natriuretic-peptide--brain and Lupus-Erythematosus--Systemic

This study aims to evaluate the survival of patients with systemic lupus erythematosus (SLE)-associated pulmonary arterial hypertension (PAH) by a systematic review and meta-analysis.. Studies were searched from MEDLINE (OVID), EMBASE, Cochrane Central Register of Controlled Trial and Scopus databases, and were selected according to the inclusion and exclusion criteria. Two independent reviewers extracted data from selected studies. Quality assessments were also performed using the Newcastle-Ottawa Scale. All pooled analyses were conducted both for random-effects model and fixed-effects model. Subgroup analysis and sensitivity analysis were conducted to investigate the origins of heterogeneity. Publication bias was evaluated using Begg's funnel plots and Egger's test.. Six studies encompassing 323 patients with SLE-associated PAH were included in the meta-analysis. The pooled 1-, 3- and 5-year survival rates were 88% (95% CI, 0.80-0.93), 81% (95% CI, 0.67-0.90) and 68% (95% CI, 0.52-0.80), respectively. No significant publication bias was shown. WHO Functional class (Fc) III/IV was found to be an independent prognostic factor of mortality. Higher mean pulmonary arterial pressure (mPAP), higher pulmonary vascular resistance (PVR), lower six minutes walking distance (6MWD), higher brain natriuretic peptide (BNP) and higher N-terminal proBNP (NT-proBNP) level were also related to poor survival.. The long-term survival of patients with SLE-associated PAH is poor, which is worth paying greater clinical and academic attention. This study suggested that early diagnosis and management are recommended in patients with SLE-associated PAH for a better outcome of survival.

Topics: Early Diagnosis; Humans; Hypertension, Pulmonary; Lupus Erythematosus, Systemic; Natriuretic Peptide, Brain; Peptide Fragments; Prognosis

In patients with systemic lupus erythematosus (SLE), myocardial involvement is the third leading course of death after lupus nephropathy (LN) and infections. Previous autopsy studies have demonstrated a high incidence of cardiovascular abnormalities in the myocardium. However, the patients with typical symptoms are far much fewer than expected from post-mortem examinations.. The current study aimed to evaluate the technetium-99m-sestamibi (. Thirty patients were studied with rest myocardial perfusion imaging, and summed rest score (SRS), summed motion score (SMS), and summed thickening score (STS) were calculated automatically. Biomarkers, including N-terminal prohormone of brain natriuretic peptide (NT-proBNP) and creatine-kinase-MB (CK-MB), were detected simultaneously. GMPI parameters, LV functions and biomarkers were compared between two NT-proBNP groups. The relationships between these parameters were studied by correlation analysis.. SMS, STS, and glomerular filtration rate (eGFR) were the main influencing factors of NTproBNP level (p = 0.001, <0.001, 0.042, respectively). Thirteen patients with an evaluated concentration of NT-proBNP had the lower left ventricular ejection fraction (LVEF), peak filling rate (PFR), eGFR and higher levels of CK-MB (in all comparisons, p < 0.05), and SRS was the only influencing factor of NT-proBNP (p = 0.007). Within thirteen patients with SRS≥2, there was a significant correlation between SRS and NT-proBNP (p < 0.001).

Topics: Biomarkers; Correlation of Data; Humans; Lupus Erythematosus, Systemic; Myocardium; Natriuretic Peptide, Brain; Perfusion; Stroke Volume; Technetium Tc 99m Sestamibi; Tomography, Emission-Computed, Single-Photon; Ventricular Function, Left

Ventricular thrombus is an uncommon, severe condition with high morbidity and mortality. Simultaneous left and right ventricular thrombi caused by lupus myocardiopathy have not been previously reported in the literature. This case presents a 42-year-old woman who has bilateral ventricular thrombi with reduced left ventricular ejection fraction (35.4%) and acute kidney injury. Pro-brain natriuretic peptide was >35000 pg/mL. Systemic lupus erythematosus was confirmed based on multiorgan injuries including malar rash, anemia, renal injury, positive antinuclear, anti-Smith antibodies, and decreased complements. Renal biopsy revealed lupus nephritis class III + V. Low molecular weight heparin, steroids, and mycophenolate mofetil were initiated, after which the patient experienced transient numbness in the right limbs and hemoptysis. She then recovered quickly and improved significantly with recovery of left ventricular systolic function (left ventricular ejection fraction 46%) and the eventual disappearance of thrombi. Simultaneous left and right ventricular thrombi are rare but life-threatening condition, prompting consideration of myocardiopathy caused by autoimmune diseases such as lupus. Timely treatment with immunosuppressants and anticoagulants may resolve the thrombi and improve cardiac function.

Topics: Acute Kidney Injury; Adult; Anticoagulants; Biopsy; Cardiomyopathies; Drug Therapy, Combination; Female; Heart Ventricles; Humans; Immunosuppressive Agents; Lupus Erythematosus, Systemic; Lupus Nephritis; Natriuretic Peptide, Brain; Peptide Fragments; Stroke Volume; Thrombosis; Treatment Outcome; Ventricular Dysfunction, Left

Cardiac involvement in systemic lupus erythematosus (SLE) is often undiagnosed in its early phases. Specific heart biomarkers may identify patients at risk. We sought to investigate the prevalence and associated factors for such biomarkers in SLE.. Brain natriuretic peptide (BNP) and cardiac troponin I (cTnI) were measured simultaneously in 151 consecutive patients with no history of heart disease or pulmonary arterial hypertension (PAH). None had electrocardiographic abnormalities suggestive of acute coronary syndrome. Cross-sectional comparisons and logistic regression analyses were performed. Patients with abnormal biomarkers were investigated to delineate the specific cause.. Sixteen patients (16/151, 10.6%) had elevated BNP, and 9 of them also had abnormal cTnI. Compared to subjects with normal biomarkers, they were older, had longer disease and antimalarial (AM) use duration, and more frequently persistent creatine phosphokinase (CPK) elevation. Multivariable regression analysis showed prolonged AM treatment (> 5.6 yrs) and persistent CPK elevation to be important predictors for elevated cardiac biomarkers. Six patients were diagnosed with definite (based on endomyocardial biopsy, n = 2) or possible (based on cardiac magnetic resonance after exclusion of other causes) AM-induced cardiomyopathy (AMIC); all had both BNP and cTnI elevated. Alternative causes were identified in 5, while no definitive diagnosis could be made in the remaining patients.. About 10% of patients with SLE had elevated myocardial biomarkers, in the absence of prior cardiac disease or PAH. One-third of them were diagnosed with AMIC. Prolonged AM therapy and persistent CPK elevation conferred an increased risk for abnormal BNP and cTnI, which might predict AMIC.

Topics: Adult; Aged; Antimalarials; Biomarkers; Cardiomyopathies; Creatine Kinase; Cross-Sectional Studies; Female; Humans; Lupus Erythematosus, Systemic; Malaria; Male; Middle Aged; Natriuretic Peptide, Brain; Risk Factors; Troponin I

Antimalarials (AM) are recommended for all systemic lupus erythematosus (SLE) patients without specific contraindications. Their main adverse effect is retinal damage; however, heart disease has been described in isolated cases. The aim of our study is to describe 8 patients with AM-induced cardiomyopathy (AMIC) in a defined SLE cohort.. Patients attending the Toronto Lupus Clinic and diagnosed with definite (based on endomyocardial biopsy; EMB) and possible AMIC were included [based on cardiac magnetic resonance imaging (cMRI) and other investigations].. Eight female patients (median age 62.5 yrs, disease duration 35 yrs, AM use duration 22 yrs) were diagnosed with AMIC in the past 2 years. Diagnosis was based on EMB in 3 (extensive cardiomyocyte vacuolation, intracytoplasmic myelinoid, and curvilinear bodies). In 4 patients, cMRI was highly suggestive of AMIC (ventricular hypertrophy and/or atrial enlargement and late gadolinium enhancement in a nonvascular pattern). Another patient was diagnosed with complete atrioventricular block, left ventricular and septal hypertrophy, along with concomitant ocular toxicity. All patients had abnormal cardiac troponin I (cTnI) and brain natriuretic peptide (BNP), whereas 7/8 also had chronically elevated creatine phosphokinase. During followup, 1 patient died from refractory heart failure. In the remaining patients, hypertrophy regression and a steady decrease of heart biomarkers were observed after AM cessation.. Once considered extremely rare, AMIC seems to be underrecognized, probably because of the false attribution of heart failure or hypertrophy to other causes. Certain biomarkers (cTnI, BNP) and imaging findings may lead to early diagnosis and enhance survival.

Topics: Aged; Antimalarials; Biomarkers; Biopsy; Canada; Cardiomyopathies; Female; Follow-Up Studies; Humans; Lupus Erythematosus, Systemic; Magnetic Resonance Imaging; Middle Aged; Natriuretic Peptide, Brain; Rare Diseases; Troponin I; Withholding Treatment

This study intended to explore the relation between heart rate recovery at 1 minutes (HRR1) during the recovery phase of cardiopulmonary exercise test (CPET) and exercise capacity in female systemic lupus erythematosus associated pulmonary arterial hypertension (SLE-PAH) patients.. Twenty-one female SLE-PAH patients underwent right heart catheterization (RHC), pulmonary function test (PFT) and CPET. Forty-two healthy subjects matched with SLE-PAH patients in age, sex and BMI were recruited as a control group. The correlations between HRR1 with clinical and CPET parameters were performed.. Peak HR, ΔHR, HRR1, Peak HR-warm HR. HRR1 is an easily obtained auxiliary parameter in SLE-PAH patients to reflect an altered autonomic tone. SLE-PAH patients with HRR1 < 16 have more severe hemodynamics, worse clinical findings and marked oxygen uptake inefficiency than those with HRR1 ≥ 16.

Topics: Adult; Cardiac Catheterization; Exercise Test; Female; Heart Rate; Hemodynamics; Humans; Lupus Erythematosus, Systemic; Middle Aged; Natriuretic Peptide, Brain; Oxygen Consumption; Peptide Fragments; Pulmonary Arterial Hypertension; Respiratory Function Tests; Retrospective Studies; Walk Test

Pulmonary arterial hypertension (PAH) prevalence has been reported to be between 0.5% and 17% in systemic lupus erythematosus (SLE). This study assessed PAH prevalence and predictors in an SLE cohort.. The Borg dyspnea scale, DLCO, N-terminal pro-brain natriuretic peptide (NT-proBNP), and Doppler echocardiographic (DE) were performed. An echocardiographic Doppler exercise test was conducted in selected patients. When DE systolic pulmonary arterial pressure was ≥ 45 mmHg or increased during exercise > 20 mmHg, a right heart catheterization was performed. Hemodynamic during exercise was measured if rest mean pulmonary arterial pressure was < 25 mmHg.. Of the 203 patients with SLE, 152 were included. The mean age was 44.9 ± 12.3 years, and 94% were women. Three patients had known PAH. The algorithm diagnosed 1 patient with chronic thromboembolic pulmonary hypertension and 5 with exercise-induced pulmonary artery pressure increase (4 with occult left diastolic dysfunction). These patients had significantly more dyspnea, higher NT-proBNP, and lower DLCO.. These data confirm the low prevalence of PAH in SLE. In our cohort, occult left ventricular diastolic dysfunction was a frequent diagnosis of unexplained dyspnea. Dyspnea, DLCO, and NT-proBNP could be predictors of pulmonary hypertension in patients with SLE.

Topics: Adult; Echocardiography, Doppler; Exercise Test; Female; Humans; Hypertension, Pulmonary; Lupus Erythematosus, Systemic; Male; Middle Aged; Natriuretic Peptide, Brain; Peptide Fragments; Prevalence; Risk Factors

To estimate the point prevalence of pulmonary hypertension (PH) and determine the associated factors for PH in patients with systemic lupus erythematosus (SLE).. A prospective cross-sectional study of 114 patients with SLE was conducted in a single tertiary center. Transthoracic echocardiography was performed to estimate the pulmonary arterial pressures. PH was defined as resting systolic pulmonary artery pressure (sPAP) ≥ 40 mmHg, in the absence of left heart disease.. PH was identified in nine patients (7.9%) who had few cardiopulmonary symptoms. SLE patients with PH had higher SLE disease activity index score. In particular, serum uric acid (UA) was significantly higher in patients with PH than in those without PH. In multivariate analysis, UA remained significant for the presence of PH. Moreover, serum UA level correlated significantly with plasma NT-pro-B-type natriuretic peptide level as well as sPAP. At the cutoff level of 6.5 mg/dL, serum UA had reasonable accuracy for predicting the presence of PH in SLE patients (sensitivity 66.7% and specificity 96.2%).. A significant number of SLE patients in rheumatology practice have undiagnosed PH with few discernible symptoms. Serum UA level may be useful as a surrogate marker for screening of PH in patients with SLE.

Topics: Adult; Biomarkers; Blood Pressure; Comorbidity; Cross-Sectional Studies; Echocardiography; Female; Humans; Hypertension, Pulmonary; Lupus Erythematosus, Systemic; Mass Screening; Natriuretic Peptide, Brain; Peptide Fragments; Prevalence; Prospective Studies; Sensitivity and Specificity; Uric Acid

Cardiac manifestations of neonatal lupus (cardiac NL) include congenital heart block and cardiomyopathy. Several candidate biomarkers were evaluated in cases at risk for cardiac NL on the basis of potential roles in inflammation, fibrosis, and cardiac dysfunction: C-reactive protein (CRP); NT-pro-B-type natriuretic peptide (NT-proBNP); troponin I; matrix metalloproteinase (MMP)-2; urokinase plasminogen activator (uPA); urokinase plasminogen activator receptor (uPAR); plasminogen; and vitamin D.. Identification of maternal and fetal biomarkers associated with development and morbidity of cardiac NL should provide clues to pathogenesis with translational implications for management.. Cord (139) and maternal (135) blood samples collected during pregnancies at risk for cardiac NL were available for study. Levels of cord and maternal CRP, cord NT-proBNP, and cord troponin I were evaluated using multiplex assays. Cord and maternal vitamin D were assessed by liquid chromatography-mass spectrometry. MMP-2, uPA, uPAR, and plasminogen were evaluated using ELISA.. Cord CRP, NT-proBNP, MMP-2, uPA, uPAR, and plasminogen levels were higher in cardiac NL-affected fetuses than in unaffected cases, independent of maternal rheumatic disease, season at highest risk of cardiac NL development, and medications taken during pregnancy. These biomarkers were positively associated with a disease severity score derived from known risk factors for mortality in cardiac NL. Maternal CRP and cord troponin I levels did not differ between the groups. Cord and maternal vitamin D levels were not significantly associated with cardiac NL, but average maternal vitamin D level during pregnancy was positively associated with longer time to postnatal pacemaker placement.. These data support the association of fetal reactive inflammatory and fibrotic components with development and morbidity of cardiac NL. Following CRP and NT-proBNP levels after birth can potentially monitor severity and progression of cardiac NL. MMP-2 and the uPA/uPAR/plasminogen cascade provide therapeutic targets to decrease fibrosis. Although decreased vitamin D did not confer increased risk, given the positive influence on postnatal outcomes, maternal levels should be optimized.

Topics: Antibodies, Antinuclear; Biomarkers; C-Reactive Protein; Female; Heart Diseases; Humans; Infant, Newborn; Lupus Erythematosus, Systemic; Male; Matrix Metalloproteinase 2; Natriuretic Peptide, Brain; Peptide Fragments; Pregnancy; Receptors, Urokinase Plasminogen Activator; Troponin I; Vitamin D

A previously healthy young man presented with a 12-hour history of sudden dyspnea and severe chest pain at rest. Initial findings of physical examination, electrocardiogram and chest radiography showed typical pericarditis and clinical instability. Echocardiogram revealed small pericardial effusion with right ventricle dilatation. The patient was admitted in the ICU; a new echocardiogram revealed moderate pericardial effusion and diagnosis of pericarditis complicated with acute cardiac tamponade was established. The patient transiently improved after pericardial window. In the following hours, the diagnosis of myocarditis with predominantly right ventricular involvement (MPRVI) with severe right heart failure was supported by clinical, chest radiography and echocardiogram data, despite normal B-type natriuretic peptide. On day 2, cardiac troponin I detection was observed. By day 3, B-type natriuretic peptide in the range of ventricular dysfunction was identified. Cardiovascular magnetic resonance findings supported the diagnosis of MPRVI. A systematic MEDLINE/PubMed from 1993 to 2013 does not identify any cases of MPRVI related to systemic lupus erythematosus. Simultaneous acute MPRVI with normal B-type natriuretic peptide and acute cardiac tamponade heralding the diagnosis of systemic lupus erythematosus, to the best of our knowledge, has not been previously described.

Topics: Adult; Cardiac Tamponade; Heart Ventricles; Humans; Lupus Erythematosus, Systemic; Male; Myocarditis; Natriuretic Peptide, Brain; Pericarditis

Topics: Adult; Atherosclerosis; Biomarkers; Disability Evaluation; Female; Heart Failure; Humans; Longitudinal Studies; Lupus Erythematosus, Systemic; Male; Natriuretic Peptide, Brain; Peptide Fragments; Severity of Illness Index; Ventricular Dysfunction

The REgistry of Pulmonary Hypertension Associated with Rheumatic Disease (REOPARD) was established in Korea. The baseline data are described from the second year of the registry's operation.. Patients with a connective tissue disease (CTD) who met the modified definition of the WHO group I pulmonary arterial hypertension (PAH) were enrolled. PAH was defined as a systolic pulmonary arterial pressure> 40 mmHg by echocardiography or mean pulmonary arterial pressure> 25 mmHg by right heart catheterization. Hemodynamic parameters and clinical data such as demographics, functional class, underlying disease, organ involvement, laboratory tests and current treatment were recorded.. A total of 321 patients were enrolled during the 2-year study period from 2008 to 2010. The mean age of the patients at registration was 51.9 years and 87.5% were female. Most patients were diagnosed by echocardiography and only 24 patients (7.5%) underwent cardiac catheterization. Exertional dyspnea was present in 63.6% of patients and 31.8% were New York Heart Association class III or IV. Among the patients, systemic lupus erythematosus accounted for 35.3%, systemic sclerosis 28.3%, rheumatoid arthritis 7.8%, overlap syndrome 9.0%, and mixed connective tissue disease 5.9%. There were no significant differences in hemodynamics, functional class, diffusing capacity and N-terminal pro-brain natriuretic peptide levels between the disease subgroups. Treatments consisted of calcium antagonists (57.0%), endothelin antagonists (32.7%), prostanoids (27.1%), phosphodiesterase-5 inhibitors (14.3%) and combinations (37.4%).. Compared with previous studies, the results showed some differences: underlying diseases, functional status and treatments. This may be due to differences in ethnic background and diagnostic methods of our study.

Topics: Adult; Aged; Arthritis, Rheumatoid; Data Collection; Female; Hemodynamics; Humans; Hypertension, Pulmonary; Lupus Erythematosus, Systemic; Male; Middle Aged; Mixed Connective Tissue Disease; Natriuretic Peptide, Brain; Peptide Fragments; Pulmonary Diffusing Capacity; Registries; Republic of Korea; Retrospective Studies; Rheumatic Diseases; Scleroderma, Systemic

We identified IgG reactive with human cardiac troponin-I (cTnI) in plasma and serum samples (N = 1930) from normal blood donors, and in sample cohorts characterized on the basis of clinical biomarkers associated with cardiac, infectious, and autoimmune diseases. cTnI and brain natriuretic peptide were the biomarkers chosen to reflect myocyte damage or left ventricular dysfunction, respectively. The infectious disease cohorts were serologically positive for antibodies to hepatitis B (natural infection), hepatitis C virus, and Chagas (i.e., T.cruzi). The autoimmune cohorts were represented by samples from diagnosed systemic lupus erythematosus (biomarker: dsDNA) and rheumatoid arthritis (biomarker: rheumatoid factor) subjects. The prevalence of IgG autoantibodies reactive with cTnI was high in the normal donor cohort (95/750, 12.7%). The prevalence in the other sample cohorts was not significantly different from that in the normal blood donors, with the exception of a slight increase in the rheumatoid factor cohort (28/137, 20.4%). The presence of anti-cTnI IgG in highly reactive samples was confirmed by inhibition with the antigen and further by screening with a library of peptides derived from the human cTnI amino acid sequence. Our data suggest that these autoantibodies are polyspecific, encompassing epitopes across the entire cTnI sequence, including the cardiac-specific amino terminal region.

Topics: Adolescent; Adult; Aged; Aged, 80 and over; Amino Acid Sequence; Antibodies, Viral; Arthritis, Rheumatoid; Autoantibodies; Chagas Disease; Child; Cohort Studies; Female; Hepatitis B; Hepatitis C; Humans; Immunoglobulin G; Luminescent Measurements; Lupus Erythematosus, Systemic; Male; Middle Aged; Natriuretic Peptide, Brain; Reproducibility of Results; Troponin I; Young Adult

Cardiovascular mortality is increased in systemic lupus erythematosus (SLE). Increased plasma concentrations of N-terminal pro-brain natriuretic peptide (NT-proBNP) are associated with cardiovascular morbidity and mortality in the general population. We examined the hypothesis that NT-proBNP concentrations are higher in patients with SLE, and are related to inflammation, augmentation index, coronary atherosclerosis, and cardiovascular risk factors.. Serum concentrations of NT-proBNP were measured in 113 patients with SLE and in 80 control subjects. Coronary calcification and augmentation index were measured by electron beam computed tomography and noninvasive pulse wave analysis, respectively.. Patients with SLE had higher concentrations of NT-proBNP [median 38.6 (interquartile range 2.5-126.9) pg/ml] than controls [11.7 (1.6-47.9) pg/ml] (p = 0.002). Augmentation index was higher in patients with SLE [25.0% (20.5%-31.5%)] than controls [20.5% (12.0%-29.0%)] (p = 0.04). In patients with SLE, NT-proBNP concentrations were associated with disease damage (rho = 0.31, p < 0.001) and duration (rho = 0.21, p = 0.02) but not with disease activity, C-reactive protein, erythrocyte sedimentation rate, tumor necrosis factor-alpha, interleukin 6, coronary calcium score, or augmentation index (all p > or = 0.18).. Patients with SLE have increased concentrations of NT-proBNP, but this is not explained by atherosclerotic burden, augmentation index, or inflammatory state.

Topics: Calcinosis; Case-Control Studies; Colonography, Computed Tomographic; Coronary Artery Disease; Elasticity; Female; Humans; Lupus Erythematosus, Systemic; Male; Natriuretic Peptide, Brain; Peptide Fragments; Pulsatile Flow

Cardiovascular disease is a major cause of death in patients with systemic lupus erythematosus (SLE) especially during the late phase of the disease. This study was conducted to evaluate B-type natriuretic peptide (BNP) levels in female SLE patients without cardiac symptoms and to investigate whether BNP levels correlated with echocardiographic findings. We studied 59 women with SLE and 33 healthy women. SLE patients with history of cardiac disease, diabetes mellitus, hypertension, and other inflammatory diseases were excluded from the study. All subjects had a complete history and physical examination. Overall disease activity assessment in SLE patients at the time of the study were derived by calculation of SLE disease activity index (SLEDAI). BNP levels were determined, and transthoracic echocardiography were performed in all subjects. There was no difference between SLE patients and controls in terms of age, blood pressure, smoking status, plasma glucose, creatinine levels, and lipid profiles. Nine patients had SLEDAI score greater than 5. All subjects had an EF greater than 55%. Diastolic dysfunction was more frequent in lupus patients than in controls (15 [25.4%] vs. 2 [6%]; p = 0.022). BNP levels of SLE patients were significantly higher than controls (median 17.9 range [5-211] pg/ml vs. median 14.7 range [5-39.7] pg/ml; p = 0.033). Twenty-seven of the SLE patients (46%) and seven of the controls (21%) had BNP levels greater than or equal to 20 pg/ml (p = 0.019). There were no differences in BNP levels of SLE patients with and without diastolic dysfunction (median 17.8 range [5-117] pg/ml vs. median 18.5 range [5-211] pg/mL; p = NS). BNP levels were positively correlated with left atrium diameter (r (2) = 0.39, p = 0.001). BNP levels did not correlate with erythrocyte sedimentation rate/C-reactive protein levels, SLEDAI scores, total steroid dosage used, or other echocardigraphic parameters. BNP levels were increased in female SLE patients without cardiac symptoms as compared to healthy controls. Although none of the SLE patients in our study had clinical signs of ischemic heart disease, increased levels of BNP in SLE patients might be a reflection of a ischemic myocardial tissue.

Topics: Adult; Age Factors; Blood Pressure; Cardiovascular Diseases; Echocardiography; Female; Humans; Inflammation; Lupus Erythematosus, Systemic; Middle Aged; Models, Statistical; Natriuretic Peptide, Brain; Prognosis; Prospective Studies; Risk Factors

Sildenafil and bosentan were added recently to the treatment with great expectations, effectiveness for the acute exacerbation of pulmonary arterial hypertension (PAH) is not fully examined. Two cases of acutely exacerbated PAH associated with collagen vascular diseases were treated first with sildenafil for six months followed by bosentan for another six months and the characteristics of this treatment modality were examined. Sildenafil showed an immediate effect which started in as early as approximately 30 min and was maximized in 60-90 min after oral ingestion. Continuous use of sildenafil for six months lowered pulmonary arterial pressure, pulmonary vascular resistance and the levels of brain natriuretic peptides along with an increased distance in 6-minute-walk, and replacement of it to with bosentan kept these effects. We think it as a treatment choice to use sildenafil first as a reliever and replace it with a controller bosentan, considering the immediate effects of sildenafil.

Topics: Adult; Antihypertensive Agents; Bosentan; Drug Therapy, Combination; Exercise Tolerance; Female; Humans; Hypertension, Pulmonary; Lupus Erythematosus, Systemic; Mixed Connective Tissue Disease; Natriuretic Peptide, Brain; Piperazines; Purines; Sildenafil Citrate; Sulfonamides; Sulfones; Vasodilator Agents