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1.
Respir Res ; 22(1): 34, 2021 Feb 03.
Artigo em Inglês | MEDLINE | ID: mdl-33536021

RESUMO

BACKGROUND: The oral IP receptor agonist selexipag is approved for the long-term treatment of pulmonary arterial hypertension (PAH). Treatment interruptions should be avoided due to the progressive nature of the disease. An intravenous (IV) formulation of selexipag was developed to provide a treatment option for short-term interruptions to oral selexipag. In this prospective, multicenter, open-label study, the safety, tolerability, and pharmacokinetics of temporarily switching between oral and IV selexipag were investigated (NCT03187678, ClinicalTrials.gov). METHODS: PAH patients receiving stable oral selexipag doses were enrolled. Following three consecutive IV selexipag infusions patients resumed oral selexipag. Corresponding IV and oral doses were selected to achieve comparable exposure to the active metabolite of selexipag. Safety outcomes were monitored throughout, and pharmacokinetic samples were obtained after oral and IV administration. RESULTS: All 20 patients completed the study. Fifteen patients had adverse events (AEs), most were mild, and none resulted in discontinuation. Headache was the most common AE throughout the study (four patients). Three serious AEs occurred in two patients with underlying comorbidities when oral dosing had resumed. There were no changes in WHO functional class for any patient and no clinically symptomatic changes in blood pressure were observed. Comparable exposure to the active metabolite of selexipag was demonstrated following corresponding oral and IV selexipag doses. CONCLUSIONS: Temporarily switching between corresponding doses of oral and IV selexipag was well-tolerated with no unexpected safety findings and comparable exposure to the active metabolite. Treatment with IV selexipag is a feasible option to bridge temporary oral selexipag treatment interruptions.

2.
Chest ; 2021 Feb 03.
Artigo em Inglês | MEDLINE | ID: mdl-33548221

RESUMO

BACKGROUND: Few data are available on the long-term course and predictors of quality of life (QoL) after acute pulmonary embolism (PE). RESEARCH QUESTION: What are the kinetics and determinants of disease-specific and generic health-related QoL 3 and 12 months after acute PE? STUDY DESIGN: and methods. The FOllow-up after aCUte pulmonary emboliSm (FOCUS) study prospectively followed consecutive adult patients with objectively diagnosed PE. We considered patients who completed the Pulmonary Embolism QoL (PEmb-QoL) Questionnaire at predefined visits 3 and 12 months after PE. We studied the course of disease-specific QoL as assessed by PEmb-QoL and the impact of baseline characteristics using multivariable mixed-effects linear regression, along with the course of generic QoL as assessed by the EQ-5D-5L utility index and Visual Analogue Scale (VAS). RESULTS: In 620 patients (44% women, median age 62 years), overall disease-specific QoL improved from 3 to 12 months, with a decrease of the median PEmb-QoL score from 19.4% to 13.0% and a mean individual change of -4.3% (95% CI: -3.2% to -5.5%). Female sex, cardiopulmonary disease, and higher body-mass index were associated with worse QoL at both 3 and 12 months. Over time, the association with body-mass index became weaker, while older age and previous venous thromboembolism were associated with worsening QoL. Generic QoL also improved: the mean EQ-5D-5L utility index increased from 0.85 (standard deviation (SD) 0.22) to 0.87 (SD 0.20), the VAS from 72.9 (SD 18.8) to 74.4 (SD 19.1). INTERPRETATION: In a large cohort of survivors of acute PE, we quantified the change of QoL between months 3 and 12 after diagnosis, and identified factors independently associated with lower QoL and slower recovery of QoL. This information may facilitate the planning and interpretation of clinical trials assessing QoL and help guide patient management.

4.
Chest ; 2021 Feb 10.
Artigo em Inglês | MEDLINE | ID: mdl-33581097

RESUMO

BACKGROUND: Pulmonary hypertension in chronic obstructive pulmonary disease (PH-COPD) is a poorly investigated clinical condition. RESEARCH QUESTION: Which factors determine outcome of PH-COPD? STUDY DESIGN AND METHODS: We analyzed the characteristics and outcome of patients enrolled in the COMPERA registry with moderate or severe PH-COPD as defined during the 6th PH World Symposium who received medical therapy for PH and compared them to patients with idiopathic pulmonary arterial hypertension (IPAH). RESULTS: The population included incident patients with moderate PH-COPD (n=68), severe PH-COPD (n=307), and IPAH (n=489). PH-COPD subjects were older, predominantly male, and treated mainly with phosphodiesterase-5 inhibitors. Despite similar hemodynamic impairment, patients with PH-COPD had a worse 6-minute walking distance (6MWD) and more advanced World Health Organization functional class (WHO FC). Transplant-free survival rates at 1, 3 and 5 years were higher in the IPAH group than in the PH-COPD group (IPAH 94%, 75%, 55%, vs PH-COPD 86%, 55%, 38%; p=0.004). Risk factors for poor outcomes in PH-COPD were male sex, low 6MWD and high pulmonary vascular resistance (PVR). In patients with severe PH-COPD, improvements in 6MWD by ≥30m or improvements in WHO FC after initiation of medical therapy were associated with better outcome. INTERPRETATION: Patients with PH-COPD were functionally more impaired and had a poorer outcome than patients with IPAH. Predictors of death in the PH-COPD group were sex, 6MWD and PVR. Our data raise the hypothesis that some patients with severe PH-COPD may benefit from PH treatment. Randomized, controlled studies are necessary to further explore this hypothesis.

5.
Eur Respir J ; 2020 Dec 24.
Artigo em Inglês | MEDLINE | ID: mdl-33361099

RESUMO

OBJECTIVE: Cumulative evidence indicates that childhood maltreatment (CM) is linked to self-reported asthma and chronic obstructive pulmonary disease. However, the relation between CM and objective measures of lung function as determined by spirometry has not yet been assessed. METHODS: Medical histories and spirometric lung function were taken in 1386 adults from the general population. Participants also completed the Childhood Trauma Questionnaire for the assessment of emotional, physical and sexual abuse as well as emotional and physical neglect. RESULTS: 25.3% of the participants reported at least one type of CM. Among them, use of medication for obstructive airway diseases as well as typical signs and symptoms of airflow limitation were significantly more frequent than in the group without exposure to CM. Although participants with CM had numerically lower values for FEV1, FVC and PEF than those without, these differences were non-significant when accounting for relevant covariates like age, sex, height and smoking. Likewise, there were no differences in the FEV1/FVC ratio nor in the frequency of airflow limitation regardless of its definition. No specific type of CM was related to spirometrically determined parameters of lung function. CONCLUSIONS: Our findings call into question the association of CM with obstructive lung diseases as indicated by prior research relying on self-reported diagnoses. We consider several explanations for these discrepancies.

7.
Artigo em Inglês | MEDLINE | ID: mdl-33205518

RESUMO

Physical activity (PA) may influence cardio-respiratory fitness (CRF). Yet, PA takes place in different domains (i.e. sports related physical activity [SPA], leisure time related physical activity [LTPA] and work-related physical activity [WPA]) and not all domain-specific PA may help to maintain high CRF levels throughout life. We assessed the relationship between changes in domain specific PA and the age-related decline in CRF. We analyzed data of 353 men (median age 50 years; inter-quartile range [IQR] 40 to 60) and 335 women (median age 50 years; IQR 41 to 59) with data for domain-specific PA as well as CRF testing measured ten years apart. CRF was assessed with cardiorespiratory exercise testing. Domain specific PA was measured using the Baecke questionnaire. During the 10 year follow-up CRF decreased in men from 29.3 (IQR 25.0 to 34.7) ml/min/kg to 24.3 (IQR 20.8 to 27.3) ml/min/kg. In women CRF declined from 26.0 (IQR 21.0 to 30.9) to 21.4 (IQR 18.3 to 25.6) ml/min/kg. A one point higher SPA at baseline was related to a 1.14 (95% confidence interval [CI] -1.50 to -0.53) ml/min/kg greater decrease in VO2peak . A one point greater SPA and LTPA over time was associated with a 1.68 (95% CI 1.06 to 2.29) ml/min/kg and 1.24 (95% CI 0.57 to 1.90) ml/min/kg lower decrease in VO2peak , respectively. Neither baseline values nor changes of WPA were associated with CRF. Sports and leisure time related PA may attenuate the age related decline in CRF.

8.
J Heart Lung Transplant ; 39(12): 1435-1444, 2020 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-33082079

RESUMO

The term idiopathic pulmonary arterial hypertension (IPAH) is used to categorize patients with pre-capillary pulmonary hypertension of unknown origin. There is considerable variability in the clinical presentation of these patients. Using data from the Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension, we performed a cluster analysis of 841 patients with IPAH based on age, sex, diffusion capacity of the lung for carbon monoxide (DLCO; <45% vs ≥45% predicted), smoking status, and presence of comorbidities (obesity, hypertension, coronary heart disease, and diabetes mellitus). A hierarchical agglomerative clustering algorithm was performed using Ward's minimum variance method. The clusters were analyzed in terms of baseline characteristics; survival; and response to pulmonary arterial hypertension (PAH) therapy, expressed as changes from baseline to follow-up in functional class, 6-minute walking distance, cardiac biomarkers, and risk. Three clusters were identified: Cluster 1 (n = 106; 12.6%): median age 45 years, 76% females, no comorbidities, mostly never smokers, DLCO ≥45%; Cluster 2 (n = 301; 35.8%): median age 75 years, 98% females, frequent comorbidities, no smoking history, DLCO mostly ≥45%; and Cluster 3 (n = 434; 51.6%): median age 72 years, 72% males, frequent comorbidities, history of smoking, and low DLCO. Patients in Cluster 1 had a better response to PAH treatment than patients in the 2 other clusters. Survival over 5 years was 84.6% in Cluster 1, 59.2% in Cluster 2, and 42.2% in Cluster 3 (unadjusted p < 0.001 for comparison between all groups). The population of patients diagnosed with IPAH is heterogenous. This cluster analysis identified distinct phenotypes, which differed in clinical presentation, response to therapy, and survival.

9.
Sleep ; 2020 Oct 05.
Artigo em Inglês | MEDLINE | ID: mdl-33017007

RESUMO

Advanced brain ageing is commonly regarded as a risk factor for neurodegenerative diseases, e.g. Alzheimer's dementia, and it was suggested that sleep disorders such as obstructive sleep apnoea (OSA) are significantly contributing factors to these neurodegenerative processes. To determine the association between OSA and advanced brain ageing, we investigated the specific effect of two indices quantifying OSA, namely the apnoea-hypopnea index (AHI) and the oxygen desaturation index (ODI), on brain age, a score quantifying age-related brain patterns in 169 brain regions, using magnetic resonance imaging and overnight polysomnography data from 690 participants (48.8% women, mean age 52.5±13.4 years) of the Study of Health in Pomerania. We additionally investigated the mediating effect of subclinical inflammation parameters on these associations via a causal mediation analysis. AHI and ODI were both positively associated with brain age (AHI std. effect [95% CI]: 0.07 [0.03; 0.12], p-value: 0.002; ODI std. effect [95% CI]: 0.09 [0.04; 0.13], p-value: <0.0003). The effects remained stable in the presence of various confounders such as diabetes and were partially mediated by the white blood cell count, indicating a subclinical inflammation process. Our results reveal an association between OSA and brain age, indicating subtle but widespread age-related changes in regional brain structures, in one of the largest general population studies to date, warranting further examination of OSA in the prevention of neurodegenerative diseases.

11.
J Crit Care ; 60: 212-217, 2020 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-32871419

RESUMO

PURPOSE: Weaning from mechanical ventilation is a key component of intensive care treatment; however, this process may be prolonged as some patients require care at specialised centres. Current data indicate that weaning from invasive mechanical ventilation is successful in approximately 65% of patients; however, data on long-term survival after discharge from a weaning centre are limited. MATERIALS AND METHODS: We analysed predictors of survival among 597 patients (392 men, mean age 68 ± 11) post-discharge from a specialised German weaning centre. RESULTS: Complete weaning from mechanical ventilation was achieved in 407 (57.8%) patients, and 106 patients (15.1%) were discharged with non-invasive ventilation; thus, prolonged weaning was successful in 72.9% of the patients. The one-year and five-year survival rates post-discharge were 66.5% and 37.1%, respectively. Age, duration of mechanical ventilation, certain clusters of comorbidities, and discharged with mechanical ventilation significantly influenced survival (p < .001). Completely weaned patients who were discharged with a tracheostomy had a significantly reduced survival rate than did those who were completely weaned and discharged with a closed tracheostomy (p = .004). CONCLUSIONS: The identified predictors of survival after prolonged weaning could support therapeutic strategies during patients' intensive care unit stay. Patients should be closely monitored after discharge from a weaning centre.

12.
Pulm Circ ; 10(3): 2045894020941682, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32754309

RESUMO

Pulmonary hypertension is frequently underdiagnosed, and referral is delayed with subsequent impact on outcomes. During the SARS-CoV-2 pandemic, restrictions on daily life and changes in hospitals' daily routine care were introduced in Germany. This multi-centre study provides evidence for a negative influence of these restrictions on patient care in pulmonary hypertension expert referral centres.

13.
Br J Ophthalmol ; 2020 Aug 28.
Artigo em Inglês | MEDLINE | ID: mdl-32859720

RESUMO

AIM: To assess whether cardiorespiratory fitness (CRF) and handgrip strength, two objective markers of physical fitness, are associated with age-related macular degeneration (AMD). METHODS: We analysed cross-sectional data from the population-based Study of Health in Pomerania (2008-2012) including 1173 adult men and women aged 20-79 years. Fundus photography of the central retina was recorded with a non-mydriatic camera, and images were graded according to an established clinical AMD classification scale by an experienced reader. CRF was measured using peak oxygen uptake (peakVO2), oxygen uptake at the anaerobic threshold (VO2@AT), and maximum power output (Wmax) from standardised cardiopulmonary exercise testing on a bicycle ergometer according to a modified Jones protocol. Handgrip strength was assessed using a handheld dynamometer. Adjusted prevalence ratios (PR) for the associations of peakVO2, VO2@AT, Wmax and handgrip strength with AMD were derived from multivariable Poisson regression models. RESULTS: PeakVO2, VO2@AT, Wmax and handgrip strength were not associated with AMD. Adjusted PR for AMD associated with a 1-SD increment in peakVO2, VO2@AT, Wmax and handgrip strength were 1.05 (95% CI 0.82 to 1.34), 0.96 (95% CI 0.78 to 1.18), 1.10 (95% CI 0.86 to 1.41) and 1.01 (95% CI 0.79 to 1.30), respectively. These associations were not modified by age, sex, smoking, body mass index and diabetes. Estimates in sensitivity analysis for confounding, selection bias and missing data were similar. CONCLUSION: In our study, CRF and handgrip strength were not associated with AMD. Nevertheless, longitudinal studies with bigger sample sizes are needed to furtherly examine these associations.

14.
Pulm Circ ; 10(3)2020.
Artigo em Inglês | MEDLINE | ID: mdl-32577217

RESUMO

This prospective study compared exercise test and intravenous fluid challenge in a single right heart catheter procedure to detect latent diastolic heart failure in patients with echocardiographic heart failure with preserved ejection function. We included 49 patients (73% female) with heart failure with preserved ejection function and pulmonary artery wedge pressure ≤15 mmHg. A subgroup of 26 patients had precapillary pulmonary hypertension. Invasive haemodynamic and gas exchange parameters were measured at rest, 45° upright position, during exercise, after complete haemodynamic and respiratory recovery in lying position, and after rapid infusion of 500 mL isotonic solution. Most haemodynamic parameters increased at both exercise and intravenous fluid challenge, with the higher increase at exercise. Pulmonary vascular resistance decreased by -0.21 wood units at exercise and -0.56 wood units at intravenous fluid challenge (p = 0.3); 20% (10 of 49) of patients had an increase in pulmonary artery wedge pressure above the upper limit of 20 mmHg at exercise, and 20% above the respective limit of 18 mmHg after intravenous fluid challenge. However, only three patients exceeded the upper limit of pulmonary artery wedge pressure in both tests, i.e. seven patients only at exercise and seven other patients only after intravenous fluid challenge. In the subgroup of pulmonary hypertension patients, only two patients exceeded pulmonary artery wedge pressure limits in both tests, further five patients at exercise and four patients after intravenous fluid challenge. A sequential protocol in the same patient showed a significantly higher increase in haemodynamic parameters at exercise compared to intravenous fluid challenge. Both methods can unmask diastolic dysfunction at right heart catheter procedure, but in different patient groups.

15.
J Clin Med ; 9(5)2020 05 13.
Artigo em Inglês | MEDLINE | ID: mdl-32414075

RESUMO

INTRODUCTION: Pulmonary hypertension (PH) is a common complication in patients with congenital heart disease (CHD), aggravating the natural, post-operative, or post-interventional course of the underlying anomaly. The various CHDs differ substantially in characteristics, functionality, and clinical outcomes among each other and compared with other diseases with pulmonary hypertension. OBJECTIVE: To describe current management strategies and outcomes for adults with PH in relation to different types of CHD based on real-world data. METHODS AND RESULTS: COMPERA (Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension) is a prospective, international PH registry comprising, at the time of data analysis, >8200 patients with various forms of PH. Here, we analyzed a subgroup of 680 patients with PH due to CHD, who were included between 2007 and 2018 in 49 specialized centers for PH and/or CHD located in 11 European countries. At enrollment, the patients´ median age was 44 years (67% female), and patients had either pre-tricuspid shunts, post-tricuspid shunts, complex CHD, congenital left heart or aortic disease, or miscellaneous other types of CHD. Upon inclusion, targeted therapies for pulmonary arterial hypertension (PAH) included endothelin receptor antagonists, PDE-5 inhibitors, prostacyclin analogues, and soluble guanylate cyclase stimulators. Eighty patients with Eisenmenger syndrome were treatment-naïve. While at inclusion the primary PAH treatment for the cohort was monotherapy (70% of patients), with 30% of the patients on combination therapy, after a median observation time of 45.3 months, the number of patients on combination therapy had increased significantly, to 50%. The use of oral anticoagulants or antiplatelets was dependent on the underlying diagnosis or comorbidities. In the entire COMPERA-CHD cohort, after follow-up and receiving targeted PAH therapy (n = 511), 91 patients died over the course of a 5-year follow up. The 5-year Kaplan-Meier survival estimate for CHD associated PH was significantly better than that for idiopathic PAH (76% vs. 54%; p < 0.001). Within the CHD associated PH group, survival estimates differed particularly depending on the underlying diagnosis and treatment status. CONCLUSIONS: In COMPERA-CHD, the overall survival of patients with CHD associated PH was dependent on the underlying diagnosis and treatment status, but was significantly better as than that for idiopathic PAH. Nevertheless, overall survival of patients with PAH due to CHD was still markedly reduced compared with survival of patients with other types of CHD, despite an increasing number of patients on PAH-targeted combination therapy.

16.
Int J Mol Sci ; 21(9)2020 May 08.
Artigo em Inglês | MEDLINE | ID: mdl-32397294

RESUMO

Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare disease which is often caused by recurrent emboli. These are also frequently found in patients with myeloproliferative diseases. While myeloproliferative diseases can be caused by gene defects, the genetic predisposition to CTEPH is largely unexplored. Therefore, the objective of this study was to analyse these genes and further genes involved in pulmonary hypertension in CTEPH patients. A systematic screening was conducted for pathogenic variants using a gene panel based on next generation sequencing. CTEPH was diagnosed according to current guidelines. In this study, out of 40 CTEPH patients 4 (10%) carried pathogenic variants. One patient had a nonsense variant (c.2071A>T p.Lys691*) in the BMPR2 gene and three further patients carried the same pathogenic variant (missense variant, c.1849G>T p.Val617Phe) in the Janus kinase 2 (JAK2) gene. The latter led to a myeloproliferative disease in each patient. The prevalence of this JAK2 variant was significantly higher than expected (p < 0.0001). CTEPH patients may have a genetic predisposition more often than previously thought. The predisposition for myeloproliferative diseases could be an additional risk factor for CTEPH development. Thus, clinical screening for myeloproliferative diseases and genetic testing may be considered also for CTEPH patients.

17.
Dtsch Arztebl Int ; 117(12): 205-210, 2020 Mar 20.
Artigo em Inglês | MEDLINE | ID: mdl-32343654

RESUMO

BACKGROUND: Ever more patients are being treated with invasive ventilation in the outpatient setting. Most have no access to a structured weaning process in a specialized weaning center. The personal burden on the patients is heavy, and the costs for the health care system are high. METHODS: 61 patients who had been considered unfit for weaning were admitted to a weaning center. The primary endpoint was the number of patients who had been successfully weaned from the ventilator at six months. The comparison group consisted of health-insurance datasets derived from patients who were discharged from an acute hospital stay to receive invasive ventilation in the outpatient setting. RESULTS: 50 patients (82%; 95% confidence interval [70.5; 89.6]) were successfully weaned off of invasive ventilation in the weaning centers, 21 of them (34% [23.8; 47]) with the aid of non-invasive ventilation. The survival rate at 1 year was higher than in the group without invasive ventilation (45/50, or 90%, versus 6/11,or 55%); non-invasive ventilation was comparable in this respect to no ventilation at all. The identified risk factors for weaning failure included the presence of more than five comorbidities and a longer duration of invasive ventilation before transfer to a weaning center. CONCLUSION: If patients with prolonged weaning are cared for in a certified weaning center before being discharged to receive invasive ventilation in the outpatient setting, the number of persons being invasively ventilated outside the hospital will be reduced and the affected persons will enjoy a higher survival rate. This would also spare nursing costs.


Assuntos
Desmame do Respirador , Adulto , Idoso , Idoso de 80 Anos ou mais , Instituições de Assistência Ambulatorial , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Especialização , Falha de Tratamento
18.
Pulm Circ ; 10(1): 2045894020910136, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32206306

RESUMO

Implantable infusion pumps might improve the convenience and safety of intravenous treprostinil for pulmonary arterial hypertension. The LENUS Pro® pump (approved in Europe) has a fixed flow rate. Based on 126 pumps and 2853 refills, we retrospectively analyzed the actual flow rate from 09/2010 to 09/2018. A relevant flow rate variance is evident after three years; therefore, flow rate monitoring and dose adjustment are mandatory.

19.
Mayo Clin Proc ; 95(1): 44-56, 2020 01.
Artigo em Inglês | MEDLINE | ID: mdl-31902428

RESUMO

OBJECTIVE: To analyze the association between cardiorespiratory fitness (CRF) and global and local brain volumes. PARTICIPANTS AND METHODS: We studied 2103 adults (21-84 years old) from 2 independent population-based cohorts (Study of Health in Pomerania, examinations from June 25, 2008, through September 30, 2012). Cardiorespiratory fitness was measured using peak oxygen uptake (VO2peak), oxygen uptake at the anaerobic threshold (VO2@AT), and maximal power output from cardiopulmonary exercise testing on a bicycle ergometer. Magnetic resonance imaging brain data were analyzed by voxel-based morphometry using regression models with adjustment for age, sex, education, smoking, body weight, systolic blood pressure, glycated hemoglobin level, and intracranial volume. RESULTS: Volumetric analyses revealed associations of CRF with gray matter (GM) volume and total brain volume. After multivariable adjustment, a 1-standard deviation increase in VO2peak was related to a 5.31 cm³ (95% CI, 3.27 to 7.35 cm³) higher GM volume. Whole-brain voxel-based morphometry analyses revealed significant positive relations between CRF and local GM volumes. The VO2peak was strongly associated with GM volume of the left middle temporal gyrus (228 voxels), the right hippocampal gyrus (146 voxels), the left orbitofrontal cortex (348 voxels), and the bilateral cingulate cortex (68 and 43 voxels). CONCLUSION: Cardiorespiratory fitness was positively associated with GM volume, total brain volume, and specific GM and white matter clusters in brain areas not primarily involved in movement processing. These results, from a representative population sample, suggest that CRF might contribute to improved brain health and might, therefore, decelerate pathology-specific GM decrease.


Assuntos
Limiar Anaeróbio , Encéfalo , Aptidão Cardiorrespiratória/fisiologia , Substância Cinzenta , Adulto , Idoso , Idoso de 80 Anos ou mais , Encéfalo/diagnóstico por imagem , Encéfalo/patologia , Correlação de Dados , Teste de Esforço/métodos , Teste de Esforço/estatística & dados numéricos , Feminino , Substância Cinzenta/diagnóstico por imagem , Substância Cinzenta/patologia , Humanos , Masculino , Pessoa de Meia-Idade , Tamanho do Órgão
20.
BMC Pulm Med ; 19(1): 230, 2019 Nov 29.
Artigo em Inglês | MEDLINE | ID: mdl-31783745

RESUMO

BACKGROUND: Systemic sclerosis (SSc) is a severe rheumatic disease of the interstitial tissue, in which heart and lung involvement can lead to disease-specific mortality. Our study tests the hypothesis that in addition to established prognostic factors, cardiopulmonary exercise testing (CPET) parameters, particularly peak oxygen uptake (peakVO2) and ventilation/carbon dioxide (VE/VCO2)-slope, can predict survival in patients with SSc. SUBJECTS AND METHODS: We retrospectively assessed 210 patients (80.9% female) in 6 centres over 10 years with pulmonary testing and CPET. Survival was analysed with Cox regression analysis (adjusted for age and gender) by age, comorbidity (Charlson-Index), body weight, body-mass index, extensive interstitial lung disease, pulmonary artery pressure (measured by echocardiography and invasively), and haemodynamic, pulmonary and CPET parameters. RESULTS: Five- and ten-year survival of SSc patients was 93.8 and 86.9%, respectively. There was no difference in survival between patients with diffuse (dcSSc) and limited cutaneous manifestation (lcSSc; p = 0.3). Pulmonary and CPET parameters were significantly impaired. Prognosis was worst for patients with pulmonary hypertension (p = 0.007), 6-min walking distance < 413 m (p = 0.003), peakVO2 < 15.6 mL∙kg- 1∙min- 1, and VE/VCO2-slope > 35. Age (hazard ratio HR = 1.23; 95% confidence interval CI: 1.14;1.41), VE/VCO2-slope (HR = 0.9; CI 0.82;0.98), diffusion capacity (Krogh factor, HR = 0.92; CI 0.86;0.98), forced vital capacity (FVC, HR = 0.91; CI 0.86;0.96), and peakVO2 (HR = 0.87; CI 0.81;0.94) were significantly linked to survival in multivariate analyses (Harrell's C = 0.95). This is the first large study with SSc patients that demonstrates the prognostic value of peakVO2 < 15.6 mL∙kg- 1∙min- 1 (< 64.5% of predicted peakVO2) and VE/VCO2-slope > 35.


Assuntos
Teste de Esforço , Escleroderma Sistêmico/diagnóstico , Idoso , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Prognóstico , Estudos Retrospectivos
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