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1.
Eur Respir J ; 2020 Mar 05.
Artigo em Inglês | MEDLINE | ID: mdl-32139456

RESUMO

INTRODUCTION: Sarcoidosis associated pulmonary hypertension (SAPH) is associated with reduced survival in single center studies. An 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 enrollment. Information analysed includes right heart catheterisation data, pulmonary function testing, chest x-ray Scadding stage, 6 min walk distance (6MWD) among others. Cox regression models were used to identify independent predictors of transplant-free survival. RESULTS: Data from a total of 215 patients followed for a mean of 2.5±1.9 years were available for analysis. In the 159 pre-capillary patients the 1, 3 and 5 year transplant free survival was 98.2.1%, 78.2% and 71.2%, respectively. Incident group 83.5%, 70.3% and 58.3% and prevalent group 94.7%, 72.2%, 66.3% 1,3 and 5 year survival, respectively. Patients with reduced DLCO and 6MWD <300 m in the pre-capillary cohort had significantly worse transplant-free survival. Reduced 6MWD and preserved FEV1/FVC ratio were identified as independent risk factors for reduced transplant-free survival in the pre-capillary cohort. CONCLUSION: Reduced diffusion capacity and 6MWD <300 m at the time of registry enrollment were associated with reduced transplant-free survival in the overall precapillary cohort. Preserved FEV1/FVC ratio was also identified as an independent risk factor for worsened outcomes.

2.
Chest ; 154(4): 818-826, 2018 10.
Artigo em Inglês | MEDLINE | ID: mdl-29966665

RESUMO

BACKGROUND: Acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) has an expected median survival of 3 months. Lung transplantation is a potentially lifesaving therapy for AE-IPF. However, the current knowledge of transplantation outcomes during AE-IPF is limited to a few small retrospective studies, reporting only 1-year post-transplantation survival. METHODS: Study population included patients with IPF consecutively listed for lung transplantation at a single institution between the years 2012 and 2016. We collected lung allocation score (LAS), hospitalization, and survival data. The primary outcome was survival among patients transplanted during stable IPF vs during AE-IPF. RESULTS: Of 89 patients with IPF listed for lung transplantation, 52 were transplanted during stable IPF and 37 were hospitalized due to AE-IPF. Of these 37 patients, nine died before transplantation, and 28 were transplanted during AE-IPF. Fifty percent of patients transplanted during AE-IPF died in a mean follow-up of 1.6 ± 1.2 years compared with 12% of patients transplanted during stable IPF who died in a mean follow-up of 2.6 ± 1.2 years. The Kaplan-Meier survival curves post-transplantation after 1 and 3 years for patients who were transplanted during stable IPF were 94% and 90% vs 71% and 60% in patients who were transplanted during AE-IPF (P = .0001). LAS above 80 conferred a 3-year hazard ratio for mortality of 5.7 vs LAS lower than 80 (95% CI, 2.33-14.0; P < .0005). CONCLUSIONS: Patients with IPF transplanted during AE-IPF had significantly worse short-term and long-term survival compared with patients transplanted during stable IPF. Patients with AE-IPF and very high LAS may not experience the survival advantage expected from lung transplantation.


Assuntos
Fibrose Pulmonar Idiopática/mortalidade , Transplante de Pulmão/mortalidade , Doença Aguda , Idoso , Causas de Morte , Feminino , Volume Expiratório Forçado/fisiologia , Humanos , Fibrose Pulmonar Idiopática/complicações , Fibrose Pulmonar Idiopática/cirurgia , Estimativa de Kaplan-Meier , Masculino , Cuidados Pós-Operatórios/mortalidade , Cuidados Pré-Operatórios/mortalidade , Insuficiência Respiratória/complicações , Insuficiência Respiratória/mortalidade , Estudos Retrospectivos , Resultado do Tratamento , Capacidade Vital/fisiologia
3.
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
4.
Telemed J E Health ; 22(2): 114-122, 2016 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-26259074

RESUMO

BACKGROUND: Patients with chronic obstructive pulmonary disease (COPD) may not recognize worsening symptoms that require intensification of therapy. They may also be reluctant to contact a healthcare provider for minor worsening of symptoms. A telemedicine application for daily symptom reporting may reduce these barriers and improve patient outcomes. MATERIALS AND METHODS: Patients hospitalized for a COPD exacerbation within the past year or using supplemental O2 were approached for participation. Patients received optimal COPD care and were given a telecommunication device for symptom reporting. Initial symptom scores were obtained while patients were in their usual state of health. Patients were randomly assigned to an intervention group or a control group (usual medical care). The control group patients were instructed to seek medical care if their condition worsened. The intervention group symptom scores were assessed by a computer algorithm and compared with initial values. Scores 1 or more points above the initial score generated an "alert," and patients were reviewed by a nurse and referred to a physician who prescribed treatment. RESULTS: Eighty-six patients were screened; 79 met entry criteria and were randomized (intervention group, n=39; control group, n=40). Twelve patients submitted five or fewer symptom reports (5 intervention; 7 control) and were excluded from the analysis. Daily peak flow and dyspnea scores improved only in the intervention group. There were no differences in hospitalization and mortality rates between groups. No serious adverse events were reported. CONCLUSIONS: A telemedicine-based symptom reporting program facilitated early treatment of symptoms and improved lung function and functional status.

6.
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
7.
Curr Opin Pulm Med ; 19(5): 531-7, 2013 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-23912192

RESUMO

PURPOSE OF REVIEW: Pulmonary hypertension is a serious complication of sarcoidosis. This review discusses clinical characteristics of patients with sarcoid-associated pulmonary hypertension (SAPH) and pitfalls in the diagnosis, and highlights potential therapies. RECENT FINDINGS: SAPH is common in patients with advanced disease, but it can occur in patients with minimal disease burden. Risk factors for SAPH include restrictive lung physiology, hypoxemia, advanced Scadding chest X-ray stage, and low carbon monoxide diffusion capacity. Echocardiogram is a good initial screening tool in the diagnosis of pulmonary hypertension, but right heart catheterization is necessary to confirm the diagnosis. Treatment with pulmonary vasodilators, including endothelin antagonists, can lead to improvements in pulmonary hemodynamics in some patients but may not improve their exercise capacity. Forced vital capacity is an important predictor of exercise performance in patients with SAPH. Clinical observations and response to specific therapies for pulmonary hypertension suggest the presence of different SAPH phenotypes. SUMMARY: Patients who complain of persistent dyspnea should be screened for the presence of pulmonary hypertension. The prognosis of SAPH is poor and it is prudent to consider referral of these patients for lung transplantation. In some patients with SAPH, treatment with anti-inflammatory agents and pulmonary vasodilators can lower pulmonary arterial pressures, improve dyspnea and functionality, and enhance overall quality of life.


Assuntos
Gerenciamento Clínico , Hipertensão Pulmonar/etiologia , Sarcoidose Pulmonar/complicações , Cateterismo Cardíaco , Ecocardiografia , Endotelinas/antagonistas & inibidores , Humanos , Hipertensão Pulmonar/diagnóstico , Hipertensão Pulmonar/terapia , Vasodilatadores/uso terapêutico
8.
Med Clin North Am ; 96(4): 827-47, 2012 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-22793947

RESUMO

Over the last decade, advances in bronchoscopic and surgical techniques have expanded our treatment armamentarium for patients with severe emphysema who previously would have received a pessimistic outlook from their physician. Advances in our understanding of the different COPD phenotypes and its natural history has refined our selection process as to which group of emphysema patients will derive maximum benefit from LVR, bullectomy, or lung transplantation. Because emphysema is a progressive disease, initial treatment with bronchoscopic or surgical LVR or bullectomy does not preclude lung transplantation in the future.


Assuntos
Broncoscopia/métodos , Transplante de Pulmão , Pneumonectomia/métodos , Doença Pulmonar Obstrutiva Crônica/cirurgia , Vesícula/cirurgia , Broncoscopia/instrumentação , Humanos , Seleção de Pacientes , Complicações Pós-Operatórias , Doença Pulmonar Obstrutiva Crônica/patologia , Doença Pulmonar Obstrutiva Crônica/fisiopatologia , Doença Pulmonar Obstrutiva Crônica/terapia , Resultado do Tratamento
9.
J Am Osteopath Assoc ; 107(4): 148-56, 2007 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-17525241

RESUMO

BACKGROUND: Noninvasive positive-pressure ventilation (NPPV) is commonly used to improve ventilation and oxygenation in patients with acute respiratory failure (ARF). Mask leak and intolerance due to facial discomfort or claustrophobia often occur with NPPV and are frequently cited reasons for treatment failure. METHODS: Retrospective review of patient records from a tertiary-care referral hospital. RESULTS: We report the effectiveness of a full face mask in the application of NPPV for 10 nonambulatory patients (mean [SD], 61 [9] years) who had a combined total of 13 episodes of ARF. After these patients were unable to receive NPPV therapy via the more commonly available nasal or oronasal masks, care was provided using full face masks. Eight of 10 patients had hypercapnic respiratory failure; 2 patients, hypoxemic respiratory failure. All patients were placed on ventilation initially using a bi-level positive airway pressure device. Subsequently, patient ventilation was achieved using a Puritan Bennett 7200a ventilator for on-line respiratory monitoring. The mean (SD) duration of treatment with NPPV was 9.7 (2.7) hours per day for 3.0 (1.6) days. Following NPPV via full face mask, the patients' Paco(2) decreased (65 [20] vs 82 [27] mm Hg, P=.09) and pH increased significantly (7.36 [0.07] vs 7.26 [0.07], P<.05) in less than 2 hours. Moreover, the patients demonstrated decreased respiratory rate (18 [7] vs 32 [8] breaths/min, P<.01), heart rate (106 [13] vs 124 [16] beats/min, P=.008), and Acute Physiology and Chronic Health Evaluation II scores (12 [3] vs 17 [4], P<.005) after NPPV via full face mask. These cardiorespiratory alterations occurred as early as 1 hour after NPPV initiation and were maintained throughout treatment. Two patients required endotracheal intubation because of copious purulent secretions. CONCLUSION: For individuals with hypercapnic respiratory failure who cannot tolerate NPPV using nasal or oronasal masks, use of full face masks may improve outcomes, allowing physicians to avoid ordering endotracheal intubation and mechanical ventilation.


Assuntos
Ventilação com Pressão Positiva Intermitente/instrumentação , Máscaras , Insuficiência Respiratória/terapia , Resultado do Tratamento , Doença Aguda , Idoso , Feminino , Humanos , Hipercapnia/terapia , Ventilação com Pressão Positiva Intermitente/métodos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos
10.
J Heart Lung Transplant ; 25(1): 140-3, 2006 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-16399546

RESUMO

We report the case of a 58-year-old man with severe chronic obstructive pulmonary disease who developed acute quadriparesis during the post-operative period following bilateral lung transplantation after receiving cyclosporine for immunosuppression. Electromyography with nerve conduction study and cerebrospinal fluid analysis supported a diagnosis of Guillain-Barré Syndrome, which improved upon the discontinuation of cyclosporine, replacement with tacrolimus, and initiation of plasmapheresis. We propose the discontinuation of cyclosporine and initiation of plasmapheresis as a treatment for cyclosporine-associated Guillain-Barré syndrome.


Assuntos
Ciclosporina/efeitos adversos , Síndrome de Guillain-Barré/terapia , Imunossupressores/efeitos adversos , Transplante de Pulmão , Plasmaferese , Ciclosporina/uso terapêutico , Humanos , Imunossupressores/uso terapêutico , Masculino , Pessoa de Meia-Idade , Doença Pulmonar Obstrutiva Crônica/cirurgia , Resultado do Tratamento
11.
Am J Respir Med ; 1(5): 313-24, 2002.
Artigo em Inglês | MEDLINE | ID: mdl-14720034

RESUMO

Lung volume reduction surgery (LVRS) improves lung function, exercise capacity, and quality of life in patients with advanced emphysema. In some patients with emphysema who are candidates for lung transplantation, LVRS is an alternative treatment option to lung transplantation, or may be used as a bridge to lung transplantation. Generally accepted criteria for LVRS include severe non-reversible airflow obstruction due to emphysema associated with significant evidence of lung hyperinflation and air trapping. Both high resolution computed tomography (CT) scan of the chest and quantitative ventilation/perfusion scan are used to identify lung regions with severe emphysema which would be used as targets for lung resection. Bilateral LVRS is the preferred surgical approach compared with the unilateral procedure because of better functional outcome. Lung transplantation is the preferred surgical treatment in patients with emphysema with alpha1 antitrypsin deficiency and in patients with very severe disease who have homogeneous emphysema pattern on CT scan of the chest or very low diffusion capacity.


Assuntos
Enfisema/cirurgia , Transplante de Pulmão , Pneumonectomia , Humanos , Pulmão/fisiopatologia , Pulmão/cirurgia , Seleção de Pacientes , Pneumonectomia/mortalidade , Resultado do Tratamento
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