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1.
Eur Respir J ; 55(5)2020 May.
Artigo em Inglês | MEDLINE | ID: mdl-32139456

RESUMO

INTRODUCTION: Sarcoidosis-associated pulmonary hypertension (SAPH) is associated with reduced survival in single-centre studies. The international Registry for SAPH (ReSAPH) with long-term follow-up was established to enrich our knowledge of this complication of sarcoidosis. This analysis aims to elucidate factors associated with reduced transplant-free survival in SAPH patients. METHODS: ReSAPH contains prospectively collected outcomes of SAPH patients since the time of registry enrolment. Information analysed includes right heart catheterisation data, pulmonary function testing, chest radiography, Scadding stage and 6-min walk distance (6MWD), among others. Cox regression models were used to identify independent predictors of transplant-free survival. RESULTS: Data from 215 patients followed for a mean±sd 2.5±1.9 years were available for analysis. In the 159 precapillary patients, the Kaplan-Meier-adjusted 1-, 3- and 5-year transplant-free survival was 89.2%, 71.7% and 62.0%, respectively. Kaplan-Meier-adjusted 1-, 3- and 5-year transplant-free survival in the incident group was 83.5%, 70.3% and 58.3%, respectively, and in the prevalent group was 94.7%, 72.2% and 66.3%, respectively. Patients with reduced diffusing capacity of the lung for carbon monoxide (D LCO) (<35% predicted) and 6MWD <300 m in the precapillary cohort had significantly worse transplant-free survival. Reduced 6MWD and preserved forced expiratory volume (FEV1)/forced vital capacity (FVC) ratio were identified as independent risk factors for reduced transplant-free survival in the precapillary cohort. CONCLUSION: Reduced D LCO (<35% pred) and 6MWD (<300 m) at the time of registry enrolment were associated with reduced transplant-free survival in the overall precapillary cohort. Preserved FEV1/FVC ratio was identified as an independent risk factor for worsened outcomes.

2.
Respir Med ; 139: 72-78, 2018 06.
Artigo em Inglês | MEDLINE | ID: mdl-29858005

RESUMO

BACKGROUND: Pulmonary hypertension (PH) is a significant cause of morbidity and mortality in sarcoidosis. We established a multi-national registry of sarcoidosis associated PH (SAPH) patients. METHODS: Sarcoidosis patients with PH confirmed by right heart catheterization (RHC) were studied. Patients with pulmonary artery wedge pressure (PAWP) of 15 mmHg or less and a mean pulmonary artery pressure (mPAP) ≥ 25 Hg were subsequently analyzed. Data collected included hemodynamics, forced vital capacity (FVC), diffusion capacity of carbon monoxide (DLCO), chest x-ray, and 6-min walk distance (6MWD). RESULTS: A total of 176 patients were analyzed. This included 84 (48%) cases identified within a year of entry into the registry and 94 (53%) with moderate to severe PH. There was a significant correlation between DLCO percent predicted (% pred) andmPAP (Rho = -0.228, p = 0.0068) and pulmonary vascular resistance (PVR) (Rho = -0.362, p < 0.0001). PVR was significantly higher in stage 4 disease than in stage 0 or 1 disease (p < 0.05 for both comparisons). About two-thirds of the SAPH patients came from the United States (US). There was a significant difference in the rate of treatment between US (67.5%) versus non-US (86%) (Chi Square 11.26, p = 0.0008) sites. CONCLUSIONS: The clinical features of SAPH were similar across multiple centers in the US, Europe, and the Middle East. The severity of SAPH was related to reduced DLCO. There were treatment differences between the US and non-US centers.


Assuntos
Hipertensão Pulmonar/fisiopatologia , Sarcoidose Pulmonar/complicações , Sarcoidose Pulmonar/fisiopatologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Cateterismo Cardíaco , Europa (Continente) , Feminino , Hemodinâmica , Humanos , Hipertensão Pulmonar/diagnóstico por imagem , Masculino , Pessoa de Meia-Idade , Oriente Médio , Sistema de Registros , Sarcoidose Pulmonar/diagnóstico por imagem , Sarcoidose Pulmonar/etiologia , Estados Unidos , Capacidade Vital , Teste de Caminhada , Raios X
3.
Scand J Gastroenterol ; 53(7): 818-824, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-29852792

RESUMO

OBJECTIVE: The significantly higher incidence rates of microscopic colitis (MC) in Denmark compared to Sweden remains unexplained. METHODS: Consecutive patients with newly diagnosed MC in the neighbouring regions of Skåne in 2011-2015 and Zealand in 2010-2016 were prospectively identified. Data on large bowel endoscopies and biopsies rates were retrieved. Information on putative factors were obtained from registers and literature. Interobserver agreement between pathologists from both regions on 40 blinded hematoxylin and eosin (H&E)-stained colon biopsies (collagenous colitis (CC), lymphocytic colitis (LC), non-specific inflammation and normal) was evaluated using kappa statistics. RESULTS: The mean annual incidence per 105 inhabitants in Skåne and Zealand 2010-2015 was 5.9 (95% CI 4.6-7.3) versus 16.4 (95% confidence intervals (95% CI) 13.6-19.2) for CC and 2.7 (95% CI 1.0-4.3) versus 11.1 (95% CI 8.8-13.4) for LC, respectively. Number of endoscopies with biopsy per 1000 and the rate of MC per endoscopy with biopsy was higher in Zealand (34-52/1000) than in Skåne (12-21/1000). The kappa value for overall agreement between pathologists was good (0.72; 95% CI 0.64-0.79). Prescription of proton pump inhibitors and selective serotonin reuptake inhibitors was higher in Skåne in the relevant age groups and prescription of non-steroidal anti-inflammatory drugs and smoking rate higher in Zealand. Alcohol consumption was higher in Denmark than in Sweden. CONCLUSION: The incidence of MC and number of cases per colonic biopsy was higher in Zealand and could not be readily explained by endoscopy or biopsy rates, differences in histological assessment or putative risk factors.


Assuntos
Colite Microscópica/diagnóstico , Colite Microscópica/tratamento farmacológico , Colite Microscópica/epidemiologia , Adolescente , Adulto , Distribuição por Idade , Idoso , Idoso de 80 Anos ou mais , Anti-Inflamatórios não Esteroides/uso terapêutico , Biópsia , Dinamarca/epidemiologia , Prescrições de Medicamentos , Endoscopia , Feminino , Humanos , Incidência , Masculino , Pessoa de Meia-Idade , Inibidores da Bomba de Prótons/uso terapêutico , Fatores de Risco , Inibidores de Captação de Serotonina/uso terapêutico , Distribuição por Sexo , Suécia/epidemiologia , Adulto Jovem
4.
Clin Chest Med ; 36(4): 703-14, 2015 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-26593143

RESUMO

Pulmonary hypertension is a complication of sarcoidosis leading to dyspnea and associated with increased morbidity and mortality. Sarcoidosis-associated pulmonary hypertension (SAPH) can be due to several factors, including vascular involvement by the granulomatous inflammation, compression of the pulmonary arteries by adenopathy, fibrotic changes within the lung, and left ventricular diastolic dysfunction. Several case series have suggested that some patients with SAPH benefit from specific therapy for pulmonary hypertension. A randomized, placebo-controlled trial found 16 weeks' bosentan therapy to be associated with significant improvement in pulmonary artery pressure. Future studies may better define who would respond to treatment of pulmonary hypertension.


Assuntos
Hipertensão Pulmonar/etiologia , Pulmão/fisiopatologia , Sarcoidose/complicações , Humanos
5.
Chest ; 145(4): 810-817, 2014 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-24177203

RESUMO

BACKGROUND: Sarcoidosis-associated pulmonary hypertension (SAPH) is a common problem in patients with persistent dyspneic sarcoidosis. The objective of this study was to determine the effect of bosentan therapy on pulmonary arterial hemodynamics in patients with SAPH. METHODS: This 16-week study was a double-blind, placebo-controlled trial of either bosentan or placebo in patients with SAPH confirmed by right-sided heart catheterization. Patients were enrolled from multiple academic centers specializing in sarcoidosis care. They were stable on sarcoidosis therapy and were receiving no therapy for pulmonary hypertension. The cohort was randomized two to one to receive bosentan at a maximal dose of 125 mg or placebo bid for 16 weeks. Pulmonary function studies, 6-min walk test, and right-sided heart hemodynamics, including pulmonary artery mean pressure and pulmonary vascular resistance (PVR), were performed before and after 16 weeks of therapy. RESULTS: Thirty-five patients completed 16 weeks of therapy (23 treated with bosentan, 12 with placebo). For those treated with bosentan, repeat hemodynamic studies at 16 weeks demonstrated a significant mean±SD fall in PA mean pressure (-4±6.6 mm Hg, P=.0105) and PVR (-1.7±2.75 Wood units, P=.0104). For the patients treated with placebo, there was no significant change in either PA mean pressure (1±3.7 mm Hg, P>.05) or PVR (0.1±1.42 Wood units, P>.05). There was no significant change in 6-min walk distance for either group. Two patients treated with bosentan required an increase of supplemental oxygen by >2 L after 16 weeks of therapy. CONCLUSIONS: This study demonstrated that bosentan significantly improved pulmonary hemodynamics in patients with SAPH. TRIAL REGISTRY: ClinicalTrials.gov; No: NCT00581607; URL: www.clinicaltrials.gov.


Assuntos
Anti-Hipertensivos/uso terapêutico , Hipertensão Pulmonar/tratamento farmacológico , Sulfonamidas/uso terapêutico , Bosentana , Método Duplo-Cego , Feminino , Humanos , Hipertensão Pulmonar/etiologia , Masculino , Pessoa de Meia-Idade , Sarcoidose Pulmonar/complicações
6.
Chest ; 138(5): 1078-85, 2010 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-20348196

RESUMO

OBJECTIVE: Pulmonary hypertension (PH) has been associated with increased mortality in patients with advanced pulmonary sarcoidosis. Sarcoidosis-associated PH may be the result of left ventricular dysfunction (LVD) or isolated pulmonary vasculature abnormality. Our objective was to determine if the cause of PH affects survival in patients with sarcoidosis with persistent dyspnea. METHODS: Patients with sarcoidosis with persistent dyspnea despite immunosuppressive therapy underwent right-sided heart catheterization. Patients with a pulmonary artery occluding pressure (PAO) ≥ 15 mm Hg were defined as having LVD. Patients were classified based on hemodynamics as no PH (pulmonary artery mean pressure [PAmean] < 25 mm Hg), PH without LVD (PAmean ≥ 25 mm Hg and PAO < 15 mm Hg), and PH with LVD (PH/LVD) (PAmean ≥ 25 mm Hg and PAO ≥ 15 mm Hg). RESULTS: One hundred thirty patients were studied at one institution: 50 (38.5%) patients had PH without LVD, whereas 20 (15.4%) had PH/LVD. All patients had their diagnostic procedure at least 18 months prior to analysis. The hazard ratio (HR) for death in PH without LVD vs no PH was 10.39 (95% CI, 2.99-13.78; P < .0001). The HR for dying for PH without LVD vs PH/LVD was 3.14 (95% CI, 1.01-5.62; P < .05). The presence of stage 4 chest roentgenograms and the need for supplemental oxygen were different between the groups. In a Cox proportional hazards model, independent predictors for survival were stage 4 chest roentgenogram (P < .005) and hemodynamic group (P < .02). CONCLUSION: PH without LVD was associated with increased mortality. Proper characterization of patients required hemodynamic evaluation, as 29% of sarcoidosis-associated PH was due to LVD.


Assuntos
Cateterismo Cardíaco/métodos , Hipertensão Pulmonar/mortalidade , Pressão Propulsora Pulmonar/fisiologia , Sarcoidose Pulmonar/mortalidade , Adulto , Fatores Etários , Idoso , Idoso de 80 Anos ou mais , Feminino , Seguimentos , Humanos , Hipertensão Pulmonar/etiologia , Hipertensão Pulmonar/fisiopatologia , Masculino , Pessoa de Meia-Idade , Ohio/epidemiologia , Modelos de Riscos Proporcionais , Estudos Prospectivos , Sarcoidose Pulmonar/complicações , Sarcoidose Pulmonar/fisiopatologia , Fatores Sexuais , Taxa de Sobrevida/tendências , Adulto Jovem
7.
Med Hypotheses ; 73(3): 448-52, 2009 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-19473774

RESUMO

Significant progress has been made in the medical therapy of pulmonary hypertension in recent years, but long term results are still disappointing. We describe the theoretical underpinnings of a surgical procedure which we suggest might prevent or delay the occurrence of right ventricular failure and thereby improve long-term survival in pulmonary arterial hypertension. This is a hypothesis which could be tested in an animal model of this condition.


Assuntos
Hipertensão Pulmonar/complicações , Hipertensão Pulmonar/cirurgia , Modelos Cardiovasculares , Disfunção Ventricular Direita/etiologia , Disfunção Ventricular Direita/prevenção & controle , Adulto , Feminino , Humanos
8.
Ann Intern Med ; 149(8): 521-30, 2008 Oct 21.
Artigo em Inglês | MEDLINE | ID: mdl-18936500

RESUMO

BACKGROUND: Oral sildenafil and intravenous epoprostenol have independently been shown to be effective in patients with pulmonary arterial hypertension. OBJECTIVE: To investigate the effect of adding oral sildenafil to long-term intravenous epoprostenol in patients with pulmonary arterial hypertension. DESIGN: A 16-week, double-blind, placebo-controlled, parallel-group study. SETTING: Multinational study at 41 centers in 11 countries from 3 July 2003 to 27 January 2006. PATIENTS: 267 patients with pulmonary arterial hypertension (idiopathic, associated anorexigen use or connective tissue disease, or corrected congenital heart disease) who were receiving long-term intravenous epoprostenol therapy. INTERVENTION: Patients were randomly assigned to receive placebo or sildenafil, 20 mg three times daily, titrated to 40 mg and 80 mg three times daily, as tolerated, at 4-week intervals. Of 265 patients who received treatment, 256 (97%) patients (123 in the placebo group and 133 in the sildenafil group) completed the study. MEASUREMENTS: Change from baseline in exercise capacity measured by 6-minute walk distance (primary end point) and hemodynamic measurements, time to clinical worsening, and Borg dyspnea score (secondary end points). RESULTS: A placebo-adjusted increase of 28.8 meters (95% CI, 13.9 to 43.8 meters) in the 6-minute walk distance occurred in patients in the sildenafil group; these improvements were most prominent among patients with baseline distances of 325 meters or more. Relative to epoprostenol monotherapy, addition of sildenafil resulted in a greater change in mean pulmonary arterial pressure by -3.8 mm Hg (CI, -5.6 to -2.1 mm Hg); cardiac output by 0.9 L/min (CI, 0.5 to 1.2 L/min); and longer time to clinical worsening, with a smaller proportion of patients experiencing a worsening event in the sildenafil group (0.062) than in the placebo group (0.195) by week 16 (P = 0.002). Health-related quality of life also improved in patients who received combined therapy compared with those who received epoprostenol monotherapy. There was no effect on the Borg dyspnea score. Of the side effects generally associated with sildenafil treatment, the most commonly reported in the placebo and sildenafil groups, respectively, were headache (34% and 57%; difference, 23 percentage points [CI, 12 to 35 percentage points]), dyspepsia (2% and 16%; difference, 13 percentage points [CI, 7 to 20 percentage points]), pain in extremity (18% and 25%; difference, 8 percentage points [CI, -2 to 18 percentage points]), and nausea (18% and 25%; difference, 8 percentage points [CI, -2 to 18 percentage points]). LIMITATIONS: The study excluded patients with pulmonary arterial hypertension associated with other causes. There was an imbalance in missing data between groups, with 8 placebo recipients having no postbaseline walk assessment compared with 1 sildenafil recipient. These patients were excluded from the analysis. CONCLUSION: In some patients with pulmonary arterial hypertension, the addition of sildenafil to long-term intravenous epoprostenol therapy improves exercise capacity, hemodynamic measurements, time to clinical worsening, and quality of life, but not Borg dyspnea score. Increased rates of headache and dyspepsia occurred with the addition of sildenafil.


Assuntos
Anti-Hipertensivos/administração & dosagem , Epoprostenol/administração & dosagem , Hipertensão Pulmonar/tratamento farmacológico , Piperazinas/administração & dosagem , Sulfonas/administração & dosagem , Vasodilatadores/administração & dosagem , Administração Oral , Adolescente , Adulto , Idoso , Anti-Hipertensivos/efeitos adversos , Método Duplo-Cego , Quimioterapia Combinada , Dispepsia/induzido quimicamente , Epoprostenol/efeitos adversos , Feminino , Cefaleia/induzido quimicamente , Hemodinâmica/fisiologia , Humanos , Hipertensão Pulmonar/etiologia , Hipertensão Pulmonar/fisiopatologia , Injeções Intravenosas , Masculino , Pessoa de Meia-Idade , Piperazinas/efeitos adversos , Purinas/administração & dosagem , Purinas/efeitos adversos , Qualidade de Vida , Citrato de Sildenafila , Sulfonas/efeitos adversos , Fatores de Tempo , Vasodilatadores/efeitos adversos , Caminhada/fisiologia
9.
Am J Cardiol ; 99(8): 1177-8, 2007 Apr 15.
Artigo em Inglês | MEDLINE | ID: mdl-17437753

RESUMO

Two cases of severe pulmonary arterial hypertension in patients with neurofibromatosis are reported. The published research is reviewed. In conclusion, it is suggested that the association between these conditions be recognized in the classification of pulmonary hypertension.


Assuntos
Hipertensão Pulmonar/etiologia , Neurofibromatose 1/complicações , Idoso , Manchas Café com Leite/patologia , Feminino , Humanos , Pessoa de Meia-Idade , Neurofibromatose 1/patologia
10.
Sarcoidosis Vasc Diffuse Lung Dis ; 23(2): 108-16, 2006 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-17937106

RESUMO

BACKGROUND: Pulmonary hypertension has been notreported in some patients with sarcoidosis. METHODS: We retrospectively studied 53 sarcoidosis patients with persistent dyspnea despite systemic therapy for their sarcoidosis. All patients underwent cardiac catheterization to determine pulmonary artery (PA) pressure. RESULTS: Of the 53 patients, six were found to have left ventricle (LV) dysfunction, including four cases of diastolic dysfunction. Of the remaining 47 patients, 26 had a systolic PA pressure > or = 40 Torr and 25 had a mean PA pressure > or = 25 Torr. Using univariate analysis of those patients with normal LV function, echocardiography, vital capacity, and diffusion lung of carbon monoxide (D(L)co) correlated with systolic and/or mean pulmonary artery pressure. For the PA systolic, only the echocardiographic estimated PA pressure and D(L)CO % predicted remained in the multiple regression model (Coefficient of determination = 0.76, p < 0.005 for both). For the PA mean pressure, the only independent variable was the echocardiographic estimate of the PA pressure (Coefficient of determination = 0.70, p < 0.005). While echocardiography was useful in many cases, in nine cases PA pressure could not be estimated because there was no tricuspid regurgitation seen. Seven of these patients had a measured PA pressure of > or = 40 Torr. Seven patients with moderate to severe pulmonary hypertension were treated with pulmonary vasodilator therapy. Five patients experienced good clinical response. CONCLUSION: Pulmonary hypertension was commonly found in sarcoidosis patients with persistent dyspnea. For some of these patients, treatment of the pulmonary hypertension was associated with improved clinical status.


Assuntos
Hipertensão Pulmonar/etiologia , Pressão Propulsora Pulmonar/fisiologia , Sarcoidose Pulmonar/complicações , Adulto , Idoso , Cateterismo Cardíaco , Ecocardiografia , Feminino , Seguimentos , Humanos , Hipertensão Pulmonar/diagnóstico , Hipertensão Pulmonar/fisiopatologia , Masculino , Pessoa de Meia-Idade , Prognóstico , Testes de Função Respiratória , Estudos Retrospectivos , Fatores de Risco , Sarcoidose Pulmonar/diagnóstico , Sarcoidose Pulmonar/fisiopatologia , Volume Sistólico/fisiologia , Tomografia Computadorizada por Raios X , Função Ventricular Esquerda/fisiologia
13.
Neurology ; 59(1): 92-8, 2002 Jul 09.
Artigo em Inglês | MEDLINE | ID: mdl-12105313

RESUMO

OBJECTIVE: To estimate the incidence of elevated anti-titin antibodies titers and of thymomas in a population of patients with MG using various statistics and associations. METHODS: Extensive epidemiology, systematic measurement of anti-titin antibodies, and histologic assessment of thymomas according to the new World Health Organization classification. RESULTS: The mean annual incidence rate of MG per million population was 8.3. The analogous mean rate of thymomas was 2.0, out of which MG was encountered in about 20%. A thymoma was coexistent in 7% of the patients with MG. The finding of titin autoantibodies and the coexistence of thymomas were both associated with age at the appearance of MG. In patients with MG with a thymoma, the frequency of seropositivity was 68%, whereas acetylcholine receptor (AChR) autoantibodies were detected in all such sera. Titin autoantibody-positive sera were also anti-AChR antibodies positive. Further, all serum samples negative for anti-AChR antibodies were devoid of anti-titin antibodies. Titin autoantibodies were not detected in nonthymoma early-onset MG. CONCLUSION: Apart from MG with a thymoma, the finding of the titin autoantibodies was observed to be an exclusive feature of late-onset MG, the frequency being 55%. No data were found to suggest that patients with MG were more likely to present with thymic tumors than other patients exhibiting thymic neoplasia. In about 80%, such tumors in MG were composed of cortical cells. The concept of the anti-titin antibodies merely as a paraneoplastic marker in MG was not supported by these data.


Assuntos
Proteínas Musculares/imunologia , Proteínas Quinases/imunologia , Timoma/epidemiologia , Timoma/imunologia , Neoplasias do Timo/epidemiologia , Neoplasias do Timo/imunologia , Adolescente , Adulto , Distribuição por Idade , Idoso , Idoso de 80 Anos ou mais , Criança , Conectina , Feminino , Humanos , Incidência , Masculino , Pessoa de Meia-Idade , Estudos Soroepidemiológicos , Distribuição por Sexo
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