ArticleOrphanet journal of rare diseases2025
Associations of VEGF-D levels with clinical manifestations in lymphangioleiomyomatosis: a cross-sectional analysis of 631 cases.
Article in Orphanet journal of rare diseases, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 5 papers.
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Who cites it
5 citing papers in PubMed.
- Current insight into the molecular mechanisms of lymphangioleiomyomatosis.American journal of respiratory cell and molecular biology · 2026Review
- Article
- A Case Series of Lymphangioleiomyomatosis, a Rare Cystic Lung Disease.Respirology case reports · 2026Article
- CCL14, identified by multi-omics approach, serves as a novel indicator of disease severity and progression in lymphangioleiomyomatosis.Orphanet journal of rare diseases · 2026Article
- Lymphangioleiomyomatosis with superimposed organizing pneumonia diagnosed by transbronchial lung cryobiopsy: A case report.Respiratory medicine case reports · 2026Article
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17 authors.
Funding
Abstract
backgroundLymphangioleiomyomatosis (LAM) is a rare neoplastic disorder characterized predominantly by dyspnea, recurrent pneumothorax, chylous effusion and diffuse pulmonary cystic changes in women. Vascular endothelial growth factor-D (VEGF-D) is an important biomarker for LAM.
resultsThis study cohort comprised 631 LAM patients and investigated the correlations between serum VEGF-D levels and clinical manifestations of LAM. The median serum level of VEGF-D was 1452pg/ml (820.0-2659pg/ml) among the study population. Patients with highly-elevated VEGF-D levels exhibited younger age, lower BMI, and a higher prevalence of tuberous sclerosis complex (TSC). Elevated VEGF-D levels were associated with a lower prevalence of pneumothorax and angiomyolipomas (AMLs), and a higher risk for retroperitoneal lymphangioleiomyomas and chylous effusion. Elevated VEGF-D levels were associated with increased High-Resolution Computed Tomography (HRCT) LAM grading, reduced forced expiratory volume in one second (FEV
conclusionsThe findings highlighted the pivotal role of serum VEGF-D in LAM pathophysiology and underscore that age, TSC, retroperitoneal LAM, chylous effusion, AMLs, and high HRCT grade were independent risk factors for increased VEGF-D levels. VEGF-D is a valuable biomarker in evaluation of LAM and improve the efficiency of diagnostic algorithm.
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