Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0036421 (PSS)
 10,989 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We describe two systemic sclerosis (SSc) patients with isolated pulmonary arterial hypertension (PAH) who were given treatment with 50 mg oral sildenafil per day. We evaluated the efficacy of oral sildenafil for isolated PAH in SSc patients by direct assessment with cardiac catheterization before and 6 months after the initiation of sildenafil. Right-heart catheterization demonstrated decreased mean pulmonary artery pressure, decreased pulmonary vascular resistance, and increased cardiac output after treatment with sildenafil. Brain natriuretic peptide levels were gradually decreased. The 6-min walking distance was greatly extended. Moreover, the physical conditions of both patients were much improved. We recognized no adverse events. We propose that oral sildenafil may be beneficial as a selective pulmonary vasodilator and as long-term treatment in SSc patients with isolated PAH.
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PMID:Successful treatment with sildenafil in systemic sclerosis patients with isolated pulmonary arterial hypertension: two case reports. 1591 31

Survival rates in pulmonary arterial hypertension (PAH) associated with rheumatic diseases, in particular connective tissue diseases such as systemic sclerosis, are even lower than in idiopathic PAH. These low survival rates highlight the need for early diagnosis and treatment in these patients. Transthoracic Doppler-echocardiography is most often used for diagnostic screening of patients at risk. Other screening tests are serum pro-brain-natriuretic peptide (pro-BNP) and diffusion capacity for carbon monoxide (DLCO), which appear to be changed early in the course of the PAH associated with connective tissue diseases. The diagnosis needs to be confirmed by right heart catheterization, which is recommended in all patients with suspected PAH. Besides the conventional background therapy, a number of specific therapies have been evaluated in randomized controlled trials in the recent years. These therapies include prostacyclins and prostacyclin analogues, endothelin-receptor antagonists and phosphodiesterase-5 inhibitors. Response to treatment can be measured by exercise capacity (e.g. 6 min walk distance) and pro-BNP, although certain aspects of validation for these outcome measures are lacking in PAH associated with connective tissue diseases.
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PMID:Pulmonary arterial hypertension and rheumatic diseases--from diagnosis to treatment. 1698 Jul 19

Systemic sclerosis (SSc) is a connective tissue disease, which may lead to pulmonary artery hypertension (PAH). N-terminal pro-brain natriuretic peptide (NT-proBNP) is a biologic marker for the diagnosis and treatment of congestive heart failure. The aim of our study was to investigate the potential role of the plasma NT-proBNP assay in the assessment of functional status and right heart performance in systemic sclerosis-related pulmonary hypertension (SScPAH). Systolic pulmonary artery pressure (sPAP) assessed by echocardiography, six-minute walk test (SMWT) and plasma NT-proBNP levels were recorded from 45 SSc patients. Mean value of NT-proBNP for SSc patients with PAH (n=14) was 691.7+/-325.7 fmol/L compared to 417.4+/-167.1 fmol/L for patients without PAH (n=31) (p=0.0007). In SSc patients we found a statistically significant correlation between NT-proBNP values and sPAP (r=0.32, p=0.03). Amongst SScPAH patients, NT-proBNP values were significantly correlated with sPAP (r=0.73, p=0.003) and inversely correlated with the SMWT (r=-0.60, p=0.02). These results suggest NT-proBNP as a useful additional biological tool in the evaluation and management of SScPAH patients.
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PMID:Neurohormonal activation in patients with systemic sclerosis-related pulmonary arterial hypertension. 1708 4

We evaluated the clinical significance of serum N-terminal pro-brain natriuretic peptide (NT-proBNP) level in systemic sclerosis (SSc). We studied 45 SSc patients (30 with limited and 15 with diffuse cutaneous SSc) of mean age +/- SD 47.1 +/- 12.9 years, mean duration of disease 10.2 +/- 6.0 years, and 45 age- and sex-matched healthy controls. Pulmonary artery pressure was measured by echocardiography. Lung involvement was evaluated by pulmonary function testing and by using high-resolution computed tomography scores. Serum NT-proBNP levels were measured using a sandwich electrochemiluminescent immunoassay. Serum NT-proBNP levels were significantly higher in patients with SSc compared to healthy controls. When the patients were divided into clinical subsets, serum NT-proBNP was higher in diffuse SSc than in limited SSc. Serum NT-proBNP levels were found to be positively correlated with age, skin thickness score, and systolic pulmonary artery pressure and negatively correlated with percentage of carbon monoxide diffusion capacity (DLco). Multivariate analysis showed that serum NT-proBNP levels were positively correlated with age (p = 0.010), skin thickness score (p = 0.000), and blood pressure (p = 0.021) and negatively correlated with %DLco (p = 0.016). Fifty-seven percent of the variation in log (proBNP) can be explained by the multivariate model (R (2) = 0.57). Serum NT-proBNP levels were higher in SSc patients (particularly the diffuse subset) than in healthy controls and were found to be correlated with skin thickness and %DLco. We conclude that serum NT-proBNP may be a biologic marker of skin fibrosis and pulmonary vascular involvement in SSc.
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PMID:The clinical significance of serum N-terminal pro-brain natriuretic peptide in systemic sclerosis patients. 1903 12

Systemic sclerosis (SSc) is characterized by vascular dysfunction that may lead to pulmonary artery hypertension (PAH). The N-terminal pro-B type natriuretic peptide (NT-proNBP), a marker of cardiac failure, is a diagnostic marker of early PAH in patients with SSc without heart failure. Our aim was to determine whether NT-proBNP levels may be a useful tool to evaluate the response to bosentan therapy in patients with PAH secondary to SSc. Ten patients with symptomatic, severe PAH secondary to SSc, received bosentan, 62.5 mg twice a day for 4 weeks followed by 125 mg twice a day for 7 months. Ten patients with SSc without PAH served as controls for basal level of NT-proBNP. Blood samples were obtained before the beginning of the therapy and after 3 and 7 months of treatment. SSc patients with PAH had significantly higher serum levels of NT-proBNP than those without PAH, at baseline. After 3 and 7 months of therapy, NT-proBNP concentration showed a progressive decrease, nearly approaching statistical difference at 7 months when compared to baseline levels (P=0.953 and P=0.600). Our results show that serum NT-proBNP levels may be a useful marker for the response to bosentan therapy in patients with PAH secondary to SSc.
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PMID:N-terminal pro-BNP in sclerodermic patients on bosentan therapy for PAH. 1869 Apr 44

Elevated serum brain natriuretic peptide (BNP) released from myocytes of ventricles upon stretch have been found in patients with isolated right ventricular (RV) pressure overload. However, limited data suggest that serum BNP may be elevated in systemic sclerosis (SSc) patients, especially with RV dysfunction. We assessed serum N-terminal proBNP (NT-proBNP) in SSc and evaluated whether it reflects the severity of RV overload. We prospectively studied 51 consecutive patients (47F, mean age 53.3 +/- 15.2 years) with SSc (mean disease duration 9 +/- 12.4 years). The control group formed 31 healthy subjects (27F, mean age 52.6 +/- 12.1 years). NT-proBNP level, 6-minute walking test (6MWT), and transthoracic echocardiography (TTE) for the assessment of RV overload were performed. Serum NT-proBNP exceeded the reference value of 125 pg/mL in 31 (61%) SSc patients. The mean serum log NT-proBNP concentration in SSc was higher than in controls (2.138 +/- 0.527 vs. 1.634 +/- 0.420 pg/mL, p < 0.001). 13 (25%) SSc patients have tricuspid regurgitation peak gradient (TRPG) exceeding 31 mmHg reflecting pulmonary arterial hypertension (PAH). The SSc presented other echocardiographic signs of RV overload. Mean 6MWT distance was shorter in SSc than in controls (528 +/- 100 vs. 617 +/- 80 m, p < 0.001). NT-proBNP level correlated positively with TRPG, RV diameter, RV Tei index and negatively with 6MWT distance. ROC analysis identified >115 pg/ml as the best NT-proBNP threshold predicting PAH for SSc patients (sensitivity 92%, specificity 44%). Results of our study suggest that NT-proBNP measurement is a useful screening method for PAH in SSc patients. Since elevated plasma NT-proBNP level reflects the degree of right ventricular overload and limitation of exercise capacity, abnormal NT-proBNP levels should imply further evaluation including echocardiography.
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PMID:Non-invasive diagnostic and functional evaluation of cardiac involvement in patients with systemic sclerosis. 1825 71

N-terminal pro-brain natriuretic peptide (NT-proBNP) is a marker of neurohormonal activation that is useful in the diagnosis and prognosis of various forms of pulmonary arterial hypertension (PAH). We sought to characterise and compare NT-proBNP in a cohort of PAH related to systemic sclerosis (PAH-SSc) and idiopathic PAH (IPAH) patients. NT-proBNP levels, collected from PAH-SSc and IPAH patients followed prospectively, were compared and correlated with haemodynamic variables. Cox proportional hazard models were created to assess the predictive value of NT-proBNP. 98 patients (55 PAH-SSc, 43 IPAH) were included. Haemodynamics were similar, except for lower mean pulmonary arterial pressure in PAH-SSc. NT-proBNP levels were significantly higher in PAH-SSc (3,419+/-3,784 versus 1,393+/-1,633 pg x mL(-1); p<0.01) and were more closely related to haemodynamics in PAH-SSc than IPAH. 28 patients died. NT-proBNP predicted survival (hazard ratio (HR) 3.18; p<0.01) in the overall cohort; however, when stratified by group, predicted survival only in PAH-SSc (HR 3.07, p<0.01 versus 2.02, p = 0.29 in IPAH). This is the first description showing NT-proBNP levels are 1) significantly higher in PAH-SSc than IPAH despite less severe haemodynamic perturbations, and 2) stronger predictors of survival in PAH-SSc, suggesting that neurohormonal regulation may differ between PAH-SSc and IPAH. Future studies to define pertinent mechanisms are warranted.
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PMID:Disproportionate elevation of N-terminal pro-brain natriuretic peptide in scleroderma-related pulmonary hypertension. 2004 55

Heart involvement, including primary myocardial involvement, is very common in systemic sclerosis (SSc). When clinically evident, cardiac involvement is recognized to be a very poor prognostic factor. Thus pre-clinical identification is highly encouraged. Echocardiography, including pulsed tissue Doppler echocardiography, is the cornerstone of routine heart assessment as it allows the detection of reduced systolic/ diastolic function, as well as the measurement of pulmonary artery pressure, and the possible detection of valvular or pericardial involvement. Myocardial perfusion may be also assessed by single photon emission computed tomography, and if available, by cardiac magnetic resonance imaging. Since the introduction of routine assay for natriuretic peptides, and their initial application for the diagnosis of acute heart failure, B-type natriuretic peptide assays are now recommended for a wide number of applications and have been introduced as a major tool, in particular in primary care, in worldwide guidelines. Within the context of SSc, recent studies have demonstrated that BNP and NT-proBNP are highly relevant for the diagnosis and the prediction of pulmonary hypertension occurrence. Moreover, NT-proBNP allows the detection of both reduced left ventricular/right ventricular contractility, or pulmonary hypertension, suggesting its potential role as a first line tool in primary care setting for the overall cardiac assessment of SSc in which cardiovascular complications are a burden.
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PMID:N-terminal pro brain natriuretic peptide: the new cornerstone of cardiovascular assessment in systemic sclerosis. 1979 64

Primary myocardial involvement is common in systemic sclerosis (SSc). We evaluated the atrial and ventricular electromechanical characteristics by using tissue Doppler echocardiography in SSc patients with subclinical cardiac involvement. Twenty-seven consecutive patients (24 women; mean age +/- SD 49.9 +/- 11.3 years) presenting with SSc without pulmonary arterial hypertension or symptomatic heart failure were prospectively studied. Electrocardiographic P-wave dispersion (Pd), corrected QT dispersion (QTcd), interatrial, intra-atrial, interventricular, and intraventricular electromechanical delays were analyzed by tissue Doppler echocardiography, and brain natriuretic peptide levels were measured. Results were compared with 17 healthy controls. There was no difference in conventional and tissue Doppler parameters between the two groups. However, patients with SSc had higher mean Pd (mean [+/-SD] 46.8 +/- 15 and 36 +/- 8 ms, respectively, P = 0.004) and mean interatrial electromechanical delay time (DT) (mean [+/-SD] 32.2 +/- 9.2 and 24.7 +/- 9.7 ms, respectively, P = 0.01), mean electromechanical delay time for all segments (Mean Ts) (mean [+/-SD] 148.8 +/- 18.8 and 129.3 +/- 13.4 ms, respectively, P < 0.001), and intraventricular DT (mean [+/-SD] 27.6 +/- 12.5 and 16.2 +/- 7.2 ms, respectively, P < 0.001). Intraventricular DT was the only parameter that correlated significantly with the Mean Ts. Brain natriuretic peptide levels were within normal limits in both groups; however, they were higher in patients with SSc than in controls (mean [+/-SD] 37.5 +/- 28.5 and 23.1 +/- 16.0 pg/ml, respectively, P = 0.03). The evaluation of atrial and ventricular electromechanical parameters by using tissue Doppler echocardiography seems to be useful for detection of subclinical cardiac involvement in SSc patients with normal conventional echocardiographic findings.
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PMID:Impact of systemic sclerosis on electromechanical characteristics of the heart. 2051 50

Early detection of pulmonary hypertension (PH) in patients with systemic sclerosis (SSc) is essential as it leads to substantial morbidity and mortality irrespective of its etiology. The aim of our study was to determine whether noninvasive biochemical and/or echocardiographic indices can predict the presence of PH in these patients. We prospectively studied 66 patients (mean age of 57.7 +/- 12.1 years, 63 women) with SSc without clinical manifestations of heart failure. All patients underwent standard and tissue Doppler echocardiography. Plasma N-terminal pro-B type natriuretic peptide (NT-proBNP) and asymmetric dimethylarginine (ADMA) levels were also measured. In 24 (36%) patients, the diagnosis of PH was established by echocardiography (systolic pulmonary artery pressure value > or =40 mmHg). Left atrial (LA) volume, NT-proBNP, ADMA, ratio of early transmitral filling velocity to early diastolic velocity of the mitral annulus (mitral E/E (m)), and right ventricular myocardial performance index (MPI) were univariate predictors of PH. In multivariate analysis, NT-proBNP, LA volume, and right ventricular MPI were independent predictors of PH in SSc patients. LA volume and NT-proBNP may be useful noninvasive markers for the prediction of elevated pulmonary artery pressure in patients with SSc. These parameters should be considered when assessing this population for risk stratification and for identification of patients demanding further investigation and institution of specific therapy for the disease at the time when it is most likely to be effective.
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PMID:Left atrial volume and N-terminal pro-B type natriuretic peptide are associated with elevated pulmonary artery pressure in patients with systemic sclerosis. 2052 41


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