ArticleAmerican journal of respiratory and critical care medicine2015
Serum endostatin is a genetically determined predictor of survival in pulmonary arterial hypertension.
Article in American journal of respiratory and critical care medicine, 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 75 papers, 1 of them a synthesis that pooled it.
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The trial behind it
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Who cites it
75 citing papers in PubMed, 1 synthesis or guideline pooled it, 107 citations in OpenAlex.
- Assessment of Right Ventricular Function in the Research Setting: Knowledge Gaps and Pathways Forward. An Official American Thoracic Society Research Statement.American journal of respiratory and critical care medicine · 2018Guideline
- Angiogenesis in Lung Regeneration and Aging.Arteriosclerosis, thrombosis, and vascular biology · 2026Review
- Protein biomarkers in pulmonary arterial hypertension: advances, clinical relevance, and translational challenges.Journal of translational medicine · 2025Review
- Plasma endostatin and its association with new-onset acute kidney injury in critical care.Journal of intensive care · 2025Article
- Plasma endostatin at intensive care admission is independently associated with acute kidney injury, dialysis, and mortality in COVID-19.Intensive care medicine experimental · 2025Article
- Observational
- Equivalency of Multiple Biomarkers to Clinical Pulmonary Arterial Hypertension Survival Risk Models.Chest · 2024Article
- Circulating biomarkers in pulmonary arterial hypertension: State-of-the-art review and future directions.JHLT open · 2024Review
- Preacinar Arterial Dilation Mediates Outcomes of Quantitative Interstitial Abnormalities in the COPDGene Study.American journal of respiratory and critical care medicine · 2024Article
- Risk stratification and treatment goals in pulmonary arterial hypertension.The European respiratory journal · 2024Review
- Collagen 18A1/Endostatin Expression in the Progression of Right Ventricular Remodeling and Dysfunction in Pulmonary Arterial Hypertension.American journal of respiratory cell and molecular biology · 2024Article
- Recent developments in connective tissue disease associated pulmonary arterial hypertension.International journal of cardiology. Congenital heart disease · 2024Review
- Circulating Biomarkers in Pulmonary Arterial Hypertension: An Update.Biomolecules · 2024Review
- Pentastatin, a matrikine of the collagen IVα5, is a novel endogenous mediator of pulmonary endothelial dysfunction.American journal of physiology. Cell physiology · 2023Article
- Low-affinity insulin-like growth factor binding protein 7 and its association with pulmonary arterial hypertension severity and survival.Pulmonary circulation · 2023Article
- Risk Stratification in Pulmonary Arterial Hypertension, Update and Perspectives.Journal of clinical medicine · 2023Review
- Article
- Vascular pathobiology of pulmonary hypertension.The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation · 2023Review
- Insulin-like growth factor binding Protein-4: A novel indicator of pulmonary arterial hypertension severity and survival.Pulmonary circulation · 2023Article
- Serum Biomarkers in Connective Tissue Disease-Associated Pulmonary Arterial Hypertension.International journal of molecular sciences · 2023Review
15 more citing papers are in PubMed but not listed here.
Corrections and comments
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Authors and funding
21 authors at 6 institutions in 1 country.
Funding
Abstract
rationalePulmonary arterial hypertension (PAH) is a medically incurable disease resulting in death from right ventricular (RV) failure. Both pulmonary vascular and RV remodeling are linked to dynamic changes in the microvasculature. Therefore, we hypothesized that circulating angiostatic factors could be linked to outcomes and represent novel biomarkers of disease severity in PAH.
objectivesWe sought to determine the relationship of a potent angiostatic factor, endostatin (ES), with disease severity and mortality in PAH. Furthermore, we assessed genetic predictors of ES expression and/or function and their association with outcomes in PAH.
methodsWe measured levels of serum ES in two independent cohorts of patients with PAH. Contemporaneous clinical data included New York Heart Association functional class, 6-minute-walk distance, invasive hemodynamics, and laboratory chemistries. MEASUREMENTS AND MAIN
resultsSerum ES correlated with poor functional status, decreased exercise tolerance, and invasive hemodynamics variables. Furthermore, serum ES was a strong predictor of mortality. A loss-of-function, missense variant in the gene encoding ES, Col18a1, was linked to lower circulating protein and was independently associated with reduced mortality.
conclusionsOur data link increased expression of ES to disease severity in PAH and demonstrate a significant relationship with adverse outcomes. Circulating ES levels can be genetically influenced, implicating ES as a genetically determined modifier of disease severity impacting on survival. These observations support serum ES as a potential biomarker in PAH with the capacity to predict poor outcomes. More importantly, this study implicates Col18a1/ES as a potential new therapeutic target in PAH.
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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.