ArticleLymphatic research and biology2010
The natural history of lymphangioleiomyomatosis: markers of severity, rate of progression and prognosis.
Article in Lymphatic research and biology, 2010. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 50 papers.
What it found
Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.
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
50 citing papers in PubMed, 101 citations in OpenAlex.
- Article
- Exploring glucocorticoid receptor signalling in lymphangioleiomyomatosis.ERJ open research · 2026Article
- Neutrophil and neutrophil-lymphocyte ratios as predictors of clinical characteristics in patients with Lymphangioleiomyomatosis.Frontiers in medicine · 2025Article
- Ultra-low dose chest CT with silver filter and deep learning reconstruction significantly reduces radiation dose and retains quantitative information in the investigation and monitoring of lymphangioleiomyomatosis (LAM).European radiology · 2024Article
- Surgical Management of Solitary Extrapulmonary Lymphangioleiomyomatosis in the Mesentery: A Case Report.Cureus · 2024Article
- Lymphangioleiomyomatosis and pregnancy: a mini-review.Archives of gynecology and obstetrics · 2024Review
- Clinical, tomographic and functional comparison of sporadic and tuberous sclerosis complex-associated forms of lymphangioleiomyomatosis: a retrospective cohort study.ERJ open research · 2024Article
- Small airways morphological alterations associated with functional impairment in lymphangioleiomyomatosis.BMC pulmonary medicine · 2024Article
- Lymphangioleiomyomatosis: where endocrinology, immunology and tumor biology meet.Endocrine-related cancer · 2023Review
- Lymphangioleiomyomatosis: a metastatic lung disease.American journal of physiology. Cell physiology · 2023Review
- Article
- A pulmonary lymphangioleiomyomatosis with multi-site angiomyolipoma: a case report.Translational cancer research · 2022Article
- Long-term clinical course and progression of lymphangioleiomyomatosis in a single lung transplant referral centre in Korea.Scientific reports · 2022Article
- Clinical-Pathological Conference Series from the Medical University of Graz : Case No 170: A 33-year-old psychologist with severe dyspnea and right-sided chylothorax.Wiener klinische Wochenschrift · 2021Article
- Pulmonary benign metastasizing leiomyoma: A case report and review of the literature.World journal of clinical cases · 2020Article
- Analysis of the MILES cohort reveals determinants of disease progression and treatment response in lymphangioleiomyomatosis.The European respiratory journal · 2019Article
- Angiotensin-converting enzyme inhibitors may affect pulmonary function in lymphangioleiomyomatosis.JCI insight · 2019Article
- Article
- Comorbid connective tissue diseases and autoantibodies in lymphangioleiomyomatosis: a retrospective cohort study.Orphanet journal of rare diseases · 2018Article
- Pulmonary manifestations in tuberous sclerosis complex.American journal of medical genetics. Part C, Seminars in medical genetics · 2018Review
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
3 authors at 2 institutions in 1 country.
Funding
Abstract
Lymphangioleiomyomatosis (LAM) is a multisystem disease of women, characterized by proliferation of abnormal smooth muscle-like cells (LAM cells) that can metastasize, leading to the formation of lung cysts, fluid-filled cystic structures in the axial lymphatics (e.g., lymphangioleiomyomas), and angiomyolipomas, benign tumors usually involving the kidneys, comprising LAM cells and adipocytes, intermixed with incompletely developed vascular structures. LAM occurs sporadically or in association with tuberous sclerosis complex, an autosomal dominant syndrome characterized by hamartoma-like tumor growths. LAM may present with progressive dyspnea, recurrent pneumothorax, chylothorax, or abdominal hemorrhage. Computed tomography scans show thin-walled cysts scattered throughout the lungs, abdominal angiomyolipomas, and lymphangioleiomyomas. Pulmonary function tests show reduced flow rates (FEV(1)) and diffusion capacity (DL(CO)). Exercise testing may reveal gas exchange abnormalities, ventilatory limitation, and hypoxemia, which can occur with near-normal lung function. Methods used to grade the severity of disease are the LAM histology score, semiquantitative and quantitative computer tomography, pulmonary function testing, and cardiopulmonary exercise testing. Currently, progression of disease is best assessed by serial measurements of FEV(1), DL(CO), and exercise performance. New quantitative radiographic techniques that may offer advantages over physiologic testing are now available. Several potential biomarkers, such as LAM cells in peripheral blood, urine, and chyle and chemokines, vascular endothelial growth factors, and matrix metalloproteinases, may be useful as diagnostic tools or markers of organ involvement, disease severity, and progression.
Indexed as
Identifiers
What OpenQuestion holds
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.