Pulmonary arterial hypertension associated with systemic sclerosis in the Czech Republic

Jansa, Pavel; Bečvář, Radim; Ambrož, David; Paleček, Tomáš; Tomčík, Michal; Skacelova, Simona; Aschermann, Michael; Linhart, Aleš

doi:10.1007/s10067-011-1896-x

Cited by 13 publications

(22 citation statements)

References 13 publications

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“…In the same vein, in the study by Jansa et al, females outnumbered males (82.3%) and the average age of the subjects was similar to our study (53.8 years old) (16). The number of SSc patients was evaluated in line with the reference books and the age range of this disease (2).…”

Section: Discussionsupporting

confidence: 87%

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Echocardiographic Test and the Early Detection of Systolic Failure in Systemic Sclerosis Patients: Strain and Strain Rate Echocardiography

et al. 2016

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Section: Discussionsupporting

confidence: 87%

“…Comparing to the findings of an Italian cohort study (22) and a Czech Republic cohort study (16), the prevalence of PAH in SSc patients (36.6%) was found to be much higher in our study. In addition, another study found that such prevalence was even higher (64% and 61%) in patients with tricuspid valve failure (4).…”

Section: Discussioncontrasting

confidence: 56%

Echocardiographic Test and the Early Detection of Systolic Failure in Systemic Sclerosis Patients: Strain and Strain Rate Echocardiography

et al. 2016

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“…Age over 60 years, male sex and DLCO <39% are other significant predictors of mortality in patients with SSc-associated PAH [7]. The prevalence of PAH was 7.08% in a study of 203 SSc patients [8]. A larger Italian cohort (867 SSc patients) showed a lower rate of PAH prevalence (3.7%) [9].…”

Section: Discussionmentioning

confidence: 99%

Contribution of Speckle Tracking to Estimation of Pulmonary Hypertension by Standard Doppler Echocardiography in Patients with Systemic Sclerosis and MCTD

Vymětal¹,

Hutyra²,

Smarzova³

et al. 2017

Rheumatology (Sunnyvale)

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Objectives: Development of pulmonary arterial hypertension (PAH) in systemic sclerosis (SSc) represents one of the most serious complications. The study aimed at assessing right ventricular (RV) global longitudinal strain and its relation to pulmonary artery pressure.Methods: Echocardiography was performed in 74 patients of whom 60 had SSc and 14 had mixed connective tissue disease (MCTD). Besides routine left and right ventricle assessment and pulmonary pressure estimation by Doppler echocardiography, RV fractional area change (FAC) and 2D strain of the RV free wall were evaluated. At the same time, levels of N-terminal pro-brain natriuretic peptide (NT-proBNP), troponin T and auto-antibodies were measured and all patients had diffusing capacity of the lung for carbon monoxide (DLCO) determined. All patients with probable PAH underwent right heart catheterization.Results: Pulmonary hypertension was found to be unlikely in 59 patients (79.7%), possible in 10 patients (13.5%) and probable in 5 patients (6.7%). Significant associations were found between pulmonary artery systolic pressure (PASP) and RV free wall global longitudinal strain (r=0.292; p=0.023), between PASP and NT-proBNP (r=0.436; p=0.001) and between PASP and FAC (r=0.320; p=0.005). Pulmonary artery systolic pressure did not correlate with left ventricular systolic or diastolic function parameters in this cohort. Conclusion:Speckle tracking of the RV represents a useful additional tool in RV assessment in relation to PH in SSc and MCTD patients; right heart catheterization remains an essential method for PAH confirmation.

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“…sildenafil, tadalafil) or intravenous prostanoids (epoprostenol) represent current specific treatment of PAH. Early initiation of therapy is advocated 37,38 .…”

Section: Therapymentioning

confidence: 99%

Emergency situations in rheumatology with a focus on systemic autoimmune diseases

Vymětal¹,

Skácelová²,

Smrzova³

et al. 2016

BIOMED PAP

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Background and Aim. Rheumatic diseases are commonly considered chronic conditions. However, acute manifestations can be very severe and represent a diagnostic problem. Examples are systemic lupus erythematosus with acute flare, glomerulonephritis, CNS disorders and catastrophic antiphospholipid syndrome, scleroderma with interstitial lung disease, pulmonary hypertension and renal crisis and polyangiitis with alveolar haemorhage and acute respiratory failure. This aim of this paper is to overview emergency situations which can be encountered in the care of patients with autoimmune systemic diseases and vasculitides. Methods. A Pubmed search for both original and review articles, recent textbooks and current guidelines related to rheumatic diseases with possible acute situations were included in this review article. Relevant image documentation was obtained at the site over the past several years of observation. Conclusions. This paper provides an overview of facts and emergency situations which can be encountered in the care of patients with autoimmune systemic diseases and vasculitides. It is directed at clinicians working in intensive care. It provides a differential diagnostic overview and information which is rare and commonly underestimated.

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Pulmonary arterial hypertension associated with systemic sclerosis in the Czech Republic

Abstract: Prevalence of PAH in our SSc cohort was 7.08%. SSc patients diagnosed with PAH through screening have less advanced disease in terms of haemodynamics than those with PAH diagnosed previously based on symptoms; their prognosis is therefore likely to be more favourable.

Cited by 13 publications

References 13 publications

Echocardiographic Test and the Early Detection of Systolic Failure in Systemic Sclerosis Patients: Strain and Strain Rate Echocardiography

Echocardiographic Test and the Early Detection of Systolic Failure in Systemic Sclerosis Patients: Strain and Strain Rate Echocardiography

Contribution of Speckle Tracking to Estimation of Pulmonary Hypertension by Standard Doppler Echocardiography in Patients with Systemic Sclerosis and MCTD

Emergency situations in rheumatology with a focus on systemic autoimmune diseases

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