ArticleLung2014
Evaluation of the extent of pulmonary cysts and their association with functional variables and serum markers in lymphangioleiomyomatosis (LAM).
Article in Lung, 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 13 papers, 1 of them a synthesis that pooled it.
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Who cites it
13 citing papers in PubMed, 1 synthesis or guideline pooled it, 23 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
- Six-minute walk distance predicting the risk of mortality in lymphangioleiomyomatosis patients.Orphanet journal of rare diseases · 2026Article
- The development for emerging biomarkers of lymphangioleiomyomatosis.Orphanet journal of rare diseases · 2024Review
- Desaturation-Distance Ratio During Submaximal and Maximal Exercise Tests and Its Association With Lung Function Parameters in Patients With Lymphangioleiomyomatosis.Frontiers in medicine · 2021Article
- Exploring the Role of Matrix Metalloproteinases as Biomarkers in Sporadic Lymphangioleiomyomatosis and Tuberous Sclerosis Complex. A Pilot Study.Frontiers in medicine · 2021Article
- Quantitative assessment of pulmonary function in lymphangioleiomyomatosis patients using high-resolution computed tomography and pulmonary function tests.Journal of thoracic disease · 2020Article
- Quantitative analysis of airway obstruction in lymphangioleiomyomatosis.The European respiratory journal · 2020Article
- Lung function in Birt-Hogg-Dubé syndrome: a retrospective analysis of 96 patients.Orphanet journal of rare diseases · 2020Article
- Article
- Immunohistological features related to functional impairment in lymphangioleiomyomatosis.Respiratory research · 2018Article
- Oxygen desaturation during a 6-minute walk test as a predictor of maximal exercise-induced gas exchange abnormalities in sarcoidosis.Journal of thoracic disease · 2016Article
- Review
- CT grading of lung disease in lymphangioleiomyomatosis.AJR. American journal of roentgenology · 2012Article
Corrections and comments
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Authors and funding
9 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
purposeAlthough computed tomography (CT) has been used previously to assess disease severity in lymphangioleiomyomatosis (LAM), the associations between the extent of pulmonary cysts on CT and six-minute walk test (6MWT), matrix metalloproteinases (MMPs) and vascular endothelial growth factor (VEGF-D) are not well established. We performed a cross-sectional study to quantify the extent of pulmonary cysts in CT and to establish their correlations with pulmonary function tests (PFTs), 6MWT results, including a composite index (desaturation-distance ratio, DDR), and levels of VEGF-D and MMPs in LAM.
methodsTwenty-three LAM patients underwent CT scanning to automatically quantify the extent of pulmonary cysts and performed PFTs and 6MWT. Serum levels of MMP-2, MMP-9, and VEGF-D were also measured.
resultsThe severity of pulmonary cystic involvement was mild (the extent of cysts was 6.8 %) and correlated best with FEV1/FVC (r = -0.84), residual volume (r = 0.66), DLCO (r = -0.82), the DDR index (r = 0.77), and desaturation during the 6MWT (r = -0.81). There was a weak correlation with VEGF-D (r = 0.45), but no association was found with MMP-2 and MMP-9.
conclusionsThe severity of pulmonary cystic involvement was mild in this sample of LAM patients and correlated best with airway obstruction, air trapping, reduced DLCO, the DDR index, and desaturation during the 6MWT. Serum VEGF-D cannot be completely defined as a valuable marker of disease severity and there may be a mechanism independent of MMPs to explain the formation of pulmonary cysts.
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