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1.
BMC Pulm Med ; 19(1): 247, 2019 Dec 16.
Artigo em Inglês | MEDLINE | ID: mdl-31842848

RESUMO

BACKGROUND: The intensity and frequency of cough remain unclear in interstitial lung disease (ILD). The aim of this study was to evaluate the intensity and frequency of cough in idiopathic interstitial pneumonias (IIPs), connective tissue disease-associated interstitial lung disease (CTD-ILD), and chronic hypersensitivity pneumonia (CHP), and examine their associations with clinical indices. METHODS: In this cross-sectional study, the intensity and frequency of cough were evaluated using a 100-mm visual analogue scale. Scores on the Leicester Cough Questionnaire, chronic dyspnoea scale, and a frequency scale for symptoms of gastro-oesophageal reflux disease (FSSG) were collected. The correlations of cough intensity and frequency with potential predictor variables were tested using bivariate and multiple logistic regression analysis. RESULTS: The study included 70 patients with IIPs, 49 with CTD-ILD, and 10 with CHP. Patients with IIPs had the most severe cough intensity among the three patient groups. In patients with IIPs, both the intensity and frequency of cough were negatively associated with the diffusing capacity of the lung for carbon monoxide and positively with the Composite Physiologic Index (CPI). In CTD-ILD, both the intensity and frequency of cough were correlated with a higher FSSG score. In multivariate analysis of patients with ILD, IIPs and the FSSG score were independently associated with both components of cough, and CPI tended to be independently associated with cough frequency. Finally, we examined the features of the differences between cough intensity and frequency in all patients with ILD. Patients in whom cough frequency was predominant had a greater impairment of health status relative to other patients. CONCLUSIONS: Cough intensity was greater in IIPs than in other ILDs. Different clinical indices were associated with patient-reported cough intensity and frequency according to the subtype of ILD. Cough frequency was more strongly associated with health status than was cough intensity. These findings suggest that medical staff could manage patients with ILD by considering cough-related factors when assessing the intensity and frequency of cough.


Assuntos
Alveolite Alérgica Extrínseca/complicações , Tosse/etiologia , Pneumonias Intersticiais Idiopáticas/complicações , Doenças Pulmonares Intersticiais/complicações , Idoso , Alveolite Alérgica Extrínseca/fisiopatologia , Tosse/fisiopatologia , Estudos Transversais , Feminino , Humanos , Pneumonias Intersticiais Idiopáticas/fisiopatologia , Modelos Logísticos , Doenças Pulmonares Intersticiais/fisiopatologia , Masculino , Pessoa de Meia-Idade , Análise Multivariada , Autorrelato
2.
BMC Pulm Med ; 19(1): 192, 2019 Oct 31.
Artigo em Inglês | MEDLINE | ID: mdl-31672127

RESUMO

BACKGROUND: Connective tissue disease-associated interstitial lung disease (CTD-ILD) is associated with reduced quality of life and poor prognosis. Prior studies have not identified a consistent combination of variables that accurately predict prognosis in CTD-ILD. The objective of this study was to identify baseline demographic and clinical characteristics that are associated with progression and mortality in CTD-ILD. METHODS: Patients were retrospectively identified from an adult CTD-ILD clinic. The predictive significance of baseline variables on serial forced vital capacity (FVC), diffusion capacity (DLCO), and six-minute walk distance (6MWD) was assessed using linear mixed effects models, and Cox regression analysis was performed to assess impact on mortality. RESULTS: 359 patients were included in the study. Median follow-up time was 4.0 (IQR 1.5-7.6) years. On both unadjusted and multivariable analysis, male sex and South Asian ethnicity were associated with decline in FVC. Male sex, positive smoking history, and diagnosis of systemic sclerosis (SSc) vs. other CTD were associated with decline in DLCO. Male sex and usual interstitial pneumonia (UIP) pattern predicted decline in 6MWD. There were 85 (23.7%) deaths. Male sex, older age, First Nations ethnicity, and a diagnosis of systemic sclerosis vs. rheumatoid arthritis were predictors of mortality on unadjusted and multivariable analysis. CONCLUSION: Male sex, older age, smoking, South Asian or First Nations ethnicity, and UIP pattern predicted decline in lung function and/or mortality in CTD-ILD. Further longitudinal studies may add to current clinical prediction models for prognostication in CTD-ILD.


Assuntos
Doenças do Tecido Conjuntivo/complicações , Pneumonias Intersticiais Idiopáticas/mortalidade , Pneumonias Intersticiais Idiopáticas/fisiopatologia , Pulmão/fisiopatologia , Adulto , Idoso , Artrite Reumatoide/complicações , Canadá/epidemiologia , Bases de Dados Factuais , Demografia , Progressão da Doença , Feminino , Humanos , Pneumonias Intersticiais Idiopáticas/etiologia , Fibrose Pulmonar Idiopática/fisiopatologia , Masculino , Pessoa de Meia-Idade , Análise Multivariada , Prognóstico , Estudos Retrospectivos , Escleroderma Sistêmico/complicações , Análise de Sobrevida , Tomografia Computadorizada por Raios X
3.
Respir Med ; 152: 44-50, 2019 06.
Artigo em Inglês | MEDLINE | ID: mdl-31128609

RESUMO

BACKGROUND AND PURPOSE: Patients with anti-aminoacyl-tRNA synthetase (ARS) antibodies frequently experience complications of interstitial pneumonia (ARS-IP), and the computed tomography (CT) of ARS-IP frequently shows nonspecific interstitial pneumonia (NSIP) pattern. The CT pattern of ARS-IP might be different from that of idiopathic IP. However, the clinical differences in patients with ARS-IP and idiopathic IP showing the similar CT patterns have not yet been well studied. The objective of this study was to evaluate the clinical differences between patients with ARS-NSIP and idiopathic NSIP (I-NSIP). METHODS: Two groups of 34 patients each, with ARS-NSIP and I-NSIP, who visited Hiroshima University Hospital between January 2005 and December 2017, were enrolled. Clinical features and outcomes were retrospectively compared between the two groups. RESULTS: The ARS-NSIP group included more female patients and significantly younger patients than the I-NSIP group. The percentage of lymphocytes in bronchoalveolar lavage fluid (BALF) was significantly higher, and the CD4/CD8 ratio in BALF was significantly lower in the ARS-NSIP group compared with the I-NSIP group. The proportion of patients with traction bronchiectasis detected by CT was significantly higher in I-NSIP compared with ARS-NSIP. The number of patients who received corticosteroid and/or immunosuppressant therapy was significantly larger in the ARS-NSIP group than in the I-NSIP group. In addition, the patients in the I-NSIP group who underwent the immunosuppressive therapy demonstrated shorter survival than those who underwent no treatment; this tendency was not observed in the ARS-NSIP group. The 10-year survival rate of patients in the ARS-NSIP group was significantly higher than that of patients in the I-NSIP group (91.8% vs. 43.0%; log-rank, p = 0.012). The multivariate survival analysis revealed that positive anti-ARS antibody was an independent favorable prognostic factor in the patients with NSIP (OR, [95% CI]:0.12 [0.02-0.55], p = 0.013). CONCLUSIONS: Patients with ARS-NSIP had a significantly better prognosis than those with I-NSIP; this may be associated with the sensitivity to immunosuppressive therapies, and the different findings of BALF and HRCT between the two groups.


Assuntos
Aminoacil-tRNA Sintetases/antagonistas & inibidores , Líquido da Lavagem Broncoalveolar/imunologia , Pneumonias Intersticiais Idiopáticas/imunologia , Doenças Pulmonares Intersticiais/imunologia , Corticosteroides/uso terapêutico , Adulto , Idoso , Idoso de 80 Anos ou mais , Aminoacil-tRNA Sintetases/imunologia , Autoanticorpos/imunologia , Bronquiectasia/diagnóstico por imagem , Bronquiectasia/epidemiologia , Relação CD4-CD8/métodos , Feminino , Humanos , Pneumonias Intersticiais Idiopáticas/diagnóstico por imagem , Pneumonias Intersticiais Idiopáticas/tratamento farmacológico , Pneumonias Intersticiais Idiopáticas/fisiopatologia , Imunossupressores/uso terapêutico , Japão/epidemiologia , Doenças Pulmonares Intersticiais/diagnóstico por imagem , Doenças Pulmonares Intersticiais/tratamento farmacológico , Doenças Pulmonares Intersticiais/fisiopatologia , Linfócitos/citologia , Masculino , Pessoa de Meia-Idade , Prognóstico , Estudos Retrospectivos , Análise de Sobrevida , Taxa de Sobrevida , Tomografia Computadorizada por Raios X/métodos
4.
PLoS One ; 14(3): e0214441, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-30921412

RESUMO

BACKGROUND AND OBJECTIVE: The idiopathic interstitial pneumonias (IIPs) are diffuse parenchymal lung disorders that are associated with substantial morbidity and mortality. Early diagnosis and disease stratification of IIP patients are important because these are related with the treatment and prognosis. Idiopathic pulmonary fibrosis (IPF) and nonspecific interstitial pneumonia (NSIP) are two major distinctive pathologic patterns of pulmonary fibrosis. We researched the application of the fluid-structure interaction (FSI) to the respiratory system and compared the pulmonary acinus mechanics and functions in healthy and IIP models. METHODS: The human pulmonary alveolus is idealized by a three-dimensional honeycomb-like geometry, and a fluid-structure interaction analysis is performed to study the normal and diseased breathing mechanics. The computational domain consists of two generations of alveolar ducts within the pulmonary acinus, with alveolar geometries approximated as closely packed 14-sided polygons. FINDINGS: In a normal breathing cycle, the flow rate of the healthy model is significantly larger than that of the NSIP and IPF models. Similar trends are observed for the volume change and the maximum pressure drop. The flow rate and the volume change of the NSIP are almost the same as those of IPF. The maximum pressure drop of NSIP is 5.5% larger than that of IPF. There is a 47% decrease in the pulmonary acinus compliance for the NSIP and IPF compared with that of the healthy model. The acinus resistances of NSIP and IPF are higher than those of the healthy lung by 6.4~11.2%. In particular, the pulmonary acinus resistance of the NSIP lung is higher than that of the IPF lung by 4.5%. CONCLUSIONS: Our study demonstrates the differences of air flow and lung function in the pulmonary acinus between the healthy and the IIP models. These changes in the lung are important considerations for early diagnosis and disease stratification in patients. Patient-based geometry can to be included in the computational models in future studies.


Assuntos
Células Acinares/patologia , Ar , Pneumonias Intersticiais Idiopáticas/patologia , Pneumonias Intersticiais Idiopáticas/fisiopatologia , Pulmão/patologia , Pulmão/fisiopatologia , Modelos Biológicos , Pneumonias Intersticiais Idiopáticas/complicações , Fibrose Pulmonar Idiopática/complicações
5.
Kaohsiung J Med Sci ; 35(6): 365-372, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-30913371

RESUMO

Interstitial lung disease (ILD) is a common pulmonary manifestation of connective tissue diseases (CTD). Prognostic effect of radiological usual interstitial pneumonia (UIP) pattern in CTD-associated interstitial lung disease (CTD-ILD) is unknown. This study aimed to investigate the disease progression and mortality of patients with CTD-ILD and idiopathic interstitial pneumonias (IIP) including idiopathic pulmonary fibrosis (IPF) and idiopathic nonspecific interstitial pneumonia and the prognostic impact of the radiological UIP pattern on both disease groups. The medical records of 91 patients (55 with CTD-ILD and 36 with IIP) diagnosed with ILD at pulmonary medicine department, Faculty of Medicine, Gazi University from 2004 to 2014 were retrospectively reviewed. Patients included whose baseline high-resolution computed tomography (HRCT) scans showed either a UIP or non-UIP pattern. While 67.3% (n = 37) of CTD-ILD patients possessed UIP pattern, 38.9% (n = 14) of IIP patients had UIP pattern in HRCT. Respiratory functions including the forced expiratory volume in the first second (FEV1 ), functional vital capacity (FVC), and transfer coefficient for carbon monoxide (diffusing capacity of the lung for carbon monoxide [DLCO]) of IIP group at the time of diagnosis were significantly lower than CTD-ILD group (P = .007, P = .002, and P = .019, respectively). There was no significant survival difference between CTD-ILD and IIP by using the log-rank test (P = .76). Multivariate analysis revealed that UIP pattern in HRCT (Hazard ratio: 1.85; 95% Confidence interval = 1.14-3; P = .013), annual FVC (Hazard ratio: 0.521; 95% Confidence interval = 0.32-0.84; P = .007), and annual DLCO declines (Hazard ratio: 0.943; 95% Confidence interval = 0.897-0.991; P = .02) were independent risk factors for mortality in both CTD-ILD and IIP groups. We found that UIP pattern in HRCT and annual losses in respiratory functions were the main determinants of prognosis of ILDs either idiopathic or CTD-associated.


Assuntos
Pneumonias Intersticiais Idiopáticas/fisiopatologia , Fibrose Pulmonar Idiopática/fisiopatologia , Doenças Pulmonares Intersticiais/fisiopatologia , Pulmão/fisiopatologia , Idoso , Monóxido de Carbono/metabolismo , Progressão da Doença , Feminino , Volume Expiratório Forçado , Humanos , Pneumonias Intersticiais Idiopáticas/diagnóstico por imagem , Pneumonias Intersticiais Idiopáticas/mortalidade , Fibrose Pulmonar Idiopática/diagnóstico por imagem , Fibrose Pulmonar Idiopática/mortalidade , Pulmão/diagnóstico por imagem , Doenças Pulmonares Intersticiais/diagnóstico por imagem , Doenças Pulmonares Intersticiais/mortalidade , Masculino , Pessoa de Meia-Idade , Análise Multivariada , Prognóstico , Estudos Retrospectivos , Análise de Sobrevida , Tomografia Computadorizada por Raios X , Capacidade Vital
6.
Respir Med ; 146: 23-27, 2019 01.
Artigo em Inglês | MEDLINE | ID: mdl-30665514

RESUMO

INTRODUCTION: Idiopathic interstitial pneumonias (IIP) are diffuse lung diseases whose cause is unknown and often present with features of autoimmunity despite not meeting criteria for a connective tissue disease (CTD). Recent studies suggest that anti-RNA binding protein (anti-RBP) antibodies, which include anti-SSA, anti-SSB, anti-Sm, and anti-RNP, play a role in the loss of immune tolerance and severity of pulmonary hypertension (PH) in CTDs. We hypothesized that anti-RBP positive (RBP+) subjects would have worse measures of lung function, radiographic findings, PH, and survival than anti-RBP negative (RBP-) subjects. METHODS: Subjects with both IIP and serologies for review were identified retrospectively and stratified based on anti-RBP antibody seropositivity. Baseline cohort characteristics, pulmonary function tests (PFT), ambulatory oxygen requirement, radiographic characteristics, markers of PH, and transplant-free survival were compared between anti-RBP positive and negative groups. RESULTS: Five hundred twenty patients with IIP were identified, of which ten percent (n = 53) were anti-RBP positive. RBP+ as compared to RBP- subjects had significantly worse PFTs as indicated by FEV1 (59.6 vs. 64.9, p = 0.046) and FVC (71.6 vs. 78.8, p = 0.018). There was a higher prevalence of radiographic honeycombing (49.1% vs. 38.3%, p = 0.006) and emphysema (22.6% vs. 5.1%, p < 0.001) in the RBP+ group despite no difference in smoking history. The Pulmonary Artery-Aorta ratio was also larger in the RBP+ group (0.93 vs. 0.88, p = 0.040). There was no difference in transplant-free survival between groups (log rank = 0.912). CONCLUSION: Anti-RBP+ IIP patients may have worse lung function, increased chest radiographic abnormalities, and PH compared with those without these antibodies.


Assuntos
Proteínas de Transporte/metabolismo , Pneumonias Intersticiais Idiopáticas/sangue , Pneumonias Intersticiais Idiopáticas/epidemiologia , Pneumonias Intersticiais Idiopáticas/fisiopatologia , Idoso , Anticorpos Antinucleares/imunologia , Autoimunidade/imunologia , Proteínas de Transporte/sangue , Doenças do Tecido Conjuntivo/epidemiologia , Feminino , Humanos , Hipertensão Pulmonar/fisiopatologia , Pneumonias Intersticiais Idiopáticas/diagnóstico por imagem , Pulmão/irrigação sanguínea , Pulmão/diagnóstico por imagem , Pulmão/fisiopatologia , Masculino , Pessoa de Meia-Idade , Prevalência , Radiografia Torácica/métodos , Testes de Função Respiratória/métodos , Estudos Retrospectivos , Tomografia Computadorizada por Raios X
7.
Intern Med ; 57(13): 1827-1832, 2018 Jul 01.
Artigo em Inglês | MEDLINE | ID: mdl-29434143

RESUMO

Objective Although lung squamous cell carcinoma (SCC) accounts for 20-30% of lung cancer cases, new treatment options are limited. The CA031 study showed that nanoparticle albumin-bound-paclitaxel (nab-PTX) plus carboplatin produced a significantly higher overall response rate (41%) than solvent-based paclitaxel plus carboplatin in patients with lung SCC. However, the safety and efficacy of combination chemotherapy of nab-PTX and carboplatin has not yet been established for patients with concurrent lung SCC and idiopathic interstitial pneumonias (IIPs). The aim of this study was to assess the safety and efficacy profiles of nab-PTX and carboplatin in patients with lung SCC and concurrent IIPs. Methods Eight patients with inoperable-stage lung SCC and IIPs were treated with nab-PTX plus carboplatin in a first-line setting between June 2013 and December 2016. One of the eight was a woman, and the median age was 77 (range=72-80) years. Their clinical outcomes, including chemotherapy-associated acute exacerbation of IIPs, were retrospectively investigated. Results The overall response rate was 50%, the median progression-free survival time was 5.6 months, and the median overall survival time was 8.1 months. No patients experienced chemotherapy-related exacerbation of IIPs in the first-line treatment with nab-PTX plus carboplatin. However, IIPs worsened in two of four patients who received second-line chemotherapy. Conclusion Combination chemotherapy of nab-PTX and carboplatin may be an effective and safe treatment option for patients with inoperable lung SCC with IIPs. To confirm this, a large-scale prospective study is needed.


Assuntos
Albuminas/uso terapêutico , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Carboplatina/uso terapêutico , Carcinoma Pulmonar de Células não Pequenas/tratamento farmacológico , Carcinoma Pulmonar de Células não Pequenas/fisiopatologia , Carcinoma de Células Escamosas/tratamento farmacológico , Pneumonias Intersticiais Idiopáticas/fisiopatologia , Neoplasias Pulmonares/tratamento farmacológico , Paclitaxel/uso terapêutico , Idoso , Idoso de 80 Anos ou mais , Comorbidade , Feminino , Humanos , Neoplasias Pulmonares/fisiopatologia , Masculino , Estudos Prospectivos , Estudos Retrospectivos
8.
Respir Investig ; 56(1): 40-47, 2018 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-29325680

RESUMO

BACKGROUND: The purpose of this study was to clarify the clinico-radio-pathological characteristics and prognostic factors of unclassifiable-idiopathic interstitial pneumonias (U-IIPs) diagnosed by surgical lung biopsy. METHODS: Among 86 patients with interstitial pneumonia who underwent surgical lung biopsy from January 2005 to September 2013, 33 (38.4%; 16 male patients; mean age, 64.4 ± 8.8 years) were diagnosed with U-IIPs. They were subsequently categorized into rapidly progressive (n = 7), slowly progressive (n = 7), and stable (n = 19) groups based on the decrease of the percent predicted forced vital capacity or percent predicted diffusing capacity of the lung carbon monoxide and the occurrence of acute exacerbation. The clinico-radio-pathological features and survival rates of the patients who were followed up for at least 3 years were examined. These cases were reevaluated retrospectively by multidisciplinary discussion. RESULTS: The rapidly progressive group had a significantly poorer prognosis than that of the other groups (p < 0.0001). Although there were no significant pattern differences on the chest high-resolution computed tomography, the fibrosis scores were significantly higher in the rapidly progressive group (p = 0.002). Furthermore, the percentage of fibroblastic foci assessed by the pathological analysis was also significantly higher in the rapidly progressive group (p = 0.006). Nine (27.3%) patients developed connective tissue diseases during follow-up. CONCLUSIONS: The radiologic patterns were not significantly different among the three clinical U-IIPs subgroups. Nevertheless, our findings suggested that the fibrosis scores and the percentage of fibroblastic foci could provide a prognostic assessment in U-IIPs.


Assuntos
Pneumonias Intersticiais Idiopáticas/diagnóstico por imagem , Pneumonias Intersticiais Idiopáticas/patologia , Pulmão/patologia , Idoso , Progressão da Doença , Feminino , Fibrose , Seguimentos , Humanos , Pneumonias Intersticiais Idiopáticas/classificação , Pneumonias Intersticiais Idiopáticas/fisiopatologia , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Fatores de Tempo , Tomografia Computadorizada por Raios X
9.
Respirology ; 23(5): 507-511, 2018 May.
Artigo em Inglês | MEDLINE | ID: mdl-29178216

RESUMO

BACKGROUND AND OBJECTIVE: Idiopathic pulmonary fibrosis (IPF) is increasingly diagnosed by clinical and computed tomography (CT) criteria; however, surgical lung biopsy (SLB) may still be required in patients who lack definite CT features of usual interstitial pneumonia (UIP). We reviewed a cohort of elderly patients who underwent SLB, to evaluate the benefit of SLB in diagnosing idiopathic interstitial pneumonia (IIP). METHODS: We searched the pathology records of Mayo Clinic for ambulatory patients at least 75 years old, who underwent SLB between 2000 and 2012 for indeterminate IIP. Histologic slides were reviewed and clinical data were extracted from the record. RESULTS: A total of 55 patients (35 male) were enrolled. Median (interquartile range) age was 77 (76-80) years. Forced vital capacity was 70 (61-76)% and diffusing capacity of the lungs for carbon monoxide was 48 (42-54)% of predicted. In total, 37 (67%) patients had IPF, including 61% of those with HRCT findings inconsistent with UIP. Thirty-day mortality was 10% and 90-day mortality was 15%. CONCLUSION: The high mortality rate of SLB complicates the risk-benefit analysis in elderly patients with IIP. The expected value of the SLB is probably highest when the HRCT features are inconsistent with UIP, due to the frequent (39%) retrieval of patterns other than UIP.


Assuntos
Fibrose Pulmonar Idiopática/diagnóstico , Fibrose Pulmonar Idiopática/patologia , Pulmão/patologia , Idoso , Idoso de 80 Anos ou mais , Biópsia/mortalidade , Monóxido de Carbono , Feminino , Humanos , Pneumonias Intersticiais Idiopáticas/diagnóstico por imagem , Pneumonias Intersticiais Idiopáticas/patologia , Pneumonias Intersticiais Idiopáticas/fisiopatologia , Fibrose Pulmonar Idiopática/fisiopatologia , Masculino , Capacidade de Difusão Pulmonar , Estudos Retrospectivos , Tomografia Computadorizada por Raios X , Capacidade Vital
10.
J Int Med Res ; 46(1): 448-456, 2018 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-28758849

RESUMO

Objective Unclassifiable interstitial lung disease (ILD) is a common problem in clinical practice. These patients pose a distinct challenge with regard to appropriate evaluation and management. We investigated the clinical features and prognosis of unclassifiable ILD and compared its clinical profile with that of idiopathic pulmonary fibrosis (IPF) and idiopathic nonspecific interstitial pneumonia (NSIP). Methods Patients with IPF (n = 40), NSIP (n = 14), and unclassifiable ILD (n = 27) were selected from an ongoing database. Baseline clinical features, pulmonary function, and the extent of fibrosis on high-resolution computed tomography (HRCT) were evaluated. Mortality was estimated based on the ILD-Gender, Age, Physiology (ILD-GAP) index and composite physiologic index (CPI). Results IPF was associated with the worst survival (hazard ratio [HR] = 4.361 compared with NSIP), followed by unclassifiable cases (HR = 1.251 compared with NSIP). Increasing mortality was significantly impacted by age (HR = 1.04 per 1-year increase), lower carbon monoxide diffusing capacity of the lung (HR = 0.97), HRCT interstitial score (HR = 1.119 per 1-point increase), ILD-GAP score (HR = 1.570 per 1-point increase), and CPI (HR = 1.039 per 1-point increase). Conclusions Patients with unclassifiable ILD had an intermediate prognosis between that of IPF and NSIP. Patients at high risk of mortality can be identified using baseline clinical, physiological, and radiological features.


Assuntos
Pneumonias Intersticiais Idiopáticas/fisiopatologia , Fibrose Pulmonar Idiopática/fisiopatologia , Doenças Pulmonares Intersticiais/fisiopatologia , Adulto , Idoso , Diagnóstico Diferencial , Feminino , Humanos , Pneumonias Intersticiais Idiopáticas/diagnóstico por imagem , Pneumonias Intersticiais Idiopáticas/mortalidade , Pneumonias Intersticiais Idiopáticas/patologia , Fibrose Pulmonar Idiopática/diagnóstico por imagem , Fibrose Pulmonar Idiopática/mortalidade , Fibrose Pulmonar Idiopática/patologia , Pulmão/patologia , Pulmão/fisiopatologia , Doenças Pulmonares Intersticiais/diagnóstico por imagem , Doenças Pulmonares Intersticiais/mortalidade , Doenças Pulmonares Intersticiais/patologia , Masculino , Pessoa de Meia-Idade , Prognóstico , Testes de Função Respiratória , Estudos Retrospectivos , Índice de Gravidade de Doença , Análise de Sobrevida , Tomografia Computadorizada por Raios X
11.
Sci Rep ; 7(1): 16912, 2017 12 05.
Artigo em Inglês | MEDLINE | ID: mdl-29208971

RESUMO

Nonspecific interstitial pneumonia (NSIP) is characterised by interstitial infiltration of lymphocytes and varying amounts of interstitial fibrosis. B cells have been suggested to contribute to the pathogenesis of NSIP. However, the relationship between B-lymphocyte and the clinical outcomes of NSIP was unclear. In this study, 50 patients with histopathologically confirmed NSIP from Peking Union Medical College Hospital between April 2003 to December 2012 were retrospectively analyzed. Using immunohistochemical analyses, CD20+ B cells were counted in the lymphoid follicles, perivascular, interstitial, and peribronchiolar regions of lung tissure. The CD20+ lymphocytes were mainly present in the lymphoid follicles. The number of follicular CD20+ lymphocytes was higher in the fibrosing than cellular NSIP pattern [255.08 (132.92-449.71) vs. 121.33 (63.54-282.88)/0.1 mm2, p = 0.017]. After 1 year of therapy, the follicular CD20+ lymphocytes were significantly higher in patients whose forced vital capacity (FVC) worsened as compared to those who improved (p = 0.014). Additionally, follicular CD20+ lymphocytes were negatively correlated with the post-treatment percentage change in FVC (rho = -0.397, p = 0.004). However, follicular CD20+ lymphocytes were not correlated with survival. These results suggested that pulmonary follicular CD20+ lymphocytes were correlated with the fibrosing pattern of NSIP and predicted less clinical improvement after treatment.


Assuntos
Antígenos CD20/metabolismo , Linfócitos B/patologia , Pneumonias Intersticiais Idiopáticas/patologia , Adulto , Idoso , Linfócitos B/metabolismo , Feminino , Humanos , Pneumonias Intersticiais Idiopáticas/diagnóstico por imagem , Pneumonias Intersticiais Idiopáticas/mortalidade , Pneumonias Intersticiais Idiopáticas/fisiopatologia , Receptores de Lipopolissacarídeos/metabolismo , Pulmão/patologia , Tecido Linfoide/patologia , Masculino , Pessoa de Meia-Idade , Tomografia Computadorizada por Raios X , Capacidade Vital
12.
Respir Med ; 132: 189-194, 2017 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-29229096

RESUMO

BACKGROUND: Anti-aminoacyl-tRNA synthetase (ARS) antibodies have been detected in patients with polymyositis/dermatomyositis (PM/DM) and are especially correlated with interstitial lung disease (ILD). The aim of this study was to clarify the clinical features of patients with anti-ARS antibody positive idiopathic interstitial pneumonias (IIPs). METHODS: Patients were classified into three groups: 1) IIP with anti-ARS antibodies (ARS(+)IIP), 2) IIP without anti-ARS antibodies (ARS(-)IIP), and 3) PM/DM-associated ILD with anti-ARS antibodies (ARS(+)PM/DM-ILD). Clinical characteristics were compared retrospectively between the ARS(+)IIP group and the ARS(-)IIP group or ARS(+)PM/DM-ILD group. RESULTS: Eighteen ARS(+)IIP, 284 ARS(-)IIP, and 20 ARS(+)PM/DM-ILD patients were enrolled. The ARS(+)IIP group was significantly older and the male sex was predominant, had a lower prevalence of signs of connective tissue disease, differences in HRCT findings and patterns, and higher KL-6 levels compared to the ARS(+)PM/DM-ILD group. The findings in the bronchoalveolar lavage fluid (BALF) showing lymphocytosis and a lower CD4/CD8 ratio were similar between the two groups. However, the ARS(+)IIP group had significantly lower percentage of sputum, higher prevalence of mechanic's hand, higher KL-6 levels, lower percentage of vital capacity in the pulmonary function test, and lower CD4/CD8 ratio in BALF, compared to the ARS(-)IIP group. CONCLUSIONS: The present study demonstrated that features of pulmonary involvement were similar to those in the ARS(+)PM/DM-ILD group; however, some differences including HRCT findings and higher KL-6 levels suggest that ARS(+)IIP has severe ILD compared with ARS(+)PM/DM-ILD. Further prospective studies with a larger number of patients will elucidate the exact role of anti-ARS antibodies in IIPs.


Assuntos
Aminoacil-tRNA Sintetases/imunologia , Autoanticorpos/imunologia , Pneumonias Intersticiais Idiopáticas/imunologia , Fatores Etários , Idoso , Líquido da Lavagem Broncoalveolar/citologia , Relação CD4-CD8 , Doenças do Tecido Conjuntivo/epidemiologia , Dermatomiosite/epidemiologia , Dermatomiosite/imunologia , Feminino , Humanos , Pneumonias Intersticiais Idiopáticas/diagnóstico por imagem , Pneumonias Intersticiais Idiopáticas/epidemiologia , Pneumonias Intersticiais Idiopáticas/fisiopatologia , Japão/epidemiologia , Masculino , Pessoa de Meia-Idade , Mucina-1 , Estudos Retrospectivos , Fatores Sexuais , Tomografia Computadorizada por Raios X , Capacidade Vital
13.
Int J Chron Obstruct Pulmon Dis ; 12: 3221-3229, 2017.
Artigo em Inglês | MEDLINE | ID: mdl-29138550

RESUMO

Smoking-related interstitial lung disease (ILD) consists of a heterogeneous group of disorders that are considered a distinct entity. The 2013 American Thoracic Society and European Respiratory Society recommendations classified respiratory bronchiolitis (RB)/RB-ILD and desquamative interstitial pneumonia (DIP) as smoking-related idiopathic interstitial pneumonias (IIPs). The overlapping histopathological and radiological patterns of smoking-related IIPs must be considered. Overlap patterns of smoking-related IIPs are not easily classified as a single disorder. The initial radiological manifestation and follow-up changes are heterogeneous, even when diagnosed pathologically as RB or DIP. Therefore, a clinical-radiological-pathological consensus is important in the diagnosis of smoking-related IIPs, and long-term evaluation is essential to monitor the morphological changes in these patients. In this article, we reviewed the clinical, radiological, and pathological findings, and also the changes in radiological manifestations of smoking-related IIPs over time.


Assuntos
Pneumonias Intersticiais Idiopáticas/diagnóstico , Pulmão , Fumar/efeitos adversos , Tomografia Computadorizada por Raios X , Adulto , Idoso , Biópsia , Feminino , Humanos , Pneumonias Intersticiais Idiopáticas/epidemiologia , Pneumonias Intersticiais Idiopáticas/patologia , Pneumonias Intersticiais Idiopáticas/fisiopatologia , Pulmão/diagnóstico por imagem , Pulmão/patologia , Pulmão/fisiopatologia , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Prognóstico , Reprodutibilidade dos Testes , Fatores de Risco , Fumar/epidemiologia , Fumar/patologia , Fumar/fisiopatologia , Fatores de Tempo
14.
Radiother Oncol ; 125(2): 310-316, 2017 11.
Artigo em Inglês | MEDLINE | ID: mdl-28919004

RESUMO

PURPOSE: To compare toxicity and survival after stereotactic body radiotherapy (SBRT) between lung cancer patients with or without idiopathic interstitial pneumonias (IIPs), and to investigate the potential value of SBRT for the patients. METHODS: Among lung cancer patients receiving SBRT between 2005 and 2016, we evaluated those treated with a total dose of 40-60Gy in five fractions with curative intent who either were staged as cT1-4N0M0 or experienced postoperative isolated local recurrence. We analyzed the incidence of radiation pneumonitis (RP) in all patients and local recurrence and overall survival (OS) in T1a-2a patients. RESULTS: A total of 508 patients were eligible, including 42 with IIPs. The median follow-up was 32.3 (6.0-120.9) months. Significantly more patients with IIPs had grade ≥3RP than did those without IIPs (12% vs. 3%, p=0.009). The 2-year local recurrence rate was low in both groups (3.4% vs. 5.6%, p=0.38). The 2-year OS rate was significantly lower in the patients with IIPs (42.2% vs. 80.9%, p<0.001), although death from lung cancer was comparable (p=0.74). CONCLUSION: SBRT achieved excellent local control with acceptable pulmonary toxicity in lung cancer patients with IIPs. SBRT can be a reasonable option for early lung cancer patients with IIPs.


Assuntos
Pneumonias Intersticiais Idiopáticas/fisiopatologia , Neoplasias Pulmonares/radioterapia , Pneumonite por Radiação/etiologia , Radiocirurgia/métodos , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Incidência , Masculino , Pessoa de Meia-Idade , Recidiva Local de Neoplasia/radioterapia , Radiocirurgia/efeitos adversos , Estudos Retrospectivos , Taxa de Sobrevida
15.
Pulm Pharmacol Ther ; 46: 78-87, 2017 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-28865842

RESUMO

Idiopathic pulmonary fibrosis (IPF) is characterized by infiltration of inflammatory cells, excessive collagen production and accumulation of myofibroblasts. We explored the possible role of subepithelial lung myofibroblasts (SELMs) in the development of fibrosis in IPF. SELMs, isolated from surgical specimens of healthy lung tissue, were cultured with pro-inflammatory factors or bronchoalveolar lavage fluid (BALF) from patients with IPF or idiopathic non-specific interstitial pneumonia (iNSIP) and their fibrotic activity was assessed. Stimulation of SELMs with pro-inflammatory factors induced a significant increase of Tissue Factor (TF) and Tumor necrosis factor-Like cytokine 1 A (TL1A) expression and collagen production in culture supernatants. Stimulation with BALF from IPF patients with mild to moderate, but not severe disease, and from iNSIP patients induced a significant increase of TF expression. BALF from all IPF patients induced a significant increase of TL1A expression and collagen production, while BALF from iNSIP patients induced a significant increase of TL1A, but not of collagen production. Interestingly, TGF-ß1 and BALF from all IPF, but not iNSIP patients, induced a significant increase in SELMs migration. In conclusion, BALF from IPF patients induces fibrotic activity in lung myofibroblasts, similar to mediators associated with lung fibrosis, indicating a key role of SELMs in IPF.


Assuntos
Líquido da Lavagem Broncoalveolar , Pneumonias Intersticiais Idiopáticas/fisiopatologia , Fibrose Pulmonar Idiopática/fisiopatologia , Miofibroblastos/metabolismo , Colágeno/metabolismo , Humanos , Índice de Gravidade de Doença , Tromboplastina/metabolismo , Fator de Crescimento Transformador beta1/metabolismo , Membro 15 da Superfamília de Ligantes de Fatores de Necrose Tumoral/metabolismo
16.
Respirology ; 22(7): 1363-1370, 2017 10.
Artigo em Inglês | MEDLINE | ID: mdl-28508494

RESUMO

BACKGROUND AND OBJECTIVE: Acute exacerbation (AE) of idiopathic pulmonary fibrosis (IPF) (AE-IPF) is a poor prognostic disorder. AE is also reported to occur in other idiopathic interstitial pneumonias (IIPs). There are limited data available regarding the effectiveness of treatment for AE-IIPs. The objective of this study was to clarify the prognostic impact of the initial dose of prednisolone (PSL) for treating AE-IIPs. METHODS: Eighty-five patients with AE-IIPs, diagnosed according to the criteria of the Japanese Respiratory Society, were enrolled in this study (IPF/non-IPF: 63/22 patients) from 2004 to 2013. We performed multivariate Cox proportional hazard regression analysis to identify poor prognostic factors. HRCT patterns at the onset of AE-IIPs were classified as diffuse or non-diffuse. We evaluated the prognostic significance of the initial dose of PSL by adjusting for other prognostic factors. RESULTS: Median survival time (MST) after AE-IIPs diagnosis was 49 days. MST of AE-IPF and AE-non-IPF was 39 and 49 days, respectively. A diffuse HRCT pattern, lower serum IgG and higher serum surfactant protein-D at AE diagnosis, long-term oxygen therapy (LTOT) before AE and positive pressure ventilation (PPV) use for AE were significant poor prognostic factors for all patients, as were LTOT before AE and lower serum IgG for no-PPV patients. High-dose PSL ≥ 0.6 mg/kg was a significant prognostic factor for no-PPV patients after adjusting for other prognostic factors. CONCLUSION: We concluded that a dose of PSL ≥ 0.6 mg/kg after i.v. high-dose methylprednisolone therapy should be recommended for the treatment of AE-IIPs.


Assuntos
Anti-Inflamatórios/uso terapêutico , Progressão da Doença , Pneumonias Intersticiais Idiopáticas/tratamento farmacológico , Metilprednisolona/uso terapêutico , Idoso , Feminino , Humanos , Pneumonias Intersticiais Idiopáticas/mortalidade , Pneumonias Intersticiais Idiopáticas/fisiopatologia , Masculino , Pessoa de Meia-Idade , Prognóstico , Taxa de Sobrevida , Resultado do Tratamento
17.
Chest ; 152(1): 103-112, 2017 07.
Artigo em Inglês | MEDLINE | ID: mdl-28300570

RESUMO

BACKGROUND: Some patients with autoimmune characteristics and idiopathic interstitial pneumonia, particularly usual interstitial pneumonia (UIP), do not fit neatly into the category of connective tissue disease-associated interstitial lung disease (CTD-ILD), idiopathic pulmonary fibrosis (IPF), or recently proposed yet to be validated criteria for interstitial pneumonia with autoimmune features (IPAF). Outcomes of these patients are unknown. METHODS: This was a retrospective single-center study. Analyses of variance compared differences in mean change in FVC and diffusion capacity (Dlco) over 1 year among 124 well-defined patients (20 patients with positive autoantibodies with or without symptoms of connective tissue disease [AI-ILD], 15 patients with IPAF, 36 patients with CTD-ILD, and 53 patients with IPF with negative CTD serologies [Lone-IPF]). RESULTS: Of the patients, 75% with AI-ILD, 33% with IPAF, and 33% with CTD-ILD had UIP. Initial FVC and Dlco were similarly moderately reduced across groups. Mean change in FVC over 12 months was as follows: -60 mL (IPAF), -110 mL (AI-ILD), -10 mL (CTD-ILD), and -90 mL (Lone-IPF) (P = .52). Mean change in Dlco was as follows: 2.39 mL/mm Hg/min (IPAF), -1.15 mL/mm Hg/min (AI-ILD), -0.27 mL/mm Hg/min (CTD-ILD), and -1.05 mL/mm Hg/min (Lone-IPF) (P < .001). By pattern of disease, the mean change in FVC was as follows: -140 mL (UIP), 10 mL (nonspecific interstitial pneumonia), and 12 mL (unclassifiable/other) (P = .001). CONCLUSIONS: No clinically significant differences in pulmonary function to distinguish between patients with AI-ILD, IPAF, CTD-ILD, and Lone-IPF were observed after 1 year. Longer periods of follow-up are needed to understand the outcomes of these patients. It is not yet clear whether AI-ILD is a distinct phenotype or a variant of the newly proposed entity IPAF.


Assuntos
Autoanticorpos/sangue , Doenças do Tecido Conjuntivo , Pneumonias Intersticiais Idiopáticas , Fibrose Pulmonar Idiopática , Doenças Pulmonares Intersticiais , Idoso , Autoimunidade/imunologia , Estudos de Coortes , Doenças do Tecido Conjuntivo/diagnóstico , Doenças do Tecido Conjuntivo/fisiopatologia , Diagnóstico Diferencial , Feminino , Seguimentos , Humanos , Pneumonias Intersticiais Idiopáticas/diagnóstico , Pneumonias Intersticiais Idiopáticas/epidemiologia , Pneumonias Intersticiais Idiopáticas/imunologia , Pneumonias Intersticiais Idiopáticas/fisiopatologia , Fibrose Pulmonar Idiopática/diagnóstico , Fibrose Pulmonar Idiopática/imunologia , Fibrose Pulmonar Idiopática/fisiopatologia , Pulmão/diagnóstico por imagem , Pulmão/fisiopatologia , Doenças Pulmonares Intersticiais/diagnóstico , Doenças Pulmonares Intersticiais/imunologia , Doenças Pulmonares Intersticiais/fisiopatologia , Masculino , Pessoa de Meia-Idade , Determinação de Necessidades de Cuidados de Saúde , Testes de Função Respiratória/métodos , Estatística como Assunto , Tomografia Computadorizada por Raios X/métodos , Washington/epidemiologia
19.
Acta Radiol ; 58(5): 550-557, 2017 May.
Artigo em Inglês | MEDLINE | ID: mdl-27565631

RESUMO

Background Although fibrotic idiopathic interstitial pneumonias (IIPs) alone and those combined with pulmonary emphysema are naturally progressive diseases, the process of deterioration and outcomes are variable. Purpose To evaluate and compare serial changes of computed tomography (CT) abnormalities and prognostic predictive factors in fibrotic IIPs alone and those combined with pulmonary emphysema. Material and Methods A total of 148 patients with fibrotic IIPs alone (82 patients) and those combined with pulmonary emphysema (66 patients) were enrolled. Semi-quantitative CT analysis was used to assess the extents of CT characteristics which were evaluated on initial and follow-up CT images. Univariate and multivariate analyses were performed to assess the effects of clinical and CT variables on survival. Results Significant differences were noted between fibrotic scores, as determined using initial CT scans, in the fibrotic IIPs alone (21.22 ± 9.83) and those combined with pulmonary emphysema groups (14.70 ± 7.28) ( P < 0.001). At follow-up CT scans, changes in the extent of ground glass opacities (GGO) were greater ( P = 0.031) and lung cancer was more prevalent ( P = 0.001) in the fibrotic IIPs combined with pulmonary emphysema group. Multivariate Cox proportional hazards analysis showed changes in the extent of GGO (hazard ratio, 1.056) and the presence of lung cancer (hazard ratio, 4.631) were predictive factors of poor survivals. Conclusion Although patients with fibrotic IIPs alone and those combined with pulmonary emphysema have similar mortalities, lung cancer was more prevalent in patients with fibrotic IIPs combined with pulmonary emphysema. Furthermore, changes in the extent of GGO and the presence of lung cancer were independent prognostic factors of poor survivals.


Assuntos
Pneumonias Intersticiais Idiopáticas/diagnóstico por imagem , Fibrose Pulmonar Idiopática/diagnóstico por imagem , Enfisema Pulmonar/diagnóstico por imagem , Tomografia Computadorizada por Raios X/métodos , Idoso , Feminino , Humanos , Pneumonias Intersticiais Idiopáticas/fisiopatologia , Fibrose Pulmonar Idiopática/complicações , Fibrose Pulmonar Idiopática/fisiopatologia , Pulmão/diagnóstico por imagem , Pulmão/fisiopatologia , Masculino , Prognóstico , Enfisema Pulmonar/complicações , Enfisema Pulmonar/fisiopatologia , Estudos Retrospectivos
20.
Intern Med ; 55(20): 2939-2944, 2016.
Artigo em Inglês | MEDLINE | ID: mdl-27746429

RESUMO

Objective The influence of smoking on the pathogenesis and clinical course of interstitial pneumonia has recently attracted attention. To clarify the influence of smoking on the clinical patient characteristics and therapeutic effects in patients with interstitial pneumonia presenting with a non-specific interstitial pneumonia (NSIP) pattern, we compared the clinical patient characteristics and therapeutic effects in smokers and nonsmokers in this study. Methods We divided 31 NSIP (16 idiopathic nonspecific interstitial pneumonia and 15 collagen vascular disease-associated nonspecific interstitial pneumonia) patients into smoker and non-smoker groups for each case. The patient characteristics, pulmonary function tests, Krebs von den Lungen 6 (KL-6), surfactant protein D (SP-D), bronchoalveolar lavage fluid findings, and clinical courses for two years were compared between the smoker and non-smoker groups. Results The smoking subgroup (n=15) of NSIP patients had a significantly lower % diffusing capacity for carbon monoxide/ alveolar ventilation (DLCO/VA) and tended to have higher SP-D values than the nonsmoking subgroup (n=16). Although no difference was observed regarding the prognosis, 5 of 6 cases with NSIP, which had worsening of lung disease were heavy smokers with a pack-year history of 40 or greater. Conclusion Smoking is thus suggested to negatively influence the diffusing capacity caused by damage to alveolar epithelial cells. In addition, smoking may also be potentially related to resistance to therapy in NSIP cases.


Assuntos
Pneumonias Intersticiais Idiopáticas/fisiopatologia , Abandono do Hábito de Fumar , Fumar/efeitos adversos , Adulto , Idoso , Gerenciamento Clínico , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Prognóstico , Testes de Função Respiratória , Prevenção do Hábito de Fumar , Avaliação de Sintomas/métodos
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