scholarly journals Endothelin-1 Levels in Scleroderma Patients: A Pilot Study

2013 ◽  
Vol 2013 ◽  
pp. 1-4 ◽  
Author(s):  
Emanuele Cozzani ◽  
Sanja Javor ◽  
Erika Laborai ◽  
Massimo Drosera ◽  
Aurora Parodi
Keyword(s):  
Retrospective Study ◽  
Systemic Sclerosis ◽  
Pilot Study ◽  
Serum Levels ◽  
Vascular Wall ◽  
Digital Ulcers ◽  
Normal Range ◽  
Endothelin 1 ◽  
Treatment And Prevention ◽  
Severity Of The Disease

Endothelin-1 (ET-1) is a potent endogenous vasoconstrictor, which mediates vascular wall cells proliferation, fibrosis, and inflammation through two types of ET-1 receptors (ET-A and ET-B). In our retrospective study the serum levels of ET-1 in 18 systemic sclerosis (SSc) patients with and without digital ulcers (DUs) were assessed to observe possible correlation between the levels of ET-1, the evolution of SSc, and the therapy with an ET-1 antagonist (bosentan). In all our patients, the levels of ET-1 were found higher than normal range and correlate with the severity of the disease. Furthermore we also observed that in patients without DUs the levels of ET-1 were higher and did not correlate with new DUs development. In conclusion, the levels of ET-1 in our studied patients do not correlate with the possible development of DUs. The reduction of ET-1 levels in DUs patients in therapy with bosentan confirms the efficacy of this molecule both for treatment and prevention of digital ulcers. The inhibition of ET-A receptor by its antagonist may activate the opposite ET-B receptors, with well-known function ET-1 degradation and reducing of ET-1 serum level as confirmed in our pilot study.

scholarly journals A PILOT STUDY ON SOLUBLE (PRO)RENIN RECEPTOR IN SYSTEMIC SCLEROSIS

2015 ◽  
Vol 24 (2) ◽  
pp. 108-113
Author(s):  
Marilena Gorga ◽  
◽  
Alina-Mihaela Soare ◽  
Ana-Maria Gherghe ◽  
Rucsandra Dobrota ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Pilot Study ◽  
Wnt Signaling ◽  
Angiotensin Receptor Blockers ◽  
Severe Disease ◽  
Serum Levels ◽  
Digital Ulcers ◽  
Serum Samples ◽  
Reported Data ◽  
Spearman Test

Background. Wnt signaling is involved in fibrosis, but the mechanisms of cross-talk with other pathways, like TGF-β, are not fully understood. (Pro)renin receptor (PRR) functions as an accessory protein to a V-ATP-ase responsible for acidic pH maintenance in intracellular compartments, and recent data indicate that PRR could be a regulator of Wnt signaling. A circulating fragment (sPRR) is generated by enzymatic cleavage of the extracellular domain and can be quantified in plasma, serum and urine. Objectives. This is a pilot study, to explore the serum concentrations of the soluble (pro)renin receptor (sPRR) in patients with systemic sclerosis (SSc). Patients and methods. Serum samples from 29 subjects with a confirmed diagnosis of SSc have been tested using an ELISA method. Clinical and laboratory parameters have been analysed for associations with sPRR serum levels. Results and conclusion. Serum levels of sPRR were higher in patients with a history of digital ulcers (p 0.041, Mann-Whitney U-test) and those with digital pitting scars (p 0.037, Mann-Whitney U-test). Serum levels of sPRR correlated with serum creatinine (r 0.424, Spearman test), in line with previously reported data. Our results indicate the need for a larger study to assess the sPRR changes in patients with SSc, including more cases with severe disease and excluding a possible impact of angiotensin-receptor blockers.

scholarly journals Reassessing the Role of the Active TGF-β1 as a Biomarker in Systemic Sclerosis: Association of Serum Levels with Clinical Manifestations

2016 ◽  
Vol 2016 ◽  
pp. 1-6 ◽  
Author(s):  
Andréa Tavares Dantas ◽  
Sayonara Maria Calado Gonçalves ◽  
Anderson Rodrigues de Almeida ◽  
Rafaela Silva Guimarães Gonçalves ◽  
Maria Clara Pinheiro Duarte Sampaio ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Clinical Manifestations ◽  
Serum Levels ◽  
Vascular Involvement ◽  
Digital Ulcers ◽  
Serum Samples ◽  
Peripheral Blood Mononuclear ◽  
Pbmc Culture ◽  
Significant Difference ◽  
Skin Biopsies

Objective. To determine active TGF-β1 (aTGF-β1) levels in serum, skin, and peripheral blood mononuclear cell (PBMC) culture supernatants and to understand their associations with clinical parameters in systemic sclerosis (SSc) patients.Methods. We evaluated serum samples from 56 SSc patients and 24 healthy controls (HC). In 20 SSc patients, we quantified spontaneous or anti-CD3/CD28 stimulated production of aTGF-β1 by PBMC. The aTGF-β1 levels were measured by ELISA. Skin biopsies were obtained from 13 SSc patients and six HC, and TGFB1 expression was analyzed by RT-PCR.Results. TGF-β1 serum levels were significantly higher in SSc patients than in HC (p< 0.0001). Patients with increased TGF-β1 serum levels were more likely to have diffuse subset (p= 0.02), digital ulcers (p= 0.02), lung fibrosis (p< 0.0001), positive antitopoisomerase I (p= 0.03), and higher modified Rodnan score (p= 0.046). Most of our culture supernatant samples had undetectable levels of TGF-β1. No significant difference in TGFB1 expression was observed in the SSc skin compared with HC skin.Conclusion. Raised active TGF-β1 serum levels and their association with clinical manifestations in scleroderma patients suggest that this cytokine could be a marker of fibrotic and vascular involvement in SSc.

scholarly journals Effect of sildenafil on digital ulcers in systemic sclerosis: analysis from a single centre pilot study

2009 ◽  
Vol 69 (8) ◽  
pp. 1475-1478 ◽  
Author(s):  
C. S. Brueckner ◽  
M. O. Becker ◽  
T. Kroencke ◽  
D. Huscher ◽  
H. U. Scherer ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Pilot Study ◽  
Digital Ulcers ◽  
Single Centre

Lidocaine controls pain and allows safe wound bed preparation and debridement of digital ulcers in systemic sclerosis: a retrospective study

2016 ◽  
Vol 36 (1) ◽  
pp. 209-212 ◽  
Author(s):  
Francesca Braschi ◽  
Francesca Bartoli ◽  
Cosimo Bruni ◽  
Ginevra Fiori ◽  
Claudia Fantauzzo ◽  
...  
Keyword(s):  
Retrospective Study ◽  
Systemic Sclerosis ◽  
Digital Ulcers ◽  
Wound Bed Preparation

Serum Levels of Galectin-3: Possible Association with Fibrosis, Aberrant Angiogenesis, and Immune Activation in Patients with Systemic Sclerosis

2012 ◽  
Vol 39 (3) ◽  
pp. 539-544 ◽  
Author(s):  
TAKASHI TANIGUCHI ◽  
YOSHIHIDE ASANO ◽  
KANAME AKAMATA ◽  
SHINJI NODA ◽  
YURI MASUI ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Immune Activation ◽  
Developmental Process ◽  
Systolic Pressure ◽  
Serum Levels ◽  
Digital Ulcers ◽  
Healthy Controls ◽  
Ventricular Systolic Pressure ◽  
Multifunctional Protein ◽  
Galectin 3

Objective.Galectin-3 is a multifunctional protein implicated in a variety of biological processes including fibrosis, angiogenesis, and immune activation, all of which are associated with the development of systemic sclerosis (SSc). We investigated the clinical significance of serum galectin-3 levels in SSc.Methods.Serum galectin-3 levels were determined by a specific ELISA in 58 patients with SSc and 19 healthy controls.Results.Serum galectin-3 levels were significantly lower in patients with diffuse cutaneous SSc (dcSSc) than in controls (3.29 ± 3.27 ng/ml vs 4.91 ± 2.67 ng/ml, respectively; p < 0.05), while being comparable between limited cutaneous SSc (3.70 ± 2.39 ng/ml) and healthy controls. In dcSSc, serum galectin-3 levels significantly correlated with total skin score (r = 0.45, p < 0.05). Serum galectin-3 levels were significantly decreased in early dcSSc (disease duration < 1 year; 1.64 ± 1.74 ng/ml; p < 0.05), but not in mid-stage dcSSc (1 to 6 years; 3.22 ± 3.16 ng/ml) or late-stage dcSSc (> 6 years; 4.86 ± 4.10 ng/ml), compared with controls. Serum galectin-3 levels were higher in SSc patients with both digital ulcers (DU) and elevated right ventricular systolic pressure (RVSP) than in those without each symptom (DU: 5.44 ± 3.74 ng/ml vs 2.99 ± 2.36 ng/ml, p < 0.05; elevated RVSP: 4.44 ± 3.14 ng/ml vs 2.82 ± 2.64 ng/ml, p < 0.05).Conclusion.Galectin-3 may be related to the developmental process of skin sclerosis in dcSSc and of DU and pulmonary vascular involvements in total SSc.

Autoantibodies against Endothelin 1 Type A Receptor Are Strong Predictors of Digital Ulcers in Systemic Sclerosis

2015 ◽  
Vol 42 (10) ◽  
pp. 1801-1807 ◽  
Author(s):  
Jérôme Avouac ◽  
Gabriela Riemekasten ◽  
Christophe Meune ◽  
Barbara Ruiz ◽  
André Kahan ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Type A ◽  
Digital Ulcers ◽  
Clinical Predictors ◽  
Endothelin 1 ◽  
History Of ◽  
Angiogenic Markers ◽  
Cox Analysis ◽  
Or History

Objective.To determine the predictive value of functional autoantibodies against vascular receptors for the development of ischemic digital ulcers (DU) in patients with systemic sclerosis (SSc).Methods.Angiotensin II Type 1 receptor (AT1R) and endothelin 1 Type A receptor (ETAR) autoantibodies were measured at baseline in a prospective cohort of 90 patients with SSc together with 5 validated angiogenic markers. The primary outcome was the occurrence of at least 1 new ischemic DU during the 5-year followup.Results.Twenty-four patients developed at least 1 new DU during the followup period. Univariate Cox analysis revealed that concentrations above the median value of anti-AT1R and anti-ETAR antibodies were predictive of the occurrence of ischemic DU (HR 2.85, 95% CI 1.19–6.84 and HR 3.39, 95% CI 1.35–8.50, respectively). A first multivariate Cox analysis including functional autoantibodies and clinical predictors of new DU confirmed anti-ETAR autoantibodies as independent predictors of the occurrence of new ischemic DU (HR 3.15, 95% CI 1.22–8.13) together with a history of DU at baseline. In a second model implemented with angiogenic markers, anti-ETAR autoantibodies remained an independent predictor of the occurrence of new ischemic DU (HR 9.59, 95% CI 1.75–52.64) together with the presence at baseline of active DU or history of DU.Conclusion.Anti-ETAR autoantibodies can be used together with the presence of current or past DU to identify patients with SSc who are at risk for the development of subsequent DU. These autoantibodies may allow for earlier management and therapeutic intervention.

Endothelin‐1 serum levels correlate with MCP‐1 but not with homocysteine plasma concentration in patients with systemic sclerosis

2006 ◽  
Vol 35 (2) ◽  
pp. 133-137 ◽  
Author(s):  
D. Peterlana ◽  
A. Puccetti ◽  
P. Caramaschi ◽  
D. Biasi ◽  
R. Beri ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Plasma Concentration ◽  
Serum Levels ◽  
Endothelin 1

scholarly journals Altered serum level of metabolic and endothelial factors in patients with systemic sclerosis

2019 ◽  
Vol 312 (6) ◽  
pp. 453-458
Author(s):  
Anna Stochmal ◽  
Joanna Czuwara ◽  
Michał Zaremba ◽  
Lidia Rudnicka
Keyword(s):  
Adipose Tissue ◽  
Systemic Sclerosis ◽  
Serum Level ◽  
Elevated Serum ◽  
Serum Levels ◽  
Enzyme Linked Immunosorbent Assay ◽  
Vascular Abnormalities ◽  
Endothelin 1 ◽  
Fibrotic Process ◽  
Galectin 3

Abstract Systemic sclerosis (SSc) is a chronic connective tissue disease characterized by progressive fibrosis, vascular impairment and immune abnormalities. In recent years, adipokines (mediators synthetized by adipose tissue) have been indicated as a possible missing link in the pathogenesis of SSc. The aim of this study was to investigate the serum concentration of metabolic adipose tissue factors: adiponectin, resistin, leptin and endothelial proteins: endothelin-1, fractalkine and galectin-3 in patients with systemic sclerosis. The study included 100 patients with confirmed SSc diagnosis and 20 healthy individuals. The concentration of respective proteins was determined by enzyme-linked immunosorbent assay. The following markers showed statistically significant increased mean concentrations in patients with SSc in comparison to healthy control: resistin (13.41 vs 8.54 ng/mL; P = 0.0012), endothelin-1 (1.99 vs 1.31 pg/mL; P = 0.0072) and fractalkine (2.93 vs 1.68 ng/mL; P = 0.0007). Elevated serum levels of galectin-3 (4.54 vs 3.26 ng/mL; P = 0.0672) and leptin (19,542 vs 14,210 pg/mL; P = 0.1817) were observed. Decreased concentration of adiponectin was found in patients with SSc (5150 vs 8847 pg/mL; P = 0.0001). Fractalkine and galectin-3 levels were significantly higher in diffuse cutaneous SSc than limited cutaneous SSc subset (3.93 ng/mL vs 2.58 ng/mL, P = 0.0018; 6.86 ng/mL vs 3.78 ng/mL, P = 0.0008, respectively) and correlated positively with modified Rodnan Skin Score in total SSc patients (r = 0.376, P = 0.0009; r = 0.236, P = 0.018, respectively). In conclusion, an increased serum level of resistin associated with increased endothelin-1 and fractalkine level and decreased adiponectin level may indicate a significant role of the adipose tissue in the development and progression of vascular abnormalities in patients with systemic sclerosis. Fractalkine and galectin-3 may participate in promoting and exacerbating the fibrotic process in SSc.

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