratory research · 2022Article
- Long-term clinical course and progression of lymphangioleiomyomatosis in a single lung transplant referral centre in Korea.Scientific reports · 2022Article
- Multidisciplinary team approach to diagnosing lymphangioleiomyomatosis.BMJ case reports · 2021Article
- Lymphangioleiomyomatosis associated with prolactinoma: A case report and literature review.Respiratory medicine case reports · 2021Article
- Pulmonary lymphangioleiomyomatosis masquerading as unilateral heterogeneous emphysema.Journal of surgical case reports · 2020Article
- Ultra-rare cystic disease.European respiratory review : an official journal of the European Respiratory Society · 2020Article
- Pulmonary Lymphangioleiomyomatosis: A Case Report and Literature Review.Hawai'i journal of health & social welfare · 2020Review
- Lymphangioleiomyomatosis: a clinical review.Breathe (Sheffield, England) · 2020Review
- Effects of sirolimus in lymphangioleiomyomatosis patients on lung cysts and pulmonary function: long-term follow-up observational study.European radiology · 2020Observational
- The vitamin D binding protein axis modifies disease severity in lymphangioleiomyomatosis.The European respiratory journal · 2018Article
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
5 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundLymphangioleiomyomatosis (LAM) is a rare and progressive disease of young women with no effective treatment. Previous estimates of 10 year survival, based mostly on case series or patients from tertiary centres, have ranged from 40% to 79%; no data are available on the progression of respiratory disability. In order to provide data for patients and for planning intervention studies, we have looked at the time course of LAM using a national cohort.
methodsTime to death, time to MRC dyspnoea grades 2-5, and need for oxygen in patients on the UK LAM database were analysed using Kaplan-Meier analysis and Cox regression.
resultsFifty seven of 72 patients responded with a median duration of follow up of 12.6 years (range 2.3-37) from the onset of symptoms. Ten year survival was 91% from onset of symptoms but varied widely with 11 patients alive after 20 years. Median time to MRC grade 3 dyspnoea (breathless walking on the flat) was 9.3 years (95% CI 5.1 to 13.4) from onset of symptoms.
conclusionsSurvival from LAM appears to be better than that reported in early studies. These data should be helpful for patients and for planning clinical trials.
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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.