scholarly journals Serum uric acid as a marker of disease risk, severity, and survival in systemic sclerosis-related pulmonary arterial hypertension

2019 ◽  
Vol 9 (3) ◽  
pp. 204589401985947 ◽  
Author(s):  
Catherine E. Simpson ◽  
Rachel L. Damico ◽  
Laura Hummers ◽  
Rubina M. Khair ◽  
Todd M. Kolb ◽  
...  
Keyword(s):  
Uric Acid ◽  
Systemic Sclerosis ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Disease Severity ◽  
Serum Uric Acid ◽  
Elevated Serum ◽  
Baseline Serum ◽  
Increased Risk ◽  
Pulmonary Arterial

The object of this paper is to assess associations between serum uric acid (UA) and pulmonary arterial hypertension (PAH) risk, disease severity, and mortality in a well-characterized cohort of systemic sclerosis (SSc) patients referred for evaluation of possible PAH. Consecutive SSc patients aged >18 years with serum UA drawn within two weeks of a diagnostic right heart catheterization (RHC) were included. Associations between baseline serum UA and PAH at RHC were examined using logistic regression and receiver operating characteristic curves. Relationships between UA levels and metrics of disease severity were assessed using Pearson and Spearman correlation. Associations between UA and survival were assessed using Kaplan–Meier analysis and Cox proportional hazard modeling. A total of 162 SSc patients were included; 82 received a diagnosis of PAH at RHC. Patients found to have PAH had significantly higher UA than those without PAH. Elevated baseline UA was associated with significantly increased odds of PAH diagnosis at RHC (odds ratio [OR] = 4.07, 95% confidence interval [CI] = 2.11–7.87, P < 0.001). Each mg/dL higher UA was associated with a 14% increase in mortality (hazard ratio [HR] = 1.14, 95% CI = 1.02–1.28, P < 0.05). In multivariable models adjusting for potential confounders of the relationship between UA and survival, UA > 6.3 mg/dL remained significantly associated with increased mortality (HR = 1.84, 95% CI = 1.02–3.32, P < 0.05). Among SSc patients with suspected PAH, elevated serum UA is associated with increased risk of SSc-PAH. Among individuals diagnosed with SSc-PAH by RHC, UA is associated with disease severity and survival. These results indicate UA is a useful predictor of PAH risk and prognosis in SSc.

scholarly journals Serum uric acid is associated with disease severity and may predict clinical outcome in patients of pulmonary arterial hypertension secondary to connective tissue disease in Chinese: a single-center retrospective study

2020 ◽  
Vol 20 (1) ◽  
Author(s):  
Jingya Wang ◽  
Yuanyuan Wang ◽  
Xiaodi Li ◽  
Yingheng Huang ◽  
Xiaoxuan Sun ◽  
...  
Keyword(s):  
Uric Acid ◽  
Retrospective Study ◽  
Survival Rate ◽  
Pulmonary Arterial Hypertension ◽  
Connective Tissue ◽  
Arterial Hypertension ◽  
Serum Uric Acid ◽  
Connective Tissue Disease ◽  
Baseline Serum ◽  
Pulmonary Arterial

Abstract Background Previous studies have shown that serum uric acid (UA) levels are correlated with the severity of idiopathic pulmonary arterial hypertension (IPAH) and are predictors of disease prognosis. Still, few studies have explored the value of serum UA in pulmonary arterial hypertension secondary to connective tissue disease (CTD-PAH). This retrospective study aimed to investigate the clinical value of serum UA levels in patients with CTD-PAH. Methods Fifty CTD-PAH patients were enrolled in our study, from which baseline UA levels, respective variations, and additional clinical data were collected. The potential association between baseline UA level and severity of CTD-PAH was investigated. Furthermore, the relationship between baseline UA and survival rate of CTD-PAH patients, as well as between UA variations and survival rate of pulmonary hypertension secondary to connective tissue disease (CTD-PH) patients was discussed. Results Baseline serum UA levels were positively correlated with pulmonary vascular resistance (PVR). During the follow-up period, 3 CTD-PAH and 12 CTD-PH patients died. Kaplan-Meier survival curves showed lower survival rate in patients with hyperuricemia than in patients with normouricemia, in both groups (CTD-PAH group p = 0.041, CTD-PH group p = 0.013). Concerning serum UA variations, patients with persistent hyperuricemia showed the lowest survival rate when compared with patients with steady normouricemia (p = 0.01) or patients with decresing serum UA levels, i.e. undergoing from a status of hyperuricemia to a status of normouricemia (p = 0.023). Conclusion Baseline serum UA levels might predict severity of CTD-PAH. Together with baseline values, changes of uric acid level may predict the clinical prognosis of the disease.

Pulmonary arterial hypertension screening of systemic sclerosis patients in clinical practice: an independent chart review

2017 ◽  
Vol 2 (3) ◽  
pp. 231-234 ◽  
Author(s):  
Dylan Kelly ◽  
Karen A. Beattie ◽  
Maggie J. Larché
Keyword(s):  
Systemic Sclerosis ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Chart Review ◽  
Screening Tests ◽  
Future Research ◽  
Increased Risk ◽  
Annual Screening ◽  
Pulmonary Arterial ◽  
Function Testing

Patients with systemic sclerosis (SSc) are at increased risk of pulmonary arterial hypertension (PAH). Guidelines recommend annual screening with pulmonary function testing (PFT) and transthoracic echocardiogram (TTE). Through auditing the charts of 11 rheumatologists associated with McMaster University, we evaluated the proportion of SSc patients without PAH or pulmonary fibrosis who receive annual TTE, PFT, and dyspnea screening. Screening rates between self-identified SSc experts and non-experts were compared. In cases where screening tests were abnormal, charts were reviewed for evidence of cardiologist or respirologist referral. In total, 136 patients’ charts were included. Annual screening for dyspnea was very common (88% of patients, 119/134). Annual PAH screening via TTE (74%, 100/135) and PFT (79%, 107/136) was less common. Annual dyspnea screening, TTE, and PFT were more commonly performed by SSc experts than by non-experts (94% vs. 83%, p = 0.03; 85% vs. 61%, p = 0.002; 93% vs. 62%, p<0.001, respectively). Nearly all patients with an abnormal TTE (10/11, 91%) and PFT (12/14, 86%) received appropriate referrals. Future research should explore reasons for differences in screening rates between SSc experts and non-experts. Given that rheumatologists screen for dyspnea more often than they order PFT and TTE, there may be barriers to ordering these tests that warrant further investigation.

Tricuspid Annular Plane Systolic Excursion Is a Robust Outcome Measure in Systemic Sclerosis-associated Pulmonary Arterial Hypertension

2011 ◽  
Vol 38 (11) ◽  
pp. 2410-2418 ◽  
Author(s):  
STEPHEN C. MATHAI ◽  
CHRISTOPHER T. SIBLEY ◽  
PAUL R. FORFIA ◽  
JAMES O. MUDD ◽  
MICAH R. FISHER ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Pulmonary Arterial Hypertension ◽  
Cardiac Index ◽  
Arterial Hypertension ◽  
Heart Catheterization ◽  
Class Iii ◽  
Risk Of Death ◽  
Increased Risk ◽  
Pulmonary Arterial ◽  
Rv Function

Objective.The tricuspid annular plane systolic excursion (TAPSE) strongly reflects right ventricular (RV) function and predicts survival in idiopathic pulmonary arterial hypertension (PAH). But its role in systemic sclerosis (SSc)-associated PAH has not been established. Our objective was to validate the TAPSE in the assessment of RV function and prediction of survival in SSc-PAH.Methods.Fifty consecutive patients with SSc-PAH who underwent echocardiography with TAPSE measurement within 1 h of clinically indicated right heart catheterization were followed prospectively. The relationship between TAPSE and measures of RV function and measures of survival was assessed.Results.The majority of the cohort were women in New York Heart Association class III/IV with severe PAH (mean cardiac index 2.4 ± 0.8 l/min/m2). RV function was significantly impaired (mean cardiac index 2.1 ± 0.7 vs 2.9 ± 0.8 l/min/m2; p < 0.01) and RV afterload was significantly greater (mean pulmonary vascular resistance 11.1 ± 5.1 vs 5.8 ± 2.5 Wood units; p < 0.01) in subjects with a TAPSE ≤ 1.7 cm. The proportion surviving in the low TAPSE group was significantly lower [0.56 (95% CI 0.37–0.71) and 0.46 (95% CI 0.28–0.62) vs 0.87 (95% CI 0.55–0.96) and 0.79 (95% CI 0.49–0.93), 1- and 2-year survival, respectively]. TAPSE ≤ 1.7 cm conferred a nearly 4-fold increased risk of death (HR 3.81, 95% CI 1.31–11.1, p < 0.01).Conclusion.TAPSE is a robust measure of RV function and strongly predicts survival in patients with PAH-SSc. Future studies are needed to identify the responsiveness of TAPSE to PAH-specific therapy and to assess its diagnostic utility in PAH-SSc.

scholarly journals Significance of Serum Uric Acid in Children with Pulmonary Arterial Hypertension

2016 ◽  
Vol 1 (1) ◽  
pp. 46-50 ◽  
Author(s):  
Carmen Corina Şuteu ◽  
Theodora Benedek ◽  
Rodica Togănel
Keyword(s):  
Uric Acid ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Right Ventricular ◽  
Serum Uric Acid ◽  
Walk Test ◽  
Echocardiographic Parameters ◽  
Pulmonary Arterial ◽  
6 Minute Walk Test ◽  
Minute Walk

AbstractIntroduction:Pulmonary arterial hypertension (PAH) is a complex disease with poor prognosis. Serum uric acid has been proposed as a potentially non-invasive and objective parameter for prognosis and response to therapy.Objectives:To investigate the potent relationship between serum uric acid levels and functional and echocardiographic parameters in children with PAH.Methods:Serum uric acid levels were measured in 34 children with PAH and were correlated with the functional class, 6-minute walk test, and echocardiographic parameters at baseline and at 12 months follow-up.Results:In pediatric PAH patients serum uric acid levels were higher compared with the control subjects (p = 0.001). In the high uric acid group serum uric acid levels were correlated with 6-minute walk test (p = 0.008), and with several echocardiographic parameters, such as pulmonary vascular resistance (p = 0.04), fractional area change (p = 0.05), left ventricle eccentricity index (p = 0.04), right atrial area (p = 0.03), right ventricle myocardial index (p = 0.01), and pericardial effusion (p = 0.001), markers of right ventricular overload and dysfunction.Conclusions:Serum uric acid levels are easy to collect and measure, and correlate with both functional and echocardiographic parameters that reflect right ventricular dysfunction.

Comparison of Brain Natriuretic Peptide (BNP) and NT-proBNP in Screening for Pulmonary Arterial Hypertension in Patients with Systemic Sclerosis

2010 ◽  
Vol 37 (10) ◽  
pp. 2064-2070 ◽  
Author(s):  
LORENZO CAVAGNA ◽  
ROBERTO CAPORALI ◽  
CATHERINE KLERSY ◽  
STEFANO GHIO ◽  
RICCARDO ALBERTINI ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Brain Natriuretic Peptide ◽  
Natriuretic Peptide ◽  
Heart Catheterization ◽  
Multicenter Studies ◽  
Link Type ◽  
Increased Risk ◽  
Pulmonary Arterial

Objective.To compare the performance of brain natriuretic peptide (BNP) and N-terminal pro-brain natriuretic peptide (NT-proBNP) in screening for pulmonary arterial hypertension (PAH) in systemic sclerosis (SSc).Methods.Between January 2008 and March 2009, outpatients referred to our unit and satisfying LeRoy criteria for SSc were assessed for PAH. Doppler echocardiography, BNP measurement, and NT-proBNP measurement were done concomitantly for a complete clinical, instrumental, and biochemical evaluation. Right-heart catheterization was carried out in cases of suspected PAH [estimated pulmonary arterial pressure (PAP) ≥ 36 mm Hg; diffusion capacity for carbon monoxide (DLCO) ≤ 50% of predicted value; 1-year DLCO decline ≥ 20% in absence of pulmonary fibrosis; unexplained dyspnea].Results.One hundred thirty-five patients were enrolled (124 women, 11 men; 96 limited SSc, 39 diffuse SSc); precapillary PAH was found in 20 patients (15 limited SSc, 5 diffuse SSc). The estimated PAP correlated with both BNP (R = 0.3; 95% CI 0.14–0.44) and NT-proBNP (R = 0.3, 95% CI 0.14–0.45). BNP [area under the curve (AUC) 0.74, 95% CI 0.59–0.89] was slightly superior to NT-proBNP (AUC 0.63, 95% CI 0.46–0.80) in identification of PAH, with diagnosis cutoff values of 64 pg/ml (sensitivity 60%, specificity 87%) and 239.4 pg/ml (sensitivity 45%, specificity 90%), respectively. BNP (log-transformed, p = 0.032) and creatinine (p = 0.049) were independent predictors of PAH, while NT-proBNP was not (p = 0.50).Conclusion.In our single-center study, the performance of BNP was slightly superior to that of NT-proBNP in PAH screening of patients with SSc, although normal levels of these markers do not exclude diagnosis. We observed that impaired renal function is associated with an increased risk of PAH in SSc. Further multicenter studies are needed to confirm our results (ClinicalTrials.gov ID NCT00617487).

scholarly journals Screening strategies for pulmonary arterial hypertension

2019 ◽  
Vol 21 (Supplement_K) ◽  
pp. K9-K20 ◽  
Author(s):  
David G Kiely ◽  
Allan Lawrie ◽  
Marc Humbert
Keyword(s):  
Systemic Sclerosis ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Clinical Signs ◽  
Population Based ◽  
Healthcare Data ◽  
Increased Risk ◽  
Time To Diagnosis ◽  
Patients At Risk ◽  
Pulmonary Arterial

Abstract Pulmonary arterial hypertension (PAH) is rare and, if untreated, has a median survival of 2–3 years. Pulmonary arterial hypertension may be idiopathic (IPAH) but is frequently associated with other conditions. Despite increased awareness, therapeutic advances, and improved outcomes, the time from symptom onset to diagnosis remains unchanged. The commonest symptoms of PAH (breathlessness and fatigue) are non-specific and clinical signs are usually subtle, frequently preventing early diagnosis where therapies may be more effective. The failure to improve the time to diagnosis largely reflects an inability to identify patients at increased risk of PAH using current approaches. To date, strategies to improve the time to diagnosis have focused on screening patients with a high prevalence [systemic sclerosis (10%), patients with portal hypertension assessed for liver transplantation (2–6%), carriers of mutations of the gene encoding bone morphogenetic protein receptor type II, and first-degree relatives of patients with heritable PAH]. In systemic sclerosis, screening algorithms have demonstrated that patients can be identified earlier, however, current approaches are resource intensive. Until, recently, it has not been considered possible to screen populations for rare conditions such as IPAH (prevalence 5–15/million/year). However, there is interest in the use of artificial intelligence approaches in medicine and the application of diagnostic algorithms to large healthcare data sets, to identify patients at risk of rare conditions. In this article, we review current approaches and challenges in screening for PAH and explore novel population-based approaches to improve detection.

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