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2.
Braz J Med Biol Res ; 52(8): e8513, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31365695

RESUMO

Phenotypic differences have been described between patients with systemic sclerosis (SSc)-associated interstitial lung disease (ILD) and SSc-associated pulmonary hypertension, including performance differences in the 6-min walk test (6MWT). Moreover, the correlations between the 6MWT and traditional pulmonary function tests (PFTs) are weak, indicating the need to search for new parameters that explain exercise performance. Thus, our objective was to evaluate the impact of ventilation distribution heterogeneity assessed by the nitrogen single-breath washout (N2SBW) test and peripheral muscle dysfunction on the exercise capacity in patients with SSc-ILD and limited involvement of the pulmonary parenchyma. In this cross-sectional study, 20 women with SSc-ILD and 20 matched controls underwent PFTs (including spirometry, diffusing capacity for carbon monoxide (DLco), and the N2SBW test) and performed the 6MWT and knee isometric dynamometry. The 6-min walking distance (6MWD, % predicted) was strongly correlated with the phase III slope of the single-breath nitrogen washout (phase III slopeN2SBW) (r=-0.753, P<0.0001) and reasonably correlated with the forced vital capacity (FVC) (r=0.466, P=0.008) and DLco (r=0.398, P=0.011). The peripheral oxygen saturation (SpO2) during exercise was not significantly correlated with any of the pulmonary or muscle function parameters. The phase III slopeN2SBW was the only predictive variable for the 6MWD, whereas quadriceps strength and FVC/DLco were predictive variables for SpO2. Ventilation distribution heterogeneity is one factor that contributes to a lower 6MWD in SSc-ILD patients. In addition, muscle dysfunction and abnormal lung diffusion at least partly explain the decreased SpO2 of these patients.


Assuntos
Tolerância ao Exercício/fisiologia , Hipertensão Pulmonar/fisiopatologia , Doenças Pulmonares Intersticiais/fisiopatologia , Pulmão/fisiologia , Testes de Função Respiratória/métodos , Escleroderma Sistêmico/complicações , Adulto , Estudos de Casos e Controles , Feminino , Humanos , Hipertensão Pulmonar/etiologia , Pulmão/fisiopatologia , Doenças Pulmonares Intersticiais/etiologia , Medidas de Volume Pulmonar/métodos , Pessoa de Meia-Idade , Ventilação Pulmonar , Doença de Raynaud/complicações , Tomografia Computadorizada por Raios X/métodos , Capacidade Vital/fisiologia , Teste de Caminhada/métodos
3.
Medicine (Baltimore) ; 98(29): e16419, 2019 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-31335692

RESUMO

Squawks are lung adventitious sounds with a mix of both musical and nonmusical components heard during the inspiratory phase. Small series have described squawks in interstitial lung diseases. Hypersensitivity pneumonitis and other diseases involving small airways can result in squawks, but new interstitial lung diseases (ILDs) involving peripheral airways are being described. A retrospective analysis was performed on 1000 consecutive patients from a database of ILD of a tertiary referral center. Squawks were recorded in 49 cases (4.9%), hypersensitivity pneumonitis (23 cases), connective tissue disease (7), microaspiration (4), pleuroparenchymal fibroelastosis (4), fibrosing cryptogenic organizing pneumonia (, 3), familial ILD (2), sarcoidosis (2), idiopathic pulmonary fibrosis (IPF; 1), bronchiolitis (2), and nonspecific interstitial pneumonia (1). One patient had a final diagnosis of IPF. There was a significant association between mosaic pattern and squawks: 20 cases with squawks (40.8%) had mosaic pattern compared with 140 (14.7%) cases without squawks (x = 23.6, P < .001).Findings indicative of fibrosis were described on high-resolution chest tomography (HRCT) in 715 cases (71.5%). Squawks were more common in patients with findings indicative of fibrosis on HRCT: 45 of 715 (6.3%) compared with 4 of 285 (1.4%) of those without findings indicative of fibrosis (x = 10.46, P = .001).In conclusion, squawks are an uncommon finding on physical examination in patients with ILD, but when present suggest fibrosing ILD associated with bronchiolar involvement. However, squawks are rare in IPF.


Assuntos
Doenças Pulmonares Intersticiais , Fibrose Pulmonar , Sons Respiratórios , Auscultação/métodos , Bronquíolos/patologia , Bronquíolos/fisiopatologia , Estudos de Coortes , Feminino , Humanos , Doenças Pulmonares Intersticiais/complicações , Doenças Pulmonares Intersticiais/diagnóstico , Doenças Pulmonares Intersticiais/fisiopatologia , Masculino , Pessoa de Meia-Idade , Fibrose Pulmonar/diagnóstico por imagem , Fibrose Pulmonar/etiologia , Fibrose Pulmonar/fisiopatologia , Sons Respiratórios/diagnóstico , Sons Respiratórios/fisiopatologia , Estudos Retrospectivos , Tomografia Computadorizada por Raios X/métodos
4.
Int J Chron Obstruct Pulmon Dis ; 14: 1109-1118, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31213791

RESUMO

Objective: To examine whether the parameters of impedance measured by the broadband frequency forced oscillation technique (FOT) were reflected by changes in lung compliance induced by emphysema and fibrosis, we retrospectively compared the parameters of FOT and pulmonary functions, including static lung compliance (Cst), in obstructive lung disease (OLD) and interstitial lung disease (ILD). Patients and methods: The data of 18 patients with OLD (16 with COPD, two with asthma COPD overlap), 11 with ILD, and 24 healthy volunteers, whose respiratory impedance was measured using a MostGraph-01 and other pulmonary functions including Cst and lung resistance (RL) were measured on the same day and were retrospectively collected and compared. Results: The parameters of resistance, reactance, and resonant frequency showed good correlations with ventilation unevenness (r=0.63, -0.89, 0.77, respectively) and lung elastic resistance (r=0.59, -0.80, 0.73, respectively) in all groups (N=53). These indices were significant determinants of increased respiratory resistance and more negative shift of respiratory reactance (Xrs) at lower frequency (P<0.001). Decreased functional residual capacity was also a determinant of Xrs at 5 Hz (X5) (P<0.05). The differences in mean X5 in the expiratory phase relative to those in the inspiratory phase (within-breath changes in X5) were associated with airflow obstruction (P<0.002) and lung elastic resistance (P<0.001). However, no significant correlations between Cst and any parameters of respiratory impedance were observed. Conclusions: These findings suggest that the impedance parameters measured by FOT are reflected by airway obstruction, ventilation unevenness, and lung resistance, but hardly reflected by changes in lung compliance due to emphysema or fibrosis in both CLD and ILD.


Assuntos
Resistência das Vias Respiratórias , Doenças Pulmonares Intersticiais/diagnóstico , Pneumopatias Obstrutivas/diagnóstico , Pulmão/fisiopatologia , Ventilação Pulmonar , Testes de Função Respiratória/métodos , Idoso , Feminino , Humanos , Complacência Pulmonar , Doenças Pulmonares Intersticiais/fisiopatologia , Pneumopatias Obstrutivas/fisiopatologia , Masculino , Pessoa de Meia-Idade , Oscilometria , Valor Preditivo dos Testes , Estudos Retrospectivos
5.
Respir Investig ; 57(4): 368-375, 2019 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-31092319

RESUMO

BACKGROUND: Physical activity measures are valuable for assessing the progression of chronic respiratory diseases. The 4-m gait speed (4MGS) test is an established functional assessment in the elderly. However, the relationship between the 4MGS and daily activity in patients with chronic respiratory diseases has not been fully understood. The present study aimed to investigate whether the 4MGS predicted daily activity, including physical activity level (PAL), in patients with chronic respiratory diseases. METHODS: We enrolled 57 patients with chronic respiratory diseases, including interstitial lung disease and chronic obstructive pulmonary disease, and evaluated the correlations between the 4MGS and various clinical parameters, including respiratory function, the 6-min walk test (6MWT), and daily activities, by using an accelerometer. Linear regression analysis was performed to identify significant predictors of daily activity. RESULTS: The 4MGS was significantly correlated with daily step counts and PAL, as well as the 6 min walk distance (r = 0.477, p < 0.001; r = 0.433, p = 0.001; and r = 0.593, p < 0.001, respectively). In the multivariate linear regression analysis, the 4MGS, % predicted forced expiratory volume in 1 s, and body mass index were independent predictors of PAL. Receiver operating characteristic analysis revealed that a 4MGS <1.07 m/s was the optimal cutoff for predicting an inactive PAL (area under the curve, 0.728; 95% confidence interval, 0.589-0.866). Patients with a slower 4MGS had significantly reduced daily activity than did those with a preserved 4MGS, despite similar modified Medical Research Council dyspnea scale measures and respiratory parameters, such as oxygenation profiles. CONCLUSIONS: The 4MGS test is a simple screening test and a useful predictor of worsening daily activity in patients with chronic respiratory diseases.


Assuntos
Atividades Cotidianas , Exercício/fisiologia , Transtornos Respiratórios/fisiopatologia , Velocidade de Caminhada/fisiologia , Acelerometria , Idoso , Índice de Massa Corporal , Doença Crônica , Progressão da Doença , Feminino , Humanos , Modelos Lineares , Doenças Pulmonares Intersticiais/fisiopatologia , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Doença Pulmonar Obstrutiva Crônica/fisiopatologia , Curva ROC
6.
N Engl J Med ; 380(26): 2518-2528, 2019 06 27.
Artigo em Inglês | MEDLINE | ID: mdl-31112379

RESUMO

BACKGROUND: Interstitial lung disease (ILD) is a common manifestation of systemic sclerosis and a leading cause of systemic sclerosis-related death. Nintedanib, a tyrosine kinase inhibitor, has been shown to have antifibrotic and antiinflammatory effects in preclinical models of systemic sclerosis and ILD. METHODS: We conducted a randomized, double-blind, placebo-controlled trial to investigate the efficacy and safety of nintedanib in patients with ILD associated with systemic sclerosis. Patients who had systemic sclerosis with an onset of the first non-Raynaud's symptom within the past 7 years and a high-resolution computed tomographic scan that showed fibrosis affecting at least 10% of the lungs were randomly assigned, in a 1:1 ratio, to receive 150 mg of nintedanib, administered orally twice daily, or placebo. The primary end point was the annual rate of decline in forced vital capacity (FVC), assessed over a 52-week period. Key secondary end points were absolute changes from baseline in the modified Rodnan skin score and in the total score on the St. George's Respiratory Questionnaire (SGRQ) at week 52. RESULTS: A total of 576 patients received at least one dose of nintedanib or placebo; 51.9% had diffuse cutaneous systemic sclerosis, and 48.4% were receiving mycophenolate at baseline. In the primary end-point analysis, the adjusted annual rate of change in FVC was -52.4 ml per year in the nintedanib group and -93.3 ml per year in the placebo group (difference, 41.0 ml per year; 95% confidence interval [CI], 2.9 to 79.0; P = 0.04). Sensitivity analyses based on multiple imputation for missing data yielded P values for the primary end point ranging from 0.06 to 0.10. The change from baseline in the modified Rodnan skin score and the total score on the SGRQ at week 52 did not differ significantly between the trial groups, with differences of -0.21 (95% CI, -0.94 to 0.53; P = 0.58) and 1.69 (95% CI, -0.73 to 4.12 [not adjusted for multiple comparisons]), respectively. Diarrhea, the most common adverse event, was reported in 75.7% of the patients in the nintedanib group and in 31.6% of those in the placebo group. CONCLUSIONS: Among patients with ILD associated with systemic sclerosis, the annual rate of decline in FVC was lower with nintedanib than with placebo; no clinical benefit of nintedanib was observed for other manifestations of systemic sclerosis. The adverse-event profile of nintedanib observed in this trial was similar to that observed in patients with idiopathic pulmonary fibrosis; gastrointestinal adverse events, including diarrhea, were more common with nintedanib than with placebo. (Funded by Boehringer Ingelheim; SENSCIS ClinicalTrials.gov number, NCT02597933.).


Assuntos
Inibidores Enzimáticos/uso terapêutico , Indóis/uso terapêutico , Doenças Pulmonares Intersticiais/tratamento farmacológico , Proteínas Tirosina Quinases/antagonistas & inibidores , Escleroderma Sistêmico/complicações , Administração Oral , Adulto , Diarreia/induzido quimicamente , Progressão da Doença , Método Duplo-Cego , Inibidores Enzimáticos/efeitos adversos , Feminino , Humanos , Indóis/efeitos adversos , Doenças Pulmonares Intersticiais/etiologia , Doenças Pulmonares Intersticiais/fisiopatologia , Masculino , Pessoa de Meia-Idade , Escleroderma Sistêmico/tratamento farmacológico , Capacidade Vital
7.
Pan Afr Med J ; 32: 40, 2019.
Artigo em Francês | MEDLINE | ID: mdl-31143345

RESUMO

Anti-synthetase syndrome (ASS) is an inflammatory myopathy commonly associated with pulmonary involvement, especially parenchymal (diffuse infiltrative pneumonitis). Extrathoracic manifestations associated with pulmonary involvement can give an indication to the diagnosis: myalgias, polyarthralgias, Raynaud's syndrome, erythematous palmar hyperkeratosis with fissures and fever. Given the suggestive clinical and radiological picture, the presence of aminoacyl-transfer RNA (tRNA) synthetase antibodies enables to confirm, in particular, Anti Jo-1 antibody activity. Pulmonary involvement is a major prognostic factor, hence the indication for intensive immunosuppressive therapy based on corticosteroids, immunosuppressive medications or the association among them. A better awareness about this disorder revealed by pulmonary manifestations could enable early and adequate management and to improve patient's prognosis.


Assuntos
Imunossupressores/administração & dosagem , Doenças Pulmonares Intersticiais/etiologia , Miosite/diagnóstico , Corticosteroides/administração & dosagem , Adulto , Idoso , Anticorpos Antinucleares/imunologia , Feminino , Humanos , Doenças Pulmonares Intersticiais/tratamento farmacológico , Doenças Pulmonares Intersticiais/fisiopatologia , Masculino , Miosite/tratamento farmacológico , Miosite/imunologia , Prognóstico
8.
BMJ Case Rep ; 12(4)2019 Apr 14.
Artigo em Inglês | MEDLINE | ID: mdl-30988103

RESUMO

A 47-year-old HIV-positive man with good immune and virological status presented with chronic multiple enlarged lymph nodes, lung disease and eosinophilia. Radiologic tests showed enlarged cervical, thoracic and axillary lymph nodes, with interstitial lung damage. After several non-specific histologic studies, an elevated serum IgG4 level led us to request immunohistochemistry of a lymph node sample. The test confirmed the diagnosis of IgG4-related disease.


Assuntos
Dispneia/etiologia , Infecções por HIV/fisiopatologia , Doença Relacionada a Imunoglobulina G4/diagnóstico , Doenças Pulmonares Intersticiais/diagnóstico , Linfonodos/patologia , Azatioprina/uso terapêutico , Dispneia/imunologia , Dispneia/fisiopatologia , Glucocorticoides/uso terapêutico , Infecções por HIV/imunologia , Humanos , Imunoglobulina G/sangue , Doença Relacionada a Imunoglobulina G4/fisiopatologia , Imuno-Histoquímica , Imunossupressores/uso terapêutico , Doenças Pulmonares Intersticiais/imunologia , Doenças Pulmonares Intersticiais/fisiopatologia , Masculino , Pessoa de Meia-Idade , Prednisona/uso terapêutico , Tomografia Computadorizada por Raios X , Resultado do Tratamento
9.
Chin Med J (Engl) ; 132(11): 1283-1292, 2019 Jun 05.
Artigo em Inglês | MEDLINE | ID: mdl-31021982

RESUMO

BACKGROUNDS: Hypersensitivity pneumonitis (HP) is an immune-mediated interstitial lung disease (ILD) that develops in response to the inhalation of various antigens. The clinical pathologies are very complex and undetermined. The clinical features and outcomes of HP have not been fully elucidated. The aim of this study was to analyze the incidence, clinical features, and outcomes of HP patients and construct a simple clinical model for diagnosing chronic HP (CHP). METHODS: The cohort study included 101 patients with HP admitted to the Nanjing Drum Tower Hospital from January 2009 to December 2017. The patients were categorized into acute HP (AHP, n = 72) and CHP (n = 29) groups according to the updated international criteria. The clinical, imaging, treatment, and follow-up data were retrospectively reviewed. All patients were followed up until December 31, 2017. Statistical analysis was performed, and a clinical scoring system for CHP was constructed by SPSS 20.0 software. RESULTS: The incidence of HP was 2.4% in ILD inpatients in our center. Patients in the CHP group were older (t = -2.212, P = 0.029), had more smokers (χ = 8.428, P = 0.004), and longer duration of symptoms (t = -4.852, P < 0.001) than those in the AHP group. Weight loss, crackles, digital clubbing, and cyanosis were more common in the CHP group than those in the AHP group (χ = 5.862, P < 0.001; χ = 8.997, P = 0.003; χ = 11.939, P = 0.001; and χ = 4.025, P = 0.045, respectively). On chest high-resolution computed tomography (HRCT), reticular patterns, traction bronchiectasis, and accompanying honeycombing were more common in CHP cases than those in AHP cases (χ = 101.000, P < 0.001; χ = 32.048, P < 0.001; and χ = 36.568, P < 0.001, respectively). The clinical scoring system for CHP was established based on the clinical variables (age [A], duration of symptoms [D], smoking history [S], unidentified exposure [U], and chest HRCT [C]; ADSUC) (area under the curve 0.935, 95% confidence interval: 0.883-0.987, P < 0.001). Eleven patients (15.3%) in the AHP group developed CHP, and unidentified exposure was an independent risk factor for the progression of disease (P = 0.038). The survival of patients with CHP, smoking history, unidentified antigens and fibrosis on Chest HRCT were significantly worse (P = 0.011, P = 0.001, P = 0.005, and P = 0.011, respectively) by Kaplan-Meier analysis. Cox multivariate regression analysis revealed that unidentified exposure and total lung volume (TLC pred%) were independent prognostic predictors for HP patients (P = 0.017 and P = 0.017, respectively). CONCLUSIONS: The clinical features and outcomes of the CHP patients differ from those of the AHP patients. ADSUC is a simple and feasible clinical model for CHP. Unidentified exposure is an independent risk factor for the progression of AHP to CHP. Unidentified exposure and a low baseline TLC pred% are independent predictors for survival in HP patients.


Assuntos
Alveolite Alérgica Extrínseca/diagnóstico , Doenças Pulmonares Intersticiais/diagnóstico , Adulto , Idoso , Alveolite Alérgica Extrínseca/mortalidade , Alveolite Alérgica Extrínseca/fisiopatologia , China , Doença Crônica , Estudos de Coortes , Feminino , Fibrose , Humanos , Estimativa de Kaplan-Meier , Doenças Pulmonares Intersticiais/mortalidade , Doenças Pulmonares Intersticiais/fisiopatologia , Masculino , Pessoa de Meia-Idade , Modelos de Riscos Proporcionais , Estudos Retrospectivos , Capacidade Pulmonar Total/fisiologia
10.
Nagoya J Med Sci ; 81(1): 41-53, 2019 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-30962654

RESUMO

We evaluated the progression of interstitial lung disease (ILD) by three-dimensional curved high-resolution computed tomography (3D-cHRCT) at a constant depth from the chest wall and compare the results to pulmonary function test (PFT) results on a follow-up assessment. We reviewed the patients with ILD who underwent HRCT and concurrent PFTs at least twice from April 2008 to December 2014. Forty-five patients with ILD were enrolled. 3D-cHRCT images of the lung at various depths from the chest wall were reconstructed, and total area (TA), high-attenuation area (HAA) >-500 HU, and %HAA ([HAA/TA] × 100) were calculated. The TA, HAA, and %HAA ratios (follow-up to baseline) were assessed for use in the diagnosis of physiologically progressive ILD (defined as; forced vital capacity [FVC] ratio <0.9 or %diffusing capacity of the lung for carbon monoxide [%DLCO] ratio <0.85 [follow-up to baseline]). Of all ratios obtained from 3D-cHRCT images at 5-30mm depths, the %HAA ratio at 20-mm had the largest area under the receiver operating characteristic curve (0.815, 95 % confidence interval 0.677-0.953). By univariate logistic regression analysis, TA, HAA, and %HAA ratios at 20-mm showed significant correlations with physiologically progressive ILD. 3D-cHRCT imaging performed in parallel with the chest wall offers novel quantitative parameters that are useful for following ILD.


Assuntos
Doenças Pulmonares Intersticiais/diagnóstico por imagem , Doenças Pulmonares Intersticiais/diagnóstico , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Imagem Tridimensional/métodos , Doenças Pulmonares Intersticiais/fisiopatologia , Masculino , Pessoa de Meia-Idade , Capacidade de Difusão Pulmonar , Curva ROC , Testes de Função Respiratória , Estudos Retrospectivos , Parede Torácica/diagnóstico por imagem , Parede Torácica/patologia , Capacidade Vital/fisiologia
11.
Eur Respir Rev ; 28(151)2019 Mar 31.
Artigo em Inglês | MEDLINE | ID: mdl-30814139

RESUMO

Patients with certain types of fibrosing interstitial lung disease (ILD) are at risk of developing a progressive phenotype characterised by self-sustaining fibrosis, decline in lung function, worsening quality of life, and early mortality. It has been proposed that such progressive fibrosing ILDs, which show commonalities in clinical behaviour and in the pathogenetic mechanisms that drive progressive fibrosis, may be "lumped" together for the purposes of clinical research and, potentially, for treatment. At present, no drugs are approved for the treatment of ILDs other than nintedanib and pirfenidone for the treatment of idiopathic pulmonary fibrosis. For other progressive fibrosing ILDs, the mainstay of drug therapy is immunosuppression. However, it is postulated that, once the response to lung injury in fibrosing ILDs has reached the stage at which fibrosis has become progressive and self-sustaining, targeted antifibrotic therapy would be required to slow disease progression. Nintedanib, an intracellular inhibitor of tyrosine kinases, has shown antifibrotic, anti-inflammatory and vascular remodelling effects in several non-clinical models of fibrosis, irrespective of the trigger for the injury. Ongoing clinical trials will provide insight into the role of antifibrotic treatment with nintedanib or pirfenidone in the management of fibrosing ILDs with a progressive phenotype.


Assuntos
Doenças Pulmonares Intersticiais , Pulmão , Fibrose Pulmonar , Progressão da Doença , Nível de Saúde , Humanos , Indóis/uso terapêutico , Pulmão/efeitos dos fármacos , Pulmão/patologia , Pulmão/fisiopatologia , Doenças Pulmonares Intersticiais/tratamento farmacológico , Doenças Pulmonares Intersticiais/mortalidade , Doenças Pulmonares Intersticiais/patologia , Doenças Pulmonares Intersticiais/fisiopatologia , Fenótipo , Fibrose Pulmonar/tratamento farmacológico , Fibrose Pulmonar/mortalidade , Fibrose Pulmonar/patologia , Fibrose Pulmonar/fisiopatologia , Piridonas/uso terapêutico , Qualidade de Vida , Fármacos do Sistema Respiratório/uso terapêutico , Fatores de Risco , Índice de Gravidade de Doença , Resultado do Tratamento
12.
Med Sci Monit ; 25: 2087-2095, 2019 Mar 21.
Artigo em Inglês | MEDLINE | ID: mdl-30894506

RESUMO

BACKGROUND Obstructive sleep apnea (OSA) is often reported in connection with interstitial lung disease. As yet, there is insufficient data on the association of OSA severity parameters with lung involvement. We purposed to assess the frequency of OSA in our study group and to investigate the relationship between radiological involvement and OSA severity parameters. MATERIAL AND METHODS The study included 79 patients with interstitial lung disease who underwent spirometry, a carbon monoxide diffusion test (DLCO), high-resolution computed tomography, and polysomnography. The data were analyzed using SPSS 22 software. RESULTS Of the 79 patients, 53 patients (67.1%) had OSA, and there was a negative correlation between DLCO and the mean time spent with oxygen saturation levels below 90% (r=-0.686, P=0.001). The Warrick score was used as an indicator of the extent and severity of pulmonary involvement and was positively correlated with the apnea-hypopnea index, oxygen desaturation index, and the mean time spent with oxygen saturation below 90% (r=0.275, P=0.014; r=0.264 P=0.019; r=0.235, P=0.038). CONCLUSIONS In our study, a significant relationship was found between the Warrick score and the OSA severity parameters, as determined by polysomnography. Polysomnographic examinations might be useful, especially in patients with a Warrick score greater than 15, to avoid possible complications.


Assuntos
Doenças Pulmonares Intersticiais/fisiopatologia , Polissonografia/métodos , Apneia Obstrutiva do Sono/fisiopatologia , Adulto , Idoso , Gasometria , Índice de Massa Corporal , Feminino , Humanos , Doenças Pulmonares Intersticiais/metabolismo , Masculino , Pessoa de Meia-Idade , Oxigênio/metabolismo , Fatores de Risco , Índice de Gravidade de Doença , Apneia Obstrutiva do Sono/metabolismo , Espirometria/métodos , Tomografia Computadorizada por Raios X/métodos
13.
PLoS One ; 14(3): e0213444, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-30861018

RESUMO

OBJECTIVES: To evaluate interstitial lung disease associated with systemic sclerosis (SSc-ILD) and its changes during treatment by using quantitative analysis (QA) compared to semi-quantitative analysis (semiQA) of chest computed tomography (CT) scans. To assess the prognostic value of QA in predicting functional changes. MATERIALS AND METHODS: We retrospectively selected 35 consecutive patients with SSc-ILD with complete pulmonary functional evaluation, Doppler-echocardiography, immunological tests, and chest CT scan at both baseline and follow-up after immunosuppressive therapy. CT images were analyzed by two chest radiologists for semiQA and by a computational platform for texture analysis of ILD patterns (CALIPER) for QA. Concordance between semiQA and QA was tested. Traction bronchiectasis severity was scored. Analysis of ROC curves was performed. RESULTS: Seventy CT scans were analyzed and QA failed in 4/70 scans. Thus, the final population included 31/35 patients (51.3±12.1 years). QA had a weak-to-good concordance with semiQA (ICC reticular:0.275; ICC ground-glass:0.667) and QA correlated better than semiQA (r = -0.3 to -0.74 vs r = -0.3 to -0.4) with functional parameters. Both methods correlated with traction bronchiectases score and pulmonary artery diameter at CT. A pulmonary artery diameter ≥29mm distinguished patients with lower lung volumes and ILD extent greater than 39% (p<0.001). Changes in QA patterns during treatment were not accurate (AUC: 0.50 to 0.70; p>0.05) in predicting disease progression as assessed by functional parameters, whereas variation in total lung volume at QA accurately predicted changes in the composite functional respiratory endpoint with FVC% and DLco% (AUC = 0.74; 95%CI: 0.54 to 0.93; p = 0.03). CONCLUSIONS: Pulmonary QA of CT images can objectively quantify specific patterns of ILD changes during treatment in patients with SSc-ILD. Changes in QA patterns do not correlate with functional changes, but variation in total lung volume at QA accurately predicted changes in the composite functional respiratory endpoint with FVC% and DLco%. Pulmonary artery diameter at CT reflects the interstitial involvement, identifying patients with more severe prognosis.


Assuntos
Doenças Pulmonares Intersticiais/diagnóstico por imagem , Escleroderma Sistêmico/diagnóstico por imagem , Adulto , Bronquiectasia/diagnóstico por imagem , Feminino , Humanos , Estudos Longitudinais , Doenças Pulmonares Intersticiais/fisiopatologia , Doenças Pulmonares Intersticiais/terapia , Masculino , Pessoa de Meia-Idade , Prognóstico , Artéria Pulmonar/diagnóstico por imagem , Interpretação de Imagem Radiográfica Assistida por Computador , Testes de Função Respiratória , Estudos Retrospectivos , Rituximab/uso terapêutico , Escleroderma Sistêmico/fisiopatologia , Escleroderma Sistêmico/terapia , Tomografia Computadorizada por Raios X/estatística & dados numéricos
14.
Respir Res ; 20(1): 56, 2019 Mar 12.
Artigo em Inglês | MEDLINE | ID: mdl-30866948

RESUMO

BACKGROUND: Patients with fibrotic interstitial lung disease (ILD) are frequently physically inactive and many ILD subtypes are characterized by risk factors for myopathy; however, the importance of body composition, muscle strength, and physical performance in this population is largely unknown. METHODS: Patients were prospectively recruited from a specialized ILD clinic, baseline characteristics were collected from the clinical record, pulmonary function tests were performed per established protocols, and dyspnea was measured using the University of California San Diego Shortness of Breath Questionnaire. Dual-energy X-ray absorptiometry (DXA) was used to assess body composition; handgrip strength to determine muscle strength, and 4-m gait speed to measure physical performance. RESULTS: One hundred and fifteen patients with fibrotic ILD including 40 patients with idiopathic pulmonary fibrosis were recruited. The mean age was 69+/- 10 years in men (62% of the cohort), and 66+/- 9 years in women, with mild and moderate reduction in FVC and DLCO, respectively, for both sexes. ILD severity (measured by FVC %-predicted, DLCO %-predicted, or the Composite Physiologic Index in separate models) significantly predicted muscle mass and percent body fat including with adjustment for age, sex, and weight. ILD severity was associated with grip strength and gait speed independent from body composition. CONCLUSIONS: ILD severity has an important impact on body composition, particularly in men. Future studies are needed to confirm and further explore the possibility of additional pathways through which ILD directly impacts limb muscle function and physical performance.


Assuntos
Composição Corporal/fisiologia , Força da Mão/fisiologia , Fibrose Pulmonar Idiopática/fisiopatologia , Doenças Pulmonares Intersticiais/fisiopatologia , Força Muscular/fisiologia , Desempenho Físico Funcional , Idoso , Estudos de Coortes , Estudos Transversais , Feminino , Humanos , Fibrose Pulmonar Idiopática/diagnóstico , Doenças Pulmonares Intersticiais/diagnóstico , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos
15.
J Clin Ultrasound ; 47(5): 261-266, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-30729529

RESUMO

PURPOSE: Lung ultrasound (LUS) examination is used to evaluate patients with acute respiratory failure. The physiological LUS pattern during labor in healthy parturients has not been well described. The aim of this study was to evaluate the LUS pattern in a cohort of healthy women during uncomplicated labor. METHODS: We used the 8-point LUS assessment protocol and investigated lung sliding, A-lines, B-lines, interstitial syndrome, lung consolidation, and pleural effusion according to the International Consensus Document with two additional supradiaphragmatic projections. All patients were screened twice; once during the first stage of labor and again within 2 hours after delivery. RESULTS: We included 24 patients in this study from February 2014 to August 2015. A total of 480 LUS records were retained for further analysis. Overall, 16 of 24 patients (67%) had at least one positive region (three or more B-lines) during the peridelivery LUS evaluation. Interstitial syndrome was detected in five patients (21%). There were no differences in A-line (P = 0.38) or B-line (P = 0.68) prevalence between LUS examinations before and after delivery. CONCLUSIONS: Women in uncomplicated labor can present abnormal LUS findings, which may affect the interpretation of LUS results in patients with respiratory deterioration. Further studies should address this topic in larger cohort of patients.


Assuntos
Trabalho de Parto/fisiologia , Doenças Pulmonares Intersticiais/diagnóstico por imagem , Pulmão/diagnóstico por imagem , Complicações do Trabalho de Parto/diagnóstico por imagem , Adulto , Feminino , Humanos , Pulmão/fisiologia , Doenças Pulmonares Intersticiais/fisiopatologia , Complicações do Trabalho de Parto/fisiopatologia , Projetos Piloto , Gravidez , Estudos Prospectivos , Valores de Referência , Ultrassonografia
17.
BMC Pulm Med ; 19(1): 50, 2019 Feb 26.
Artigo em Inglês | MEDLINE | ID: mdl-30808321

RESUMO

OBJECTIVE: Long-term oxygen therapy (LTOT) during 15 h/day or more prolongs survival in patients with chronic obstructive pulmonary disease (COPD) and severe hypoxemia. No randomized controlled trial has evaluated the net effects (benefits or harms) from LTOT 24 h/day compared with 15 h/day or the effect in conditions other than COPD. We describe a multicenter, national, phase IV, non-superiority, registry-based, randomized controlled trial (R-RCT) of LTOT prescribed 24 h/day compared with 15 h/day. The primary endpoint is all-cause-mortality at 1 year. Secondary endpoints include cause-specific mortality, hospitalizations, health-related quality of life, symptoms, and outcomes in interstitial lung disease. METHODS/DESIGN: Patients qualifying for LTOT are randomized to LTOT 24 h/day versus 15 h/day during 12 months using the Swedish Register for Respiratory Failure (Swedevox). Planned sample size in this pragmatic study is 2126 randomized patients. Clinical follow-up and concurrent treatments are according to routine clinical practice. Mortality, hospitalizations, and incident diseases are assessed using national Swedish registries with expected complete follow-up. Patient-reported outcomes are assessed using postal questionnaire at 3 and 12 months. DISCUSSION: The R-RCT approach combines the advantages of a prospective randomized trial and large clinical national registries for enrollment, allocation, and data collection, with the aim of improving the evidence-based use of LTOT. TRIAL REGISTRATION: Clinical Trial registered with www.clinicaltrials.gov , Title: REgistry-based Treatment Duration and Mortality in Long-term OXygen Therapy (REDOX); ID: NCT03441204.


Assuntos
Doenças Pulmonares Intersticiais/terapia , Oxigenoterapia/métodos , Doença Pulmonar Obstrutiva Crônica/terapia , Sistema de Registros , Nível de Saúde , Hospitalização/estatística & dados numéricos , Humanos , Doenças Pulmonares Intersticiais/mortalidade , Doenças Pulmonares Intersticiais/fisiopatologia , Doença Pulmonar Obstrutiva Crônica/mortalidade , Doença Pulmonar Obstrutiva Crônica/fisiopatologia , Qualidade de Vida , Método Simples-Cego , Taxa de Sobrevida , Suécia , Fatores de Tempo , Resultado do Tratamento
18.
Intern Med ; 58(12): 1703-1712, 2019 Jun 15.
Artigo em Inglês | MEDLINE | ID: mdl-30799358

RESUMO

Objective Biological disease-modifying anti-rheumatic drugs (bDMARDs) represent an important advance in alleviating rheumatoid arthritis (RA), but their effect on rheumatic airway disease (AD) and interstitial lung disease (ILD) is still unclear. This study was performed to evaluate the association of the use of different bDMARDs with new-onset or worsening of RA-AD/ILD. Methods We performed a retrospective cohort study of RA patients who received bDMARDs and assessed their AD/ILD before and after drug initiation in our hospital over the past 10 years. We evaluated the serial changes in computed tomography (CT), classified patients according to AD/ILD progression, and analyzed associations between clinical characteristics and outcomes. Results We enrolled 49 patients. Thirty patients received tumor necrosis factor inhibitors (TNFis), 12 received abatacept (ABT), and the remaining 7 received tocilizumab (TCZ). Seventeen patients had ILD, 10 had AD, and 6 had both AD and ILD before the initiation of bDMARDs. New emergence or exacerbation of AD/ILD was observed in 18 patients after drug initiation, while the remaining 31 remained stable or improved. Multiple logistic regression analyses revealed that pre-existing AD was an independent risk factor against the emergence or exacerbation of RA-AD/ILD, and ABT use was a protective factor against it. Conclusion Our study showed that pre-existing RA-AD is associated with future worsening of RA-AD/ILD, and ABT over other bDMARDs was associated with a better prognosis. Future studies to confirm our results are needed.


Assuntos
Antirreumáticos/uso terapêutico , Artrite Reumatoide/tratamento farmacológico , Artrite Reumatoide/epidemiologia , Doenças Pulmonares Intersticiais/epidemiologia , Doenças Pulmonares Intersticiais/fisiopatologia , Abatacepte/uso terapêutico , Idoso , Anticorpos Monoclonais Humanizados/uso terapêutico , Antirreumáticos/administração & dosagem , Antirreumáticos/efeitos adversos , Artrite Reumatoide/fisiopatologia , Progressão da Doença , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Prognóstico , Estudos Retrospectivos , Fatores de Risco , Tomografia Computadorizada por Raios X , Fator de Necrose Tumoral alfa/uso terapêutico
19.
Arthritis Rheumatol ; 71(6): 972-982, 2019 06.
Artigo em Inglês | MEDLINE | ID: mdl-30624031

RESUMO

OBJECTIVE: Interstitial lung disease (ILD) in systemic sclerosis (SSc) runs a highly variable course, and prediction tools are highly desired. The aim of this study was to assess the diagnostic and prognostic performance of 4 candidate serum biomarkers for SSc-associated ILD. METHODS: Serum samples from a combined cohort of SSc patients (from Paris, France and Oslo, Norway; n = 427) were analyzed by enzyme-linked immunosorbent assay for concentrations of lung epithelial-derived surfactant protein D (SP-D), Krebs von den Lungen 6 glycoprotein (KL-6), CCL18, and OX40 ligand (OX40L). Lung fibrosis was measured by high-resolution computed tomography and pulmonary function tests. Associations of these candidate biomarkers with baseline disease involvement and prediction of disease progression over time (mean ± SD follow-up 3.2 ± 4.4 years) were investigated. RESULTS: In SSc patients at baseline, serum levels of KL-6 correlated with the forced vital capacity (FVC) (r = -0.317, P < 0.001), diffusing capacity for carbon monoxide (r = -0.335, P < 0.001), and extent of lung fibrosis (r = 0.551, P < 0.001). In multivariate analyses, serum levels of KL-6 and SP-D, but not CCL18 and OX40L, were associated with lung fibrosis (odds ratio [OR] 2.41, 95% confidence interval [95% CI] 1.43-4.07 [P = 0.001] and OR 3.15, 95% CI 1.81-5.48 [P < 0.001], respectively). In SSc patients with ILD at baseline, longitudinal, multivariate analyses showed that CCL18 serum levels were an independent predictor of a >10% decrease in the FVC (hazard ratio [HR] 2.90, 95% CI 1.25-6.73; P = 0.014) and de novo development of extensive disease (HR 3.71, 95% CI 1.02-13.52; P = 0.048). Matrix-based logistic regression models for the diagnosis and prognosis of SSc-associated ILD were constructed, and these models discriminated 3 groups of risk (mild, moderate, or high) for the diagnosis or worsening of lung fibrosis according to the serum levels of SP-D (for diagnosis) and serum levels of CCL18 (for progression of disease). CONCLUSION: These results show that SP-D is a relevant diagnostic biomarker for SSc-associated ILD, whereas KL-6 could be used to assess the severity of lung fibrosis. CCL18 appears to be a potential predictive marker for progression of ILD in SSc.


Assuntos
Quimiocinas CC/sangue , Doenças Pulmonares Intersticiais/sangue , Mucina-1/sangue , Proteína D Associada a Surfactante Pulmonar/sangue , Receptores OX40/sangue , Escleroderma Sistêmico/sangue , Idoso , Biomarcadores/sangue , Feminino , França , Humanos , Pulmão/diagnóstico por imagem , Pulmão/fisiopatologia , Doenças Pulmonares Intersticiais/diagnóstico , Doenças Pulmonares Intersticiais/etiologia , Doenças Pulmonares Intersticiais/fisiopatologia , Masculino , Pessoa de Meia-Idade , Noruega , Prognóstico , Modelos de Riscos Proporcionais , Capacidade de Difusão Pulmonar , Testes de Função Respiratória , Escleroderma Sistêmico/complicações , Tomografia Computadorizada por Raios X , Capacidade Vital
20.
Respir Res ; 20(1): 13, 2019 Jan 18.
Artigo em Inglês | MEDLINE | ID: mdl-30658650

RESUMO

BACKGROUND: Systemic sclerosis (SSc) is a rare connective tissue disease with a heterogeneous clinical course. Interstitial lung disease (ILD) is a common manifestation of SSc and a leading cause of death. MAIN BODY: All patients newly diagnosed with SSc should receive a comprehensive clinical evaluation, including assessment of respiratory symptoms, a high-resolution computed tomography (HRCT) scan of the chest, and pulmonary function tests. ILD can develop in any patient with SSc, including those with pulmonary hypertension, but the risk is increased in those with diffuse (rather than limited) cutaneous SSc, those with anti-Scl-70/anti-topoisomerase I antibody, and in the absence of anti-centromere antibody. While it can occur at any time, the risk of developing ILD is greatest early in the course of SSc, so patients should be monitored closely in the first few years after diagnosis. An increased extent of lung fibrosis on HRCT and a low forced vital capacity (FVC) are predictors of early mortality. While not all patients will require treatment, current approaches to the treatment of progressive SSc-ILD focus on immunosuppressant therapies, including cyclophosphamide and mycophenolate mofetil. In patients with severe and/or rapidly progressive disease, both haematopoietic stem cell transplantation (HSCT) and lung transplantation have been successfully used. A number of medications, including the two drugs approved for the treatment of idiopathic pulmonary fibrosis (IPF), are under active investigation as potential new therapies for SSc-ILD. CONCLUSIONS: Physicians managing patients with SSc should maintain a high level of suspicion and regularly monitor for ILD, particularly in the first few years after diagnosis.


Assuntos
Doenças Pulmonares Intersticiais/fisiopatologia , Doenças Pulmonares Intersticiais/terapia , Escleroderma Sistêmico/fisiopatologia , Escleroderma Sistêmico/terapia , Anti-Inflamatórios/uso terapêutico , Transplante de Células-Tronco Hematopoéticas/métodos , Transplante de Células-Tronco Hematopoéticas/tendências , Humanos , Doenças Pulmonares Intersticiais/epidemiologia , Transplante de Pulmão/métodos , Transplante de Pulmão/tendências , Ensaios Clínicos Controlados Aleatórios como Assunto/métodos , Escleroderma Sistêmico/epidemiologia
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