Expression of Plateled-Derived Growth Factor Receptor β (PDGFR-β) and Epidermal GFR (EGFR) in Pulmonary Vasculature of Systemic Sclerosis-Associated Pulmonary Arterial Hypertension (SScPH)vs.Idiopathic PH (IPH) and Pulmonary Veno-Occlusive Disease (PVOD).

2009 ◽  
Author(s):  
MJ Overbeek ◽  
A Boonstra ◽  
E Smit ◽  
A Voskuyl ◽  
A Vonk-Noordegraaf ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Growth Factor ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Growth Factor Receptor ◽  
Occlusive Disease ◽  
Pulmonary Vasculature ◽  
Pulmonary Arterial

scholarly journals Circulating nerve growth factor receptor positive cells are associated with severity and prognosis of pulmonary arterial hypertension

2021 ◽  
Vol 11 (1) ◽  
pp. 204589402199052
Author(s):  
Chiaki Goten ◽  
Soichiro Usui ◽  
Shin-ichiro Takashima ◽  
Oto Inoue ◽  
Hirofumi Okada ◽  
...  
Keyword(s):  
Nerve Growth Factor ◽  
Growth Factor ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Growth Factor Receptor ◽  
Nerve Growth Factor Receptor ◽  
Clinical Severity ◽  
Hemodynamic Parameters ◽  
Nerve Growth ◽  
Pulmonary Arterial

Pulmonary arterial hypertension (PAH) remains a disease with a poor prognosis, so early detection and treatment are very important. Sensitive and non-invasive markers for PAH are urgently required. This study was performed to identify sensitive markers of the clinical severity and prognosis of PAH. Patients diagnosed with PAH (n = 30) and control participants (n = 15) were enrolled in this observational study. Major EPC and MSC markers (including CD34, CD133, VEGFR2, CD90, PDGFRα, and NGFR) in peripheral blood mononuclear cells (PBMNCs) were assessed by flow cytometry. Associations of these markers with hemodynamic parameters (e.g. mean pulmonary arterial pressure, pulmonary vascular resistance, and cardiac index) were assessed. Patients with PAH were followed up for 12 months to assess the incidence of major adverse events, defined as death or lung transplantation. Levels of circulating EPC and MSC markers in PBMNCs were higher in patients with PAH than in control participants. Among the studied markers, nerve growth factor receptor (NGFR) was significantly positively correlated with hemodynamic parameters. During the 12-month follow-up period, major-event-free survival was significantly higher in patients with PAH who had relatively low frequencies of NGFR positive cells than patients who had higher frequencies. These results suggested that the presence of circulating NGFR positive cells among PBMNCs may be a novel biomarker for the severity and prognosis of PAH.

Differential expression of hepatocyte growth factor in patients with systemic sclerosis-associated pulmonary arterial hypertension

2017 ◽  
Vol 2 (3) ◽  
pp. 225-230
Author(s):  
Yon K. Sung ◽  
Roham T. Zamanian ◽  
Catriona A. Wagner ◽  
William Robinson ◽  
Virginia Steen ◽  
...  
Keyword(s):  
At Risk ◽  
Systemic Sclerosis ◽  
Growth Factor ◽  
Pulmonary Arterial Hypertension ◽  
High Risk ◽  
Hepatocyte Growth Factor ◽  
Arterial Hypertension ◽  
Risk Patients ◽  
Pulmonary Arterial ◽  
Hepatocyte Growth

Introduction Non-invasive biomarkers are needed to identify pulmonary arterial hypertension (PAH) in systemic sclerosis (SSc) patients who may benefit from early intervention. We sought to identify novel cytokines that differentiate patients with incident SSc-PAH from those at high risk for PAH. Methods The Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma (PHAROS) Registry is a multicenter registry of SSc patients at high risk for PAH (at-risk) or with incident right-heart catheterization-confirmed PAH (definite PAH). Serum from 10 at-risk and 9 definite PAH patients were profiled with Bio-PlexTM bead arrays for 48 cytokines and chemokines. We also evaluated the longitudinal change in cytokine profiles from 3 at-risk patients who subsequently developed definite PAH. Results Clinical features of at-risk versus definite PAH patients were not significantly different except for right-ventricular systolic pressure on echocardiogram (34 ± 7 vs. 45 ± 8 mmHg, p = 0.006), left atrial diameter (2.9 ± 0.5 vs. 3.7 ± 0.4 cm, p = 0.02), 6-minute walk distance (508 ± 115 vs. 393 ± 70 m, p = 0.02), mean pulmonary artery pressure (18 ± 4 vs. 32 ± 6 mmHg, p = 0.01), and pulmonary vascular resistance (111 ± 48 vs. 272 ± 109 dyn/s/cm5, p = 0.009). Serum cytokine profiling identified hepatocyte growth factor (HGF) as the only cytokine significantly different between the at-risk and definite PAH groups (225.8 ± 55.0 vs. 361.6 ± 164.5 pg/mL, q<0.1%). Profiling of longitudinal samples of at-risk to definite PAH patients did not identify any significant changes in HGF or other cytokines over time. Conclusions Definite PAH patients expressed higher levels of HGF than at-risk patients. Further studies are needed to clarify the utility of HGF as a predictive biomarker for SSc-PAH.

scholarly journals Endothelial platelet-derived growth factor-mediated activation of smooth muscle platelet-derived growth factor receptors in pulmonary arterial hypertension

2020 ◽  
Vol 10 (3) ◽  
pp. 204589402094847
Author(s):  
Kang Wu ◽  
Haiyang Tang ◽  
Ruizhu Lin ◽  
Shane G. Carr ◽  
Ziyi Wang ◽  
...  
Keyword(s):  
Smooth Muscle ◽  
Growth Factor ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Growth Factor Receptor ◽  
Platelet Derived Growth Factor ◽  
Arterial Smooth Muscle Cell ◽  
Arterial Smooth Muscle ◽  
Pulmonary Arterial ◽  
Pulmonary Arterial Smooth Muscle

Platelet-derived growth factor is one of the major growth factors found in human and mammalian serum and tissues. Abnormal activation of platelet-derived growth factor signaling pathway through platelet-derived growth factor receptors may contribute to the development and progression of pulmonary vascular remodeling and obliterative vascular lesions in patients with pulmonary arterial hypertension. In this study, we examined the expression of platelet-derived growth factor receptor isoforms in pulmonary arterial smooth muscle and pulmonary arterial endothelial cells and investigated whether platelet-derived growth factor secreted from pulmonary arterial smooth muscle cell or pulmonary arterial endothelial cell promotes pulmonary arterial smooth muscle cell proliferation. Our results showed that the protein expression of platelet-derived growth factor receptor α and platelet-derived growth factor receptor β in pulmonary arterial smooth muscle cell was upregulated in patients with idiopathic pulmonary arterial hypertension compared to normal subjects. Platelet-derived growth factor activated platelet-derived growth factor receptor α and platelet-derived growth factor receptor β in pulmonary arterial smooth muscle cell, as determined by phosphorylation of platelet-derived growth factor receptor α and platelet-derived growth factor receptor β. The platelet-derived growth factor-mediated activation of platelet-derived growth factor receptor α/platelet-derived growth factor receptor β was enhanced in idiopathic pulmonary arterial hypertension-pulmonary arterial smooth muscle cell compared to normal cells. Expression level of platelet-derived growth factor-AA and platelet-derived growth factor-BB was greater in the conditioned media collected from idiopathic pulmonary arterial hypertension-pulmonary arterial endothelial cell than from normal pulmonary arterial endothelial cell. Furthermore, incubation of idiopathic pulmonary arterial hypertension-pulmonary arterial smooth muscle cell with conditioned culture media from normal pulmonary arterial endothelial cell induced more platelet-derived growth factor receptor α activation than in normal pulmonary arterial smooth muscle cell. Accordingly, the conditioned media from idiopathic pulmonary arterial hypertension-pulmonary arterial endothelial cell resulted in more pulmonary arterial smooth muscle cell proliferation than the media from normal pulmonary arterial endothelial cell. These data indicate that (a) the expression and activity of platelet-derived growth factor receptor are increased in idiopathic pulmonary arterial hypertension-pulmonary arterial smooth muscle cell compared to normal pulmonary arterial smooth muscle cell, and (b) pulmonary arterial endothelial cell from idiopathic pulmonary arterial hypertension patients secretes higher level of platelet-derived growth factor than pulmonary arterial endothelial cell from normal subjects. The enhanced secretion (and production) of platelet-derived growth factor from idiopathic pulmonary arterial hypertension-pulmonary arterial endothelial cell and upregulated platelet-derived growth factor receptor expression (and function) in idiopathic pulmonary arterial hypertension-pulmonary arterial smooth muscle cell may contribute to enhancing platelet-derived growth factor/platelet-derived growth factor receptor-associated pulmonary vascular remodeling in pulmonary arterial hypertension.

scholarly journals Pulmonary veno-occlusive disease in a patient with recently diagnosed systemic sclerosis

2019 ◽  
Vol 5 (2) ◽  
pp. NP1-NP4
Author(s):  
Nina M van Leeuwen ◽  
Sofia Ramiro ◽  
Maarten K Ninaber ◽  
Esther Nossent ◽  
Jeska K de Vries-Bouwstra
Keyword(s):  
Systemic Sclerosis ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Occlusive Disease ◽  
Endothelin Receptor Antagonists ◽  
Endothelin Receptor ◽  
Phosphodiesterase Type 5 Inhibitors ◽  
Pulmonary Arterial ◽  
Phosphodiesterase Type ◽  
Worse Prognosis

Pulmonary veno-occlusive disease is a rare cause of pulmonary hypertension in patients with systemic sclerosis that can be misclassified as pulmonary arterial hypertension. Differentiation between pulmonary veno-occlusive disease and pulmonary arterial hypertension is challenging because of the similar clinical picture. Nevertheless, discrimination is important because pulmonary veno-occlusive disease has a worse prognosis. Vasodilators including phosphodiesterase type 5 inhibitors and endothelin receptor antagonists should be started with caution and often in combination with diuretics to prevent pulmonary edema.

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