natriuretic-peptide--brain and Lung-Diseases--Interstitial

Lung diseases are one of the most frequent causes of pulmonary hypertension (PH). The development of PH influences the course of lung disease, worsening the clinical symptoms and prognosis. According to the most recent publications, PH in the course of lung diseases develops as a result of both "parenchymal" and vascular pathology, in the patients with genetic predisposition. Prolonged infection (especially viral one) may be an additional promoting factor. Right heart catheterization (RHC), which is an invasive procedure, is the only objective method of diagnosing PH. According to the latest recommendations, the management algorithm of PH and coexisting interstitial lung disease is based on RHC and the results of pulmonary function tests. Majority of the patients develop mild PH in the course of advanced lung disease. Best treatment of underlying lung pathology combined with long term oxygen treatment is recommended in this group. In case of severe PH (mean resting pulmonary artery pressure (mPAP) ≥ 35 mm Hg) the alternate cause of PH has to be sought. PAH-specific drugs use should be limited to patients with severe PH participating in clinical trials. In this review, the value of various non-invasive methods (echocardiography, radiological examination, exercise capacity and brain natriuretic peptides assessment) in the process of screening for PH is presented, and the results of recent randomized clinical trials with PAH-specific drugs in patients with diffuse parenchymal lung diseases are discussed.

Topics: Cardiac Catheterization; Clinical Trials as Topic; Echocardiography; Exercise; Female; Humans; Hypertension, Pulmonary; Idiopathic Pulmonary Fibrosis; Lung; Lung Diseases, Interstitial; Male; Middle Aged; Natriuretic Peptide, Brain; Oxygen; Prognosis; Respiratory Function Tests

Sodium-uretic peptides (SUP) are the main humoral system opposing pathological remodeling of vascular bed in diseases of cardiovascular system and kidneys. In the paper, pathogenetic and clinical significance of the cerebral SUP in pulmonary arterial hypertension, including the one associated with interstitial diseases of the lungs.

Topics: Animals; Humans; Hypertension, Pulmonary; Lung Diseases, Interstitial; Natriuretic Peptide, Brain

It is known that prognosis of pulmonary hypertension (PH), especially connective tissue disease related PH (CTD-PH), is serious. CTD-PH is caused by not only pulmonary arterial hypertension, but also interstitial pneumonitis, hypoxia, chronic thromboembolism of pulmonary artery, or left heart diseases. Recently, prognosis of PH is improved by progress of diagnostic measures including echocardiography and development of effective vasodilators. However, early diagnosis of PH is necessary for further improvement of the prognosis. Previous reports have shown that diffusion lung capacity for carbon monoxide (DLco) is reduced in the early phase of PH, and we have shown that serum lactate dehydrogenase level is also elevated in this phase. Furthermore, we have been studying significance of stress echocardiography. Therefore, if reduced DLco or LDH elevation are recognized, follow up of laboratory data such as blood levels of KL-6, NT-proBNP, D-dimer, echocardiography, high resolution CT of the lung, in addition to clinical findings are important for prediction of PH.

Topics: Biomarkers; Carbon Monoxide; Chronic Disease; Connective Tissue Diseases; Early Diagnosis; Echocardiography; Humans; Hypertension, Pulmonary; L-Lactate Dehydrogenase; Lung; Lung Diseases, Interstitial; Lung Volume Measurements; Mucin-1; Natriuretic Peptide, Brain; Peptide Fragments; Prognosis; Pulmonary Embolism; Tomography, X-Ray Computed

Pulmonary hypertension (PH) has long been recognized as a complication of chronic respiratory disease. Recent studies have highlighted the adverse impact PH has on the clinical course of these conditions and have cast doubt on the role of hypoxia in their pathogenesis. Clinicians should carefully consider the possibility of PH during the diagnostic evaluation of chronic respiratory disorders. The usefulness of pharmacologic therapy directed toward PH remains to be determined.

Topics: Chronic Disease; Comorbidity; Humans; Hypertension, Pulmonary; Lung Diseases, Interstitial; Natriuretic Peptide, Brain; Oxygen Inhalation Therapy; Positive-Pressure Respiration; Pulmonary Disease, Chronic Obstructive; Pulmonary Wedge Pressure; Respiratory Tract Diseases; Sleep Apnea Syndromes

Myocardial involvement (MCI) is known to increase morbidity and mortality in polymyositis (PM) and dermatomyositis (DM). This study aims to investigate whether complicating with ventricular arrhythmia (VA) predicts poor outcomes in patients with PM/DM-related myocardial involvement (PM/DM-MCI).. We reviewed all PM/DM-MCI patients admitted to Peking Union Medical College Hospital from October 1997 to April 2019. VA and the other possible risk factors for the composite endpoint, including death from any cause and rehospitalization for cardiac causes, were analyzed.. A total of 75 PM/DM-MCI patients (44 PM and 31 DM) were enrolled, of which 27 (36%) met the composite endpoint during a median follow-up of 24 months. Independent prognostic factors for the composite endpoint include VA [HR 4.215, 95% CI (1.737, 10.230)], NT-proBNP > 3415 pg/ml [HR 2.606, 95% CI (1.203, 5.646)], interstitial lung disease [HR 2.688, 95% CI (1.209, 5.978)], and anti-cardiac remodelling therapy [HR 0.302, 95% CI (0.115, 0.792)]. The 3-year event-free survival rate of patients without VA was significantly higher than that of patients with VA (63.3% vs 40.7%, P = 0.034). Skin lesions [OR 0.163, 95% CI (0.051, 0.523)] and positive antimitochondrial antibody [OR 3.484, 95% CI (1.192, 10.183)] were independent predictors of VA.. VA provides prognostic insights for PM/DM-MCI patients and predicts poor outcome. Polymyositis and positive antimitochondrial antibody are closely associated with the presence of VA in PM/DM-MCI.

Topics: Adrenergic beta-Antagonists; Adult; Angiotensin Receptor Antagonists; Angiotensin-Converting Enzyme Inhibitors; Arrhythmias, Cardiac; Autoantibodies; Cardiomyopathies; Dermatomyositis; Female; Humans; Lung Diseases, Interstitial; Male; Middle Aged; Mineralocorticoid Receptor Antagonists; Mitochondria; Natriuretic Peptide, Brain; Peptide Fragments; Polymyositis; Prognosis; Progression-Free Survival; Proportional Hazards Models; Retrospective Studies; Spironolactone; Survival Rate

Heart failure (HF) is growing in importance as a significant cause of disease and mortality. When It is suspected, it can be ruled out if BNP values are below 100 pg/mL. Diagnostic certainty can be obtained if echocardiogram shows reduced ejection fraction, diastolic dysfunction or right-sided heart disease. Physiological changes at high altitude are known to affect BNP values. This study pretends to evaluate BNP values when used for HF diagnosis in Huancayo, Perú, a high altitude population located at 3,250 m above sea level.. This is a cross-sectional, diagnostic test type study. A total of 83 medical charts of patients with suspected HF, admitted to the Emergency Room and Internal Medicine Service of Ramiro Prialé Prialé National Hospital, were reviewed. Data processing was performed with SPSS program for Windows version 21.0. Pearson's Chi Square test was used for categorical variables analysis and ANOVA for continuos variables. P values under 0.05 were considered significant.. Medium age was 74 years. Patient's characteristics that were associated with confirmed HF and high BNP levels were the following: presence of fatigue, night cough, elevated heart rate, shortness of breath, history of lung fibrosis and decreased oxygen arterial saturation (p < 0.05) Pulmonary hypertension, mitral and tricuspid regurgitation, and cor pulmonale were also associated with higher BNP levels. Most subjects had BNP values >100 pg/mL, with low specificity for HF diagnosis (11.5 %). Individuals without heart failure had mean BNP values above 300 pg/mL; while individuals with cor pulmonale had a mean of 975 pg/mL. BNP values were high in patients with or without HF. A cut-off point of ≥130pg/mL is proposed to increase specificity. The predictive capacity of BNP for HF identification at this high altitude population is low because of a high number of false positive results.

Topics: Adult; Aged; Aged, 80 and over; Altitude; Cross-Sectional Studies; Echocardiography, Doppler; Female; Heart Failure; Humans; Hypertension, Pulmonary; Hypoxia; Lung Diseases, Interstitial; Male; Middle Aged; Natriuretic Peptide, Brain; Peru; Vital Capacity

Topics: Aged; Echocardiography; Female; Humans; Hypertension, Pulmonary; Lung Diseases, Interstitial; Male; Middle Aged; Natriuretic Peptide, Brain; Patient Admission

This study evaluated the role of echocardiography and BNP in patients with interstitial lung disease (ILD), to identify those with PH and RV dysfunction. The aims of this study were: 1-to evaluate the accuracy of an algorithm including BNP, DLCO and echocardiographic measurements to identify PH and RV dysfunction; 2- to evaluate BNP and Echo values concordance in relation to right catheterization measurement.. We analyzed 113 patients with diagnosis of ILD. Echo examination included: Pulmonary systolic, diastolic and mean Arterial Pressure (PAPs, PAPd, PAP mean), End-Diastolic and End-Systolic right ventricle diameters, Inferior Caval Vein diameter, and Tricuspid Annular Plane Systolic Excursion (TAPSE). Patients revealing increased PAPs at echocardiography underwent to catheterization.. Patients with PAPs > 40 mm Hg (37 patients), PAPmean ≥ 25 mm Hg (23 patients) and PAPd ≥ 20 mm Hg showed BNP increased (157 ± 96 vs 16 ± 14 pg/ml p = 0.004; 201 ± 120 vs 28 ± 17 pg/mL; 124 ± 88 vs 23 ± 18 pg/ml p < 0.001) as patients with TAPSE ≤16 mm (25 patients) (145 ± 104 vs 26 ± 21 pg/ml p < 0.001). In catheterized patients (37 patients) BNP was increased in patients with invasive PAPs > 40 mm Hg (165 ± 112 vs 29 ± 14 pg/ml p < 0.02), as well as in patients with Wedge pressure > 14 mm Hg (199 + 153 vs 54 + 39 pg/mL; p = 0.01). ROC Curve analysis showed that elevated values of BNP, PAPs, PAP mean are able to assess PH. On the other hand, lower values of DLCO (<40%) and TAPSE (≤16 mm) detect PH. Logistic regression analysis of the previous parameters, confirmed their diagnostic role in PH detection.. In patients with ILD, an algorithm including BNP, DLCO and echocardiography could be useful for non invasive screening of PH.. ARTEMIS-HP trial; ID number: NCT00879229.

Topics: Aged; Algorithms; Biomarkers; Diagnosis, Differential; Echocardiography, Doppler; Female; Humans; Hypertension, Pulmonary; Italy; Lung Diseases, Interstitial; Male; Middle Aged; Natriuretic Peptide, Brain; Patient Selection; Prevalence; Retrospective Studies; Risk Factors; Severity of Illness Index; Tertiary Care Centers

Interstitial lung diseases (ILD) are often associated with pulmonary hypertension (PH). This study aimed to evaluate the therapeutic benefit of phosphodiesterase-5 (PDE-5) inhibitors in pulmonary hypertension secondary to ILD.. Patients with ILD and PH were treated with sildenafil or tadalafil. Right heart catheterization was performed before and after a minimum of 3-month treatment. In addition, lung function, 6-min walk distance (6MWD) and plasma brain natriuretic peptide (BNP) concentration were assessed.. Ten ILD patients (three female, mean age 64.4 ± 9.0 years, six with idiopathic pulmonary fibrosis (IPF), four with hypersensitivity pneumonitis, (HP)) with significant precapillary PH (mean pulmonary artery pressure (PAPm) ≥ 25 mmHg, pulmonary vascular resistance (PVR) > 280 dyn*s*cm(-5) ; pulmonary artery wedge pressure (PAWPm) ≤ 15 mmHg) were treated with either sildenafil (n = 5) or tadalafil (n = 5). Pulmonary haemodynamics were severely impaired at baseline (PAPm 42.9 ± 5.4 mmHg; cardiac index (CI) 2.7 ± 0.6 L/min/m2; PVR 519 ± 131 dyn × sec × cm(-5)). After mean follow-up of 6.9 ± 5.8 months an increase in CI (2.9 ± 0.7 L/min/m2 , P = 0.04) and a decrease in PVR (403 ± 190 dyn × sec × cm(-5) , P = 0.03) were observed. 6MWD and BNP did not change significantly.. Our data suggest that treatment with PDE-5 inhibitors improves pulmonary haemodynamic patients with PH secondary to ILD.

Topics: Aged; Aged, 80 and over; Carbolines; Female; Follow-Up Studies; Hemodynamics; Humans; Hypertension, Pulmonary; Lung Diseases, Interstitial; Male; Middle Aged; Natriuretic Peptide, Brain; Phosphodiesterase 5 Inhibitors; Pilot Projects; Piperazines; Purines; Respiratory Function Tests; Sildenafil Citrate; Sulfonamides; Tadalafil; Walking

Topics: Acute Disease; Atrial Fibrillation; Cardiomegaly; Diabetes Mellitus, Type 2; Diuretics; Heart Failure; Hemorrhage; Humans; Hydrostatic Pressure; Hypertension; Leukocytosis; Lung Diseases; Lung Diseases, Interstitial; Male; Middle Aged; Natriuretic Peptide, Brain; Pulmonary Edema; Sleep Apnea Syndromes; Smoking; Tomography, X-Ray Computed; Ultrasonography

Pulmonary hypertension (PH) is an important complication to interstitial lung disease (ILD). The aim of the present study was to investigate the relation of NT-proBNP, fibrin D-dimer, troponin-T, uric acid and exhaled nitric oxide (NO) to the presence of PH and mortality in ILD.. In a previously described cohort of 212 ILD patients of whom 29 had PH, levels of the above mentioned biomarkers were analyzed as routine tests.. A value of NT-proBNP below 95 ng/l had a negative predictive value for PH of 99% (95% CI: 94-100). Values of troponin-T were higher in patients with PH (median (inter quartile range) = 9 (9-20) vs. 9(9-10) ng/l), and the odds ratio (OR) for PH was increased in patients with abnormal levels of uric acid (OR (95% CI) = 3.1(1.1-8.8)). NT-proBNP and troponin-T values above the 50(th) percentile, and uric acid and fibrin D-dimer values above the 90th percentile were each associated with increased mortality.. A value of NT-proBNP below 95 ng/l may be used as a rule-out test for PH in ILD, while an abnormal value of uric acid is a risk factor for PH. NT-proBNP, troponin-T, uric acid and fibrin D-dimer have prognostic value in ILD patients, while exhaled levels of NO do not seem to predict PH or mortality.

Topics: Biomarkers; Cross-Sectional Studies; Fibrin Fibrinogen Degradation Products; Humans; Hypertension, Pulmonary; Lung Diseases, Interstitial; Natriuretic Peptide, Brain; Nitric Oxide; Peptide Fragments; Prognosis; Pulmonary Fibrosis; ROC Curve; Troponin T; Uric Acid

Topics: Aged; Amyloidosis; Antibodies, Antinuclear; Antirheumatic Agents; Arthritis, Rheumatoid; Benzimidazoles; Biphenyl Compounds; Carbazoles; Carvedilol; Drug Therapy, Combination; Dyspnea; Etanercept; Female; Furosemide; Glucocorticoids; Hand; Heart Failure; Humans; Immunoglobulin G; Lung Diseases, Interstitial; Natriuretic Peptide, Brain; Propanolamines; Radiography; Receptors, Tumor Necrosis Factor; Rheumatoid Factor; Ribonucleosides; Scleroderma, Systemic; Spironolactone; Sulfasalazine; Tetrazoles; Treatment Outcome

Elevated pulmonary vascular resistance portends a poor prognosis across interstitial lung disease (ILD), irrespective of the histospecific diagnosis. Currently, no noninvasive surrogate prognostic marker exists. We explore the prognostic value of brain natriuretic peptide (BNP) and echocardiography across ILD. ILD patients with BNP concentrations performed during 2005-2007 were reviewed (n = 90). Echocardiography tapes were reviewed by a cardiologist blinded to other results. Outcome was evaluated for survival against BNP and echocardiograph parameters. A priori threshold values and composite markers were evaluated against survival. During follow-up (20±9 months) there were 28 deaths (31%). BNP correlated with right heart echocardiographic indices, including right ventricular systolic pressure (RVSP) (R(2) = 0.18, p = 0.0002) but not with parameters of left heart function. Nonsurvivors had higher BNP and RVSP levels than survivors. BNP ≥20 pmol·L(-1) (hazard ratio (HR) 2.93, 95% CI 1.28-6.73; p = 0.01) and moderate-severe pulmonary hypertension (HR 2.53, 95% CI 1.15-5.57; p = 0.02) were associated with increased mortality, independent of age, sex and pulmonary function. Patients with BNP ≥20 pmol·L(-1) had a 14-fold increased mortality over those with BNP <4 pmol·L(-1). Increased BNP levels and/or echocardiographic markers of right ventricular dysfunction were associated with increased mortality across ILD. The link between vascular parameters and mortality supports the concept that pulmonary vascular disease contributes to the final common pathway seen across ILD.

Topics: Aged; Echocardiography; Female; Follow-Up Studies; Humans; Hypertension, Pulmonary; Lung Diseases, Interstitial; Male; Middle Aged; Natriuretic Peptide, Brain; Prognosis; Regression Analysis; Treatment Outcome; Ventricular Dysfunction, Right

Limited data suggest a benefit following sildenafil treatment in patients with pulmonary hypertension (PH) and interstitial lung disease (ILD). The role of sildenafil in the management of PH in ILD is not clear. We report our experience of ILD patients with PH after 6-month sildenafil therapy.. We reviewed 15 patients (mean age 55 ± 15 years; 8 men) with ILD (mean FVC 52.6 ± 15.4%) and PH (mean right ventricular systolic pressure 73.8 ± 17.8 mm Hg). Median brain natriuretic peptide: 37 (5-452) pmol/L; mean 6MWD: 156 ± 101 m.. Following 6-month treatment with sildenafil, brain natriuretic peptide levels were lower (n = 12, P = 0.03), 6MWD was higher (n = 6, P < 0.05), but no change in right ventricular systolic pressure (n = 11) was demonstrated.. Our observations suggest that sildenafil may be useful in the management of PH in ILD. Controlled trials are warranted before therapeutic recommendations can be made.

Topics: Cohort Studies; Exercise Test; Female; Humans; Hypertension, Pulmonary; Lung Diseases, Interstitial; Male; Middle Aged; Natriuretic Peptide, Brain; Piperazines; Purines; Sildenafil Citrate; Sulfones; Treatment Outcome; Vasodilator Agents; Walking

Recent advances in the treatment of pulmonary arterial hypertension provide a rational basis for earlier, noninvasive diagnosis of pulmonary arterial hypertension. However, the reliability of transthoracic echocardiography, plasma BNP levels, and other parameters for the diagnosis of pulmonary arterial hypertension remains unclear. Thus, the purpose of this study was to determine the utility of these modes of investigation for the prediction of pulmonary arterial pressure as compared with the current gold standard, Swan-Ganz catheterization. Among 46 PAH patients, 37 had connective tissue diseases, while the remainder had primary pulmonary arterial hypertension, chronic pulmonary thromboembolism, and interstitial pneumonitis. Systolic pulmonary arterial pressure calculated by transthoracic echocardiography was significantly correlated with systolic pulmonary arterial pressure measured using a Swan-Ganz catheter (r = 0.51, P < 0.01). Plasma BNP concentration did not correlate with systolic pulmonary arterial pressure (r = 0.10, NS) in the overall patient population. However, when we excluded left ventricular heart failure and left ventricular hypertrophy, BNP concentration was correlated with systolic pulmonary arterial pressure (r = 0.508, P < 0.05). Among other variables tested, ECG electrical axis was correlated with pulmonary arterial pressure (r = 0.46, P < 0.05) but uric acid, lactate dehydrogenase, %DLCO, enhanced IIp sound, and pulmonary artery enlargement on chest x-ray did not correlate with pulmonary arterial pressure. These data suggest that echocardiography is the noninvasive modality of choice for the assessment of pulmonary arterial hypertension. Plasma BNP level also predicts pulmonary arterial pressure, when left ventricular heart failure and cardiac hypertrophy are excluded.

Topics: Aged; Catheterization, Swan-Ganz; Connective Tissue Diseases; Electrocardiography; Female; Humans; Hypertension, Pulmonary; Lung Diseases, Interstitial; Male; Middle Aged; Natriuretic Peptide, Brain; Predictive Value of Tests; Pulmonary Embolism; Retrospective Studies; Ultrasonography

To study a plasmic concentration of the brain natriuretic peptide (BNUP) in patients with interstitial pulmonary disease (IPD) as a possible diagnostic parameter in pulmonary hypertension (PH).. Plasmic BNUP concentration, external respiration function were studied in 24 patients with IPD. The following tests were also made: 6-min walk, echocardiography (echo-CG) with estimation of systolic pressure in the pulmonary artery, multislice computed tomography of the chest with measurement of the mean diameter of the pulmonary artery trunk.. As shown by echo-CG and chest MSCT half of the IPD patients had PH. IPD patients with PH had a significant elevation of plasmic BNUP concentration which, in intact left ventricular contractile function indicates development of secondary PH characterized by reduction of volume parameters of the respiratory pulmonary function. The rise of BNUP concentration correlated with reduction of 6-min walk distance and functional lung capacity.. Plasmic BNUP can be used for diagnosis of PH in IPD patients.

Topics: Blood Pressure; Echocardiography; Exercise Test; Female; Humans; Hypertension, Pulmonary; Lung Diseases, Interstitial; Male; Middle Aged; Natriuretic Peptide, Brain; Pulmonary Artery

To study the plasma concentration of brain natriuretic peptide (BNP) and endothelin-1 (ET-1) as markers of pulmonary hypertension (PH) developed in interstitial lung diseases (ILD).. Along with physical examination, 97 patients with ILD underwent measurements of the plasma concentrations of BNP and ET-1, 6-minute walk test, external respiration function test, echocardiography, by measuring pulmonary artery systolic pressure (P(syst)), and chest multispiral computed tomography, by estimating the mean diameter of the pulmonary artery trunk.. The plasma concentration of ET-1 proved to be significantly higher in the presence of active lung lesion (5.2 +/- 3.9 and 2.8 +/- 1.8 pg/ml; p = 0.0001). In patients with ILD, persistent PH was associated with the significantly elevated plasma concentrations of BNP (69.3 +/- 341.35 and 23.7 +/- 26.69 pg/ml; p = 0.018). The increase of plasma BNP concentrations correlated with the shorter distance covered during a 6-minute walk test and diminished functional vital capacity.. The increased plasma levels of ET-1 in ILD reflects the transient pulmonary artery pressure elevation associated with the activity of the pulmonary process while those of BNP are indicative of developed persistent PH.

Topics: Biomarkers; Case-Control Studies; Data Interpretation, Statistical; Endothelin-1; Female; Humans; Hypertension, Pulmonary; Lung Diseases, Interstitial; Male; Middle Aged; Natriuretic Peptide, Brain; Severity of Illness Index

We measured plasma BNP and hANP levels and studied the relationship to echocardiographic findings in 49 patients who visited or admitted in our hospital due to dyspnea (25 with chronic obstructive pulmonary disease (COPD), 8 with interstitial pneumonia, 16 with sequelae of tuberculosis). The age in the interstitial pneumonia group was lower and the arterial blood partial pressure of CO2 was higher in the sequelae of tuberculosis group. Plasma BNP and hANP levels were similar in the three groups. Plasma BNP level did not correlate with the pressure gradient measured from tricuspid valve regurgitation. Some patients had pulmonary hypertension despite having low plasma BNP levels. Hypoxemia was more severe in the higher BNP level group than in the lower BNP level group. Plasma BNP levels did not reflect pulmonary hypertension unless these was left ventricular failure or valvular diseases in these groups.

Topics: Aged; Aged, 80 and over; Atrial Natriuretic Factor; Female; Humans; Hypertension, Pulmonary; Lung Diseases, Interstitial; Male; Middle Aged; Natriuretic Peptide, Brain; Pulmonary Disease, Chronic Obstructive; Respiration Disorders

To evaluate the longitudinal development of the tricuspid gradient (TG) for screening for pulmonary arterial hypertension (PAH) in systemic sclerosis (SSc).. Doppler echocardiography was performed 506 times in order to estimate TG in 227 consecutive patients with SSc. The value of biochemical markers for predicting TG levels and development was assessed through analyses of pro-brain natriuretic peptide (proBNP), calcitonin-gene related peptide, thrombomodulin and von Willebrand factor in 76 patients with a borderline increase in TG, defined as TG 24-38 mmHg, and for the purpose of comparison also in 10 patients with a normal TG (< 23 mmHg) and in 10 patients with increased TG (TG > 38 mmHg).. TG > 23 mmHg was found in 102 patients (44.9%) at the first assessment point and in 139 patients (61.2%) respectively, cumulatively at follow-up. TG values > 33 mmHg were measured in 24 patients (10.6%) initially and in 38 patients (16.7%) cumulatively in a subsequent assessment. Age and the presence of interstitial lung disease (ILD) were associated with more frequent occurrence of TG > 23 and > 33 mmHg initially and at follow-up, but were not associated with progression rate. The change in TG (mean +/- S.D.) was 1.34 +/- 4.55 mmHg/yr. ProBNP correlated to TG.. An increased TG, indicating possible PAH, is common and progressive in SSc. Age and ILD increase the risk of increased TG. Patients with or without ILD have similar progression of TG. ProBNP has potential as an adjunct to TG in selecting patients eligible for invasive treatment.

Topics: Age Factors; Blood Pressure; Calcitonin Gene-Related Peptide; Carbon Monoxide; Echocardiography, Doppler; Humans; Hypertension, Pulmonary; Lung Diseases, Interstitial; Middle Aged; Natriuretic Peptide, Brain; Scleroderma, Systemic; Thrombomodulin; Tricuspid Valve; Tricuspid Valve Insufficiency; von Willebrand Factor