Risk assessment in patients with systemic sclerosis and pulmonary arterial hypertension

The current treatment of patients with pulmonary arterial hypertension (PAH) is founded on repeated risk stratification at diagnosis and during follow-up. The 2015 European pulmonary hypertension guidelines propose a multidimensional risk assessment strategy based on nine items and 13 distinct variables in order to predict the estimated 1-year mortality [1, 2]. Recently, several large registry studies have shown that simplified tools can be used to predict outcomes and guide treatment decisions [3–6]. These studies have focused largely on 6-min walking distance (6MWD), World Health Organization (WHO) functional class (FC), right atrial pressure (RAP), cardiac index (CI) and brain natriuretic peptides (BNP or NT-proBNP). Low-risk criteria were defined as WHO FC I or II, 6MWD>440 m, RAP
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: Pulmonary vascular disease Editorials Source Type: research

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The objective of this prospective study was to evaluate right ventricular function and pulmonary arterial compliance (PAC=stroke volume/pulse pressure) at rest and during exercise in patients with systemic sclerosis (SSc) with normal mean pulmonary artery pressures (mPAP) at rest, mildly elevated mPAP (mPAP 21 ‐24mmHg) and manifest pulmonary hypertension (mPAP≥25mmHg).MethodsPatients with SSc (n=112) underwent clinical assessment and right heart catheterization at rest and during exercise and were divided into three groups according to their resting mPAP values: normal mPAP ( ≤20mmHg), mildly elevated mPAP (21‐24...
Source: Arthritis and Rheumatology - Category: Rheumatology Authors: Tags: Full Length Source Type: research
ConclusionsPrevalence of PAH was lower than expected (3.7%). Probability of having elevated esPAP was higher among patients with dSSc and among those with ATA.
Source: Clinical Rheumatology - Category: Rheumatology Source Type: research
Background: Pulmonary involvement is the leading cause of death in SSc and can manifest as interstitial lung disease (ILD), pulmonary hypertension (PAH) or a combination (ILD-PH). Aim of this analysis was to determine prevalence, clinical characteristics and outcomes of these different forms within the German SSc Network.Methods: SSc pts were analyzed for pulmonary involvement, clinical characteristics and outcome.Results: There were 3699 pts in 42 centers with a mean follow up time of 34.4±12.6 months. At baseline, ILD was frequent (29.5%), while ILD-PH and PAH had lower prevalence (7.5%, 6.1%). At the end of follo...
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: ILD/DPLD of known origin Source Type: research
Background: Scleroderma-associated pulmonary arterial hypertension (SScPAH) has worse prognosis compared to idiopathic PAH (IPAH). Mortality risk predictors from recent European guidelines have been evaluated mostly in IPAH.Aim: To evaluate the ability of guidelines’ risk assessment tool in predicting mortality and to explore the impact of achieving low-risk profile on long-term prognosis in SScPAH.Methods: 151 newly diagnosed SScPAH enrolled in the Johns Hopkins Pulmonary Hypertension Program were categorized according to a score of 1 (low-risk), 2 (intermediate-risk) or 3 (high-risk) defined as the average of the s...
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: Pulmonary hypertension Source Type: research
Pulmonary arterial hypertension (PAH) is a leading cause of mortality in patients with systemic sclerosis (SSc). A post-hoc analysis of the DETECT study on SSc-patients demonstrated that borderline hemodynamics with mean pulmonary arterial pressure (PAP) of 21-24 mmHg (BoPAP) may represent an intermediate stage between normal mean PAP (
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: Pulmonary hypertension Source Type: research
Background: Right ventricular (RV) dysfunction is the leading cause of mortality in pulmonary arterial hypertension (PAH), and cardiac magnetic resonance (CMR) measures of RV volumes and mass are known to predict mortality in PAH. Ventricular mass index (VMI), a ratio of RV mass to left ventricular (LV) mass, predicts the presence of PAH in scleroderma. We hypothesized that VMI would predict mortality in incident PAH.Methods: 65 subjects with incident PAH underwent CMR, and VMI was calculated (RV end-diastolic mass/LV end-diastolic mass). Subjects were followed prospectively until death or censor. Associations between VMI ...
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: Pulmonary hypertension Source Type: research
Objective: Obstructive sleep apnoea (OSA) may contribute to pulmonary hypertension in patients with scleroderma (SSc). Main pulmonary artery (mPA) diameter derived from chest computer tomography (CT) is a reliable predictor of pulmonary hypertension. In the current study, we explored the relationship between OSA indices and enlarged mPA in patients with SSc.Material and Methods: Sixty-two consecutive patients with SSc (58 female) were included. All patients underwent lung function test, carbonmonoxide diffusion capacity (DLCO), chest CT, and overnight sleep recording with cardiorespiratory polygraphy. OSA diagnosis was bas...
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: Sleep and control of breathing Source Type: research
The prognostic importance of follow-up haemodynamics and the validity of multidimensional risk assessment are not well established for systemic sclerosis (SSc)-associated pulmonary arterial hypertension (PAH). We assessed incident SSc-PAH patients to determine the association between clinical and haemodynamic variables at baseline and first follow-up right heart catheterisation (RHC) with transplant-free survival. RHC variables included cardiac index, stroke volume index (SVI), pulmonary arterial compliance and pulmonary vascular resistance. Risk assessment was performed according to the number of low-risk criteria: functi...
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: Original Articles: Pulmonary hypertension Source Type: research
Publication date: Available online 12 October 2018Source: Autoimmunity ReviewsAuthor(s): José Pedro L. Nunes, André C. Cunha, Tiago Meirinhos, Alzira Nunes, Paulo M. Araújo, Ana R. Godinho, Eduardo M. Vilela, Carlos VazAbstractThe prevalence of auto-antibodies associated to pulmonary arterial hypertension in scleroderma patients was reviewed, based on reports cited in two major scientific databases.Data was collected on the following types of antibodies: antinuclear, anti-double-stranded DNA, anticentromere, anti-CENP-A, anti-CENP-B, anti-bicaudal D2, anti-nucleolar, anti-Scl-70 (anti-topoisomerase I),...
Source: Autoimmunity Reviews - Category: Allergy & Immunology Source Type: research
Authors: Rizzi M, Radovanovic D, Santus P, Airoldi A, Frassanito F, Vanni S, Cristiano A, Sarzi-Puttini P, Atzeni F Abstract The 6-minute walk test (6MWT) is a standardised, feasible and reliable measure of sub-maximal exercise capacity that has never been fully validated in systemic sclerosis (SSc). A variety of data suggest that many non-pulmonary aspects of SSc contribute to the test results, thus blunting the ability of the 6MWT to measure changes in lung function. Sources of variability are a training effect, technician experience, subject encouragement, medication, other activities on day of testing, decondit...
Source: Clinical and Experimental Rheumatology - Category: Rheumatology Tags: Clin Exp Rheumatol Source Type: research
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