scholarly journals Serum Markers of Cardiac Complications In a Systemic Sclerosis Cohort

Author(s):  
Anders Heiervang Tennøe ◽  
Klaus Murbræch ◽  
Henriette Didriksen ◽  
Thor Ueland ◽  
Vyacheslav Palchevskiy ◽  
...  

Abstract Background: Primary cardiac involvement is one of the leading causes of mortality in systemic sclerosis (SSc), but little is known regarding circulating biomarkers for cardiac SSc. Here, we aimed to investigate potential associations between cardiac SSc and candidate serum markers.Methods: Serum samples from patients of the Oslo University SSc cohort and 100 healthy controls were screened against two custom-made candidate marker panels containing molecules deemed relevant for cardiopulmonary and/or fibrotic diseases. Left (LV) and right ventricular (RV) dysfunction was assessed by protocol echocardiography, performed within three years from serum sampling. Patients suspected of pulmonary hypertension underwent right heart catheterization. Vital status at study end was available for all patients. Descriptive analyses, logistic and Cox regressions were conducted to assess associations between cardiac SSc and candidate serum markers. Results: The 371 patients presented an average age of 57.2 (± 13.9) years. Female sex (84%) and limited cutaneous SSc (73%) were predominant. Association between LV diastolic dysfunction and tumor necrosis factor-related apoptosis-inducing ligand (TRAIL) (OR 0.41, 95%CI 0.21-0.78, p=0.007) was identified. LV systolic dysfunction defined by global longitudinal strain was associated with angiopoietin 2 (ANGPT2) (OR 3.42, 95%CI 1.52-7.71, p=0.003) and osteopontin (OPN) (OR 1.95, 95%CI 1.08-3.52, p=0.026). RV systolic dysfunction, measured by tricuspid annular plane systolic excursion, was associated to markers of LV dysfunction (ANGPT2, OPN, and TRAIL) (OR 1.67, 95%CI 1.11-2.50, p=0.014, OR 1.86, 95%CI 1.25-2.77, p=0.002, OR 0.32, 95%CI 0.15-0.66, p=0.002, respectively) and endostatin (OR 1.86, 95%CI 1.22-2.84, p=0.004).Conclusion: ANGPT2, OPN and TRAIL seem to be circulating biomarkers associated with both LV and RV dysfunction in SSc.

2020 ◽  
Vol 5-6 (215-216) ◽  
pp. 15-23
Author(s):  
Nazym Junusbayeva ◽  
◽  
Bakytsholpan Issayeva ◽  

Systemic sclerosis is the most common autoimmune disease associated with pulmonary hypertension. Pulmonary hypertension is a potential predictor of early death, and therefore, recently, interest in a comprehensive study of the diagnosis of pulmonary hypertension associated with systemic sclerosis has been increasing among researchers. Aim. Consideration of current diagnostic issues, as well as the principles of using diagnostic algorithms to verify pulmonary hypertension associated with systemic sclerosis, applied in daily clinical practice. Material and methods. In order to study the literature data, a search was carried out for information on this problem up to 10 years in depth in the Web of Science, PubMed/MEDLINE. During the search, the following terms were used individually and in combination: "systemic sclerosis", "pulmonary hypertension", "DETECT algorithm", "catheterization of the right heart chambers". The main search criteria were studies based on the study of patients with pulmonary hypertension associated with systemic sclerosis: meta-analyzes, original studies, retrospective and cohort studies. Results and discussion. The clinical symptoms of pulmonary hypertension can be nonspecific, which greatly complicates the diagnosis of the disease in the early stages. The absence of a specific clinical picture before the development of an advanced stage of pulmonary hypertension leads to late verification of the diagnosis. Key algorithms used in the daily practice of a doctor make it possible to minimize the number of undiagnosed cases of pulmonary hypertension. Conclusions. Diagnosis of PH associated with systemic sclerosis is often challenging for clinicians to practice. Early diagnosis and therefore treatment of PH are of paramount importance as they improve survival rates in patients with systemic sclerosis. Keywords: systemic sclerosis, pulmonary hypertension, screening, DETECT algorithm, right heart catheterization.


2019 ◽  
Vol 46 (11) ◽  
pp. 1495-1501
Author(s):  
Sophie Billet ◽  
Grégory Pugnet ◽  
Thomas Chollet ◽  
Gaétan Charbonnier ◽  
Pauline Fournier ◽  
...  

Objective.To explore the accuracy of tricuspid regurgitation velocity (TRV) to right ventricular outflow tract time-velocity integral (TVIRVOT) ratio by Doppler to determine pulmonary vascular resistance (PVR) in patients with systemic sclerosis (SSc).Methods.Thirty-five consecutive adult patients with SSc, fulfilling the 2013 European League Against Rheumatism/American College of Rheumatology classification criteria, with sinus rhythm referred for right heart catheterization (RHC), were retrospectively included. All patients underwent a transthoracic echocardiogram (TTE) performed within 24 h of RHC. Patients with SSc were recruited regardless of disease activity, cardiac symptoms, and treatment regimen. Doppler measurements were compared to RHC measurements. A linear regression equation was generated to predict PVR by echocardiogram based on the TRV/TVIRVOT ratio. The accuracy of Doppler measurements for predicting PVR > 3 Wood units was assessed by computing the areas under the receiver-operating characteristic curves.Results.There were 20 (57%) females in the study. The mean age was 65 ± 12 years. Mean and systolic pulmonary arterial pressures were 31 ± 8 and 53 ± 15 mmHg, respectively. There was a good correlation between TRV/TVIRVOT ratio assessed by Doppler and PVR measured by RHC (R = 0.743, p < 0.001). The equation generated by this analysis was the following: PVR by Doppler = 11.3 × (TRV/TVIRVOT) + 1.7. A cutoff value of 0.21 for TRV/TVIRVOT ratio provided the best sensitivity (86%) and specificity (86%) to determine PVR > 3 Wood units.Conclusion.Our study suggests that TTE using Doppler could be a useful and noninvasive tool for estimating PVR in patients with SSc.


2011 ◽  
Vol 39 (1) ◽  
pp. 100-105 ◽  
Author(s):  
HERMAN M.A. HOFSTEE ◽  
ALEXANDRE E. VOSKUYL ◽  
ANTON VONK NOORDEGRAAF ◽  
YVO M. SMULDERS ◽  
PIET E. POSTMUS ◽  
...  

Objective.Impaired microvascular function may contribute to organ complications in patients with systemic sclerosis (SSc). We investigated whether SSc patients with and without pulmonary arterial hypertension (PAH) show a graded impairment of microvascular function compared to healthy controls.Methods.Twenty-two patients with SSc and 22 controls were studied. All patients underwent right heart catheterization; 6 had no PAH (SSc-nonPAH) and 16 had PAH (SSc-PAH). Acetylcholine (ACh)-mediated endothelium-dependent vasodilatation and sodium nitroprusside (SNP)-mediated endothelium-independent vasodilatation were assessed by iontophoresis combined with laser Doppler flowmetry.Results.Compared to sex- and age-matched controls, ACh-mediated vasodilatation was reduced in SSc-PAH (340.4% vs 79.5%, respectively; p < 0.01), but not in SSc-nonPAH (340.4% vs 397.9%; p = 0.90). No significant differences were present between the groups in SNP-mediated vasodilatation.Conclusion.Systemic microvascular endothelium-dependent vasodilatation is markedly reduced in SSc complicated by PAH.


2019 ◽  
Vol 4 (3) ◽  
pp. 219-224
Author(s):  
Gabor Kovacs ◽  
Horst Olschewski

Pulmonary vascular disease represents one of the most frequent complications in systemic sclerosis leading to increased mortality. The recognition and appropriate clinical management of early pulmonary vascular disease could significantly improve the prognosis of affected patients. Early pulmonary vascular disease is characterized by the histological signs of pulmonary vascular remodeling, mildly increased mean pulmonary arterial pressure (21–24 mmHg) at rest, abnormal pulmonary hemodynamics during exercise, decreased exercise capacity, and a high risk for development of pulmonary arterial hypertension. Pulmonary hemodynamics can be investigated during exercise by echocardiography or by right heart catheterization both representing important clinical tools for the screening and confirmation of early pulmonary vascular disease. Further studies are needed to better understand the clinical course of systemic sclerosis patients with early pulmonary vascular disease and to define the characteristics of patients that will or will not profit from pulmonary arterial hypertension treatment.


Rheumatology ◽  
1997 ◽  
Vol 36 (2) ◽  
pp. 239-243 ◽  
Author(s):  
C. P. Denton ◽  
J. B. Cailes ◽  
G. D. Phillips ◽  
A. U. Wells ◽  
C. M. Black ◽  
...  

2021 ◽  
Vol 10 (7) ◽  
pp. 1528
Author(s):  
Ioanna Minopoulou ◽  
Marieta Theodorakopoulou ◽  
Afroditi Boutou ◽  
Alexandra Arvanitaki ◽  
Georgia Pitsiou ◽  
...  

Systemic sclerosis (SSc)-related pulmonary arterial hypertension (SSc-PAH) is a leading cause of mortality in SSc. The extent of peripheral microvasculopathy assessed through nailfold capillaroscopy might correlate with the presence of PAH in SSc patients. We searched the PubMed, Cochrane Library, Scopus, and Web of Science databases and performed a random effects meta-analysis of observational studies comparing nailfold capillaroscopic alterations in SSc-PAH versus SSc-noPAH patients. Weighted mean differences (WMD) with the corresponding confidence intervals (CIs) were estimated. The quality of the included studies was evaluated using a modified Newcastle–Ottawa scale. Seven studies with 101 SSc-PAH and 277 SSc-noPAH participants were included. Capillary density was marginally reduced in the SSc-PAH group (WMD: −1.0, 95% CI: −2.0 to 0.0, I2 = 86%). This effect was strengthened once PAH diagnosis was confirmed by right heart catheterization (WMD: −1.2, 95% CI: −2.3 to −0.1, I2 = 85%). An increase in capillary loop width was observed in SSc-PAH compared to SSc-noPAH patients (WMD: 10.9, 95% CI: 2.5 to 19.4, I2 = 78%). Furthermore, SSc-PAH patients had a 7.3 times higher likelihood of active or late scleroderma pattern (95% CI: 3.0 to 18.0, I2 = 4%). SSc-PAH patients presented with worse nailfold capillaroscopic findings compared to SSc-noPAH patients.


2019 ◽  
Vol 18 (1) ◽  
pp. 48-54
Author(s):  
N. A. Klyaus ◽  
M. A. Simakova ◽  
A. L. Maslyanskiy ◽  
O. M. Moiseeva

Introduction. The problem of early detection of pulmonary arterial hypertension (PAH) is closely related to the diagnosis of systemic sclerosis (SSc). It is especially important in patients with slight manifestations of the disease, making it diffcult to verify the diagnosis before the clinic of right ventricular heart failure, and low knowledge of the general practitioners of the available diagnostic opportunities.The aim of the work was to identify the earliest clinical signs of PAH associated with SSc for its early diagnosis.Material and methods. The study included 51 patients with limited SSc. The investigators evaluated the extent of skin lesion, levels of the level of N­terminal brain natriureticpropeptide (NT­proBNP) and uric acid, the fluorescent pattern and titer of antinuclear factor. All patients underwent an echocardiography, nailfold videocapillaroscopy, a comprehensive assessment of external respiratory functions. When there were symptoms of PAH, right heart catheterization was performed to confrm it.Results. PAH was detected in 19 of the 51 patients included in the study. Patients with PAH were signifcantly older than those without PAH: (60±8) and (54±7) years, respectively (p=0.02); there were also differences between these groups in the signs of right cardiac remodeling (p<0.05), in the indices estimated in the six­minute walk test (p<0.05), in the assessment of lung diffusing capacity (p<0.001), in levels of NT­proBNP and uric acid (p<0.001), in the density of the nailfold capillaries (p=0.009); in a semiquantitative assessment of nailfold capillary alterations (p=0.022). Among patients with PAH associated with SSc, seven patients were diagnosed with SSc for the frst time after referring to cardiologists and verifying the genesis of PAH, while other 12 patients were previously observed by rheumatologists. Differences in the duration of the period from the onset of symptoms to the diagnosis of SSc (p=0.043) were revealed between these groups.Conclusion. The study shows the probability of the presence of SSc in patients with suspected idiopathic PAH, that determines the necessity of additional immunological and instrumental examinations in such patients.


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