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.

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 Replacing a phosphodiesterase-5 inhibitor with riociguat in patients with connective tissue disease-associated pulmonary arterial hypertension: a case series

2017 ◽  
Vol 7 (3) ◽  
pp. 741-746 ◽  
Author(s):  
Amresh Raina ◽  
Raymond L. Benza ◽  
Harrison W. Farber
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Connective Tissue ◽  
Arterial Hypertension ◽  
Clinical Response ◽  
Connective Tissue Disease ◽  
Case Series ◽  
Response To Treatment ◽  
Tolerability Profile ◽  
Phosphodiesterase Type 5 Inhibitors ◽  
Pulmonary Arterial

Patients with pulmonary arterial hypertension associated with connective tissue disease (PAH-PAH-CTD) such as systemic sclerosis (SSc) have a poorer response to treatment and increased mortality compared with patients with idiopathic PAH. Current treatment options for PAH-CTD include prostanoids, phosphodiesterase type-5 inhibitors (PDE-5i), endothelin receptor antagonists, and the soluble guanylate cyclase stimulator riociguat. In this case series, we describe three patients with PAH-CTD related to limited scleroderma who were switched from a PDE-5i to riociguat due to insufficient clinical response. The switch to riociguat was associated with an improvement in respiratory and hemodynamic parameters and a favorable tolerability profile. These cases demonstrate that switching to riociguat is a therapeutic option in patients with PAH-CTD who have not achieved a satisfactory clinical response to a PDE-5i.

scholarly journals Clinical experience with bosentan and sitaxentan in connective tissue disease-associated pulmonary arterial hypertension

Rheumatology ◽  
2010 ◽  
Vol 49 (11) ◽  
pp. 2147-2153 ◽  
Author(s):  
C. J. Valerio ◽  
C. E. Handler ◽  
P. Kabunga ◽  
C. J. Smith ◽  
C. P. Denton ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Connective Tissue ◽  
Arterial Hypertension ◽  
Connective Tissue Disease ◽  
Clinical Experience ◽  
Pulmonary Arterial

Pulmonary arterial hypertension associated with connective tissue disease

ESC CardioMed ◽  
2018 ◽  
pp. 2531-2534
Author(s):  
Christopher P. Denton
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Connective Tissue ◽  
Arterial Hypertension ◽  
Connective Tissue Disease ◽  
Lupus Erythematosus ◽  
Heart Catheterization ◽  
Pulmonary Arterial ◽  
Detection And Diagnosis ◽  
Health Organization

Connective tissue disease-associated pulmonary arterial hypertension (PAH) falls within World Health Organization group 1. These patients are treated as others in this group, but there are important considerations regarding detection and diagnosis. Patients with connective tissue disease are at risk of PAH and should be screened with confirmation of diagnosis by right heart catheterization. Treatment follows the European Society of Cardiology guidelines for other forms of PAH. However, more information is available for systemic sclerosis PAH regarding screening, including the DETECT algorithm, and also in terms of long-term outcome of patients with borderline elevation of mean pulmonary arterial pressure. In cases of systemic lupus erythematosus or mixed connective tissue disease, immunosuppression should be given in conjunction with targeted PAH-specific therapy. Long-term outcomes for PAH in patients with connective tissue disease have improved since targeted specific therapies became available. Recent trials with morbidity–mortality endpoints and a high proportion of patients receiving combination treatment have shown comparable benefits for patients with connective tissue disease and PAH as for those with idiopathic PAH in contrast to the blunted response that was characteristic of earlier short-term studies assessing improvement in 6 min walk test distance.

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