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1.
Zhonghua Wai Ke Za Zhi ; 57(12): 956-960, 2019 Dec 01.
Artigo em Chinês | MEDLINE | ID: mdl-31826603

RESUMO

Lung cancer is the leading cause of death in malignant tumors. Surgery is one of the main treatments for early stage lung cancer. Idiopathic pulmonary fibrosis (IPF), one of the most common interstitial lung disease, is a chronic, progressive lung disease, characterized by insidious onset and progressive deterioration. It is reported that there are common pathways in idiopathic pulmonary fibrosis and lung cancer, and patients with IPF have a higher risk of lung cancer than the general population, whose prevalence was 3.34 times than that of the general population. The incidence of acute exacerbations of IPF and postoperative mortality was significantly higher in lung cancer patients complicated with IPF than those without IPF. The long-term outcomes of patients with IPF are dramatically worse than those without IPF. It was concluded that lobectomy has been still the standard surgical procedure for such patients, but sublobectomy should be taken as potential alternative choice for patients with high risk after preoperative evaluation. Moreover, further studies should be conducted on the prevention and treatment of acute exacerbation of IPF after surgery.


Assuntos
Fibrose Pulmonar Idiopática/cirurgia , Neoplasias Pulmonares/cirurgia , Pneumonectomia/métodos , Humanos , Fibrose Pulmonar Idiopática/complicações , Fibrose Pulmonar Idiopática/mortalidade , Pulmão/cirurgia , Neoplasias Pulmonares/etiologia , Pneumonectomia/mortalidade
2.
Georgian Med News ; (294): 98-103, 2019 Sep.
Artigo em Russo | MEDLINE | ID: mdl-31687958

RESUMO

The article reveals the modern aspects of IPF pathogenesis in with an emphasis on the main proposed prognostic biomarkers. IPF remains the leader among diseases with unknown etiology, the diagnosis and management of which are not very successful, despite the obvious progress in molecular medicine. There is presented analysis of the significance of IPF potential biomarkers and their concentrations in the blood and bronchoalveolar lavage fluids (BAL): endothelin-1, CC-chemokine ligand 18, interleukin-1, surfactant protein SP-D in the review. The role of their changing levels in the blood and BAL for assessing the course of the IPF and its prognosis, as well as the prevailing importance of the polymorphism of the genes encoding them, is shown. Obviously, the progressive accumulation of fibroblast-myofibroblast cells in the lungs IPF patients worsens the prognosis of disease, forms its own environment with a set of cytokines, growth factors, collagen, fibronectin in the extracellular matrix of fibrous lungs. The insufficient amount of studies in the face of the rarity of the disease leaves a lot of controversial issues for solution in the future. Obviously, to assess the prognosis of IPF mortality, it is necessary to include a very large number of patients, to extend the observation period, which increases their cost and reduces the opportunities and desire of pharmaceutical companies to participate in these studies.


Assuntos
Biomarcadores/sangue , Líquido da Lavagem Broncoalveolar/química , Fibrose Pulmonar Idiopática/diagnóstico , Pulmão/metabolismo , Biomarcadores/análise , Quimiocinas CC , Endotelina-1 , Humanos , Fibrose Pulmonar Idiopática/sangue , Fibrose Pulmonar Idiopática/mortalidade , Interleucina-1 , Pulmão/fisiopatologia , Prognóstico , Proteína D Associada a Surfactante Pulmonar/análise , Proteína D Associada a Surfactante Pulmonar/sangue , Surfactantes Pulmonares/análise , Surfactantes Pulmonares/sangue
3.
BMC Pulm Med ; 19(1): 120, 2019 Jul 04.
Artigo em Inglês | MEDLINE | ID: mdl-31272428

RESUMO

BACKGROUND: Patients with interstitial lung disease (ILD) who show features related to autoimmunity without meeting criteria for a defined connective tissue disease are categorized as interstitial pneumonia with autoimmune features (IPAF). The present study compared clinical characteristics and clinical outcomes of patients with IPAF to patients with connective tissue disease related-interstitial lung disease (CTD-ILD) and patients with idiopathic pulmonary fibrosis (IPF). METHODS: ILD patients who were consecutively enrolled in a single institution ILD cohort between 2008 and 2015 were evaluated for the study. Clinical data had been prospectively collected, while radiologic imaging and pathologic findings were re-reviewed for the present study. RESULTS: Out of 305 patients with ILD, 54 (17.7%) patients met the classification of IPAF, 175 (57.4%) patients had IPF, and 76 (24.9%) patients were diagnosed with CTD-ILD. Compared to IPF, incidences of acute exacerbations in 1,3 and 5 years were significantly less in the IPAF group (p = 0.022, p = 0.026 and p = 0.007, respectively). From multivariate analysis for mortality, age (p = 0.034, HR 1.022, 95% CI: 1.002-1.044), FVC (p < 0.001, HR 0.970, 95% CI: 0.955-0.984), ILD exacerbation (p = 0.001, HR 2.074, 95% CI: 1.366-3.148), and ILD type (p = 0.047, HR 0.436, 95% CI: 0.192-0.984 (IPAF vs IPF), respectively) showed significant association. CONCLUSIONS: Compared to the other ILD groups, IPAF showed distinct clinical characteristics. The IPAF group showed better survival and less episodes of exacerbation when compared to the IPF group.


Assuntos
Autoimunidade , Doenças do Tecido Conjuntivo/complicações , Fibrose Pulmonar Idiopática/mortalidade , Doenças Pulmonares Intersticiais/mortalidade , Idoso , Idoso de 80 Anos ou mais , Doenças do Tecido Conjuntivo/imunologia , Bases de Dados Factuais , Feminino , Humanos , Incidência , Doenças Pulmonares Intersticiais/etiologia , Masculino , Pessoa de Meia-Idade , Análise Multivariada , Estudos Prospectivos , República da Coreia/epidemiologia , Análise de Sobrevida , Tomografia Computadorizada por Raios X
4.
Int J Rheum Dis ; 22(9): 1741-1745, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31328423

RESUMO

BACKGROUND: Acute exacerbation (AE) of idiopathic pulmonary fibrosis (IPF) is associated with high mortality, but there is limited clinical data on AE of interstitial lung disease (ILD) in connective tissue disease-associated ILD (CTD ILD). The present study was conducted to provide prevalence and clinical features of AE, as well as various risk factors associated with mortality among AE CTD ILD patients. METHODS: Between May 2013 and April 2018, 15 patients who developed AE among 105 consecutive patients with CTD with chronic ILD were included. AE was defined using the criteria recently proposed by the IPF net, with slight modification for adaptation to CVD-IP6 (collagen vascular disease-associated interstitial pneumonia), and patients having CTD with AE met all the criteria. RESULTS: Fifteen patients with mean age of 45.8 ± 13.9 years developed AE; the most common subgroup (n = 5, 33%) was systemic sclerosis. The mean duration (months) between diagnosis of ILD and AE was 56.5 ± 38.0 with mean follow-up duration of 24 ± 18.1 months. The baseline arterial oxygen pressure (PaO2 ) was 81.7 ± 8.1 mm Hg and mean forced vital capacity (%) was 57.9 ± 8.9. Five patients requiring mechanical ventilation died. Patients with shorter duration (months) of disease between onset of ILD to AE had higher mortality, 40.4 ± 45.1 vs 64.6 ± 33.6. Those who had significantly lower baseline PaO2 (mean ± SD), 72.6 ± 3.4 vs 86.2 ± 5.3 mm Hg (P = .002) had higher mortality. CONCLUSIONS: In our study, the majority of patients with AE CTD ILD had systemic sclerosis. Patients with lower baseline PaO2 and those requiring mechanical ventilation had higher mortality.


Assuntos
Doenças do Tecido Conjuntivo/epidemiologia , Fibrose Pulmonar Idiopática/epidemiologia , Doenças Pulmonares Intersticiais/epidemiologia , Adulto , Doenças do Tecido Conjuntivo/diagnóstico , Doenças do Tecido Conjuntivo/mortalidade , Doenças do Tecido Conjuntivo/terapia , Progressão da Doença , Feminino , Humanos , Fibrose Pulmonar Idiopática/diagnóstico , Fibrose Pulmonar Idiopática/mortalidade , Fibrose Pulmonar Idiopática/terapia , Imunossupressores/uso terapêutico , Índia/epidemiologia , Doenças Pulmonares Intersticiais/diagnóstico , Doenças Pulmonares Intersticiais/mortalidade , Doenças Pulmonares Intersticiais/terapia , Masculino , Pessoa de Meia-Idade , Prevalência , Prognóstico , Estudos Prospectivos , Respiração Artificial , Medição de Risco , Fatores de Risco , Fatores de Tempo
5.
BMC Pulm Med ; 19(1): 130, 2019 Jul 18.
Artigo em Inglês | MEDLINE | ID: mdl-31319833

RESUMO

BACKGROUND: The aim of this study was to analyze the relative frequency, clinical characteristics, disease onset and progression in f-IPF vs. sporadic IPF (s-IPF). METHODS: Familial IPF index patients and their family members were recruited into the European IPF registry/biobank (eurIPFreg) at the Universities of Giessen and Marburg (UGMLC). Initially, we employed wide range criteria of f-IPF (e.g. relatives who presumably died of some kind of parenchymal lung disease). After narrowing down the search to occurrence of idiopathic interstitial pneumonia (IIP) in at least one first grade relative, 28 index patients were finally identified, prospectively interviewed and examined. Their family members were phenotyped with establishment of pedigree charts. RESULTS: Within the 28 IPF families, overall 79 patients with f-IPF were identified. In the same observation period, 286 f-IIP and s-IIP patients were recruited into the eurIPFreg at our UGMLC sites, corresponding to a familial versus s-IPF of 9.8%. The both groups showed no difference in demographics (61 vs. 79% males), smoking history, and exposure to any environmental triggers known to cause lung fibrosis. The f-IPF group differed by an earlier age at the onset of the disease (55.4 vs. 63.2 years; p < 0.001). On average, the f-IPF patients presented a significantly milder extent of functional impairment at the time point of inclusion vs. the s-IPF group (FVC 75% pred. vs. FVC 62% pred., p = 0.011). In contrast, the decline in FVC was found to be faster in the f-IPF vs. the s-IPF group (4.94% decline in 6 months in f-IPF vs. 2.48% in s-IPF, p = 0.12). The average age of death in f-IPF group was 67 years vs. 71.8 years in s-IPF group (p = 0.059). The f-IIP group displayed diverse inheritance patterns, mostly autosomal-dominant with variable penetrance. In the f-IPF, the younger generations showed a tendency for earlier manifestation of IPF vs. the older generation (58 vs. 66 years, p = 0.013). CONCLUSIONS: The 28 f-IPF index patients presented an earlier onset and more aggressive natural course of the disease. The disease seems to affect consecutive generations at a younger age. TRIAL REGISTRATION: Nr. NCT02951416 http://www.www.clinicaltrials.gov.


Assuntos
Pneumonias Intersticiais Idiopáticas/diagnóstico , Fibrose Pulmonar Idiopática/diagnóstico , Sistema de Registros , Idoso , Estudos Transversais , Progressão da Doença , Europa (Continente)/epidemiologia , Feminino , Humanos , Pneumonias Intersticiais Idiopáticas/mortalidade , Fibrose Pulmonar Idiopática/mortalidade , Fibrose Pulmonar Idiopática/fisiopatologia , Pulmão/fisiopatologia , Masculino , Pessoa de Meia-Idade , Taxa de Sobrevida
6.
N Z Med J ; 132(1499): 36-42, 2019 07 26.
Artigo em Inglês | MEDLINE | ID: mdl-31352472

RESUMO

AIM: In light of new therapies and guidelines for the management of idiopathic pulmonary fibrosis (IPF), and in the absence of local epidemiological data, we sought to ascertain a current estimate of the prevalence of IPF in Canterbury and to audit local practices. METHODS: We performed a retrospective observational study of patients with IPF in Canterbury, New Zealand and the wider region. Patients were identified through a systematic search of hospital records and included if they were alive on 1 January 2017, had a histological or radiological diagnosis of usual interstitial pneumonia and clinical correlation consistent with a diagnosis of IPF. Clinical data was extracted from the clinical record. Follow up was complete until April 2018. RESULTS: Sixty-eight patients were included, median follow up 33 (14-49) months. Fifteen (22.1%) patients died during follow up, median survival 19 (6.5-54) months. Estimated prevalence of IPF was 6.53/100,000 persons. Six (8.8%) patients were discussed at the Interstitial lung disease multi-disciplinary meeting. Resting Sp02 and end-of-life discussions were documented in 44 (64.7%) and 19 (27.9%) patients respectively, while oxygen therapy was prescribed to 15 (22.7%). 20/36 (55.5%) patients eligible for pirfenidone were treated. Those treated were more likely to have undergone a six-minute walk test (5/20 vs 3/48, p<0.05) or have been hospitalised in the last 12 months (12/20 vs 3/48, p<0.05). 7/20 patients remained on treatment at the end of follow-up (eight discontinued, five deceased). CONCLUSION: In this study the estimated prevalence of IPF in the Canterbury region is 6.53/100,000 persons. Furthermore, we have identified limitations in local practice relevant for service development.


Assuntos
Fibrose Pulmonar Idiopática , Hospitalização/estatística & dados numéricos , Humanos , Fibrose Pulmonar Idiopática/diagnóstico , Fibrose Pulmonar Idiopática/epidemiologia , Fibrose Pulmonar Idiopática/mortalidade , Fibrose Pulmonar Idiopática/terapia , Nova Zelândia/epidemiologia , Prevalência , Estudos Retrospectivos
7.
Med Sci Monit ; 25: 4193-4201, 2019 Jun 05.
Artigo em Inglês | MEDLINE | ID: mdl-31166938

RESUMO

BACKGROUND The prognosis of idiopathic pulmonary fibrosis (IPF) is the worst among all interstitial lung diseases, and is related to the disease itself. Comorbidities or complications can worsen IPF. We assessed the effect of comorbidities on the survival of IPF patients. A retrospective review of patients with IPF was completed. MATERIAL AND METHODS Information on demographic features, clinical examination, and comorbidities at baseline were obtained. Then, median, 1-year, and 5-year survival was calculated. A total of 380 patients with IPF admitted to Beijing Chao-Yang Hospital from 1 April 2002 to 31 March 2015 were followed up until December 2016. RESULTS Of these 380 patients, 71.9% died during the study period. Median survival was 2.25 years and overall 5-year survival was 28.5%. Also, 86.3% of patients were males. A total of 248 cases underwent lung function tests, and 178 patients underwent bronchoalveolar lavage (BAL). Multivariate analyses showed that forced expiratory volume in 1 second/forced vital capacity (FVC), diffusing capacity of the lungs for carbon monoxide percent predicted, FVC% predicted, the number of macrophages, neutrophils, and lymphocytes in BAL fluid, pulmonary hypertension, hypoxemia, and hydropower disorder were independent prognostic indicators of IPF, GAP gender (G), age (A), and 2 pulmonary physiological parameters (P) model can help to predict prognosis of IPF. CONCLUSIONS Spirometry, GAP model, and BAL are helpful to forecast the prognosis of IPF. IPF patients also suffering from pulmonary arterial hypertension, hypoxemia, and hydropower disorder have a poor prognosis.


Assuntos
Fibrose Pulmonar Idiopática/mortalidade , Fibrose Pulmonar Idiopática/fisiopatologia , Idoso , Idoso de 80 Anos ou mais , Lavagem Broncoalveolar , Líquido da Lavagem Broncoalveolar , China , Comorbidade , Feminino , Volume Expiratório Forçado , Humanos , Hipertensão Pulmonar/patologia , Fibrose Pulmonar Idiopática/diagnóstico , Pulmão/patologia , Doenças Pulmonares Intersticiais/patologia , Masculino , Pessoa de Meia-Idade , Prognóstico , Testes de Função Respiratória , Estudos Retrospectivos , Análise de Sobrevida , Volume de Ventilação Pulmonar , Capacidade Vital
8.
Ther Adv Respir Dis ; 13: 1753466619847130, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31170875

RESUMO

BACKGROUND: Some patients with idiopathic pulmonary fibrosis (IPF) develop acute exacerbation (AE-IPF) leading to severe acute respiratory failure (ARF); despite conventional supportive therapy, the mortality rate remains extremely high. The aim of this study was to assess how a treatment algorithm incorporating high-flow nasal cannula (HFNC) oxygen therapy affects the short-term mortality of patients with AE-IPF who develop ARF. METHOD AND DESIGN: A retrospective cohort analysis was conducted. PATIENTS AND INTERVENTIONS: The study consisted of 17 patients with AE-IPF admitted to a respiratory intensive care unit (RICU) for ARF managed using a treatment algorithm incorporating HFNC. The outcome measure was mortality rate during their stay in the RICU. RESULTS: Implementation of the treatment algorithm led to a successful outcome in nine patients and to a negative one in eight patients (47.1%) who died within 39 days of being admitted to the RICU. The survival rate was 70.6% (±0.1 %) at 15 days, 52.9% (±0.1%) at 30 days, 35.3% (±0.1%) at 90 days, and 15.6% (±9.73 %) at 365 days. Overall, 4 out of 10 patients who did not respond to conventional oxygen therapy showed a satisfactory response to HFNC. CONCLUSIONS: Short-term mortality fell to below 50% when a treatment algorithm incorporating HFNC was implemented in a group of patients with AE-IPF admitted to a RICU for ARF. Patients not responding to conventional oxygen therapy seemed to benefit from HFNC. The reviews of this paper are available via the supplementary material section.


Assuntos
Algoritmos , Fibrose Pulmonar Idiopática/terapia , Oxigenoterapia/métodos , Insuficiência Respiratória/terapia , Doença Aguda , Idoso , Idoso de 80 Anos ou mais , Cânula , Estudos de Coortes , Feminino , Humanos , Fibrose Pulmonar Idiopática/complicações , Fibrose Pulmonar Idiopática/mortalidade , Unidades de Terapia Intensiva , Masculino , Pessoa de Meia-Idade , Insuficiência Respiratória/etiologia , Insuficiência Respiratória/mortalidade , Estudos Retrospectivos , Taxa de Sobrevida , Resultado do Tratamento
9.
Clin Respir J ; 13(8): 493-498, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31102566

RESUMO

INTRODUCTION: Idiopathic pulmonary fibrosis (IPF) is a fatal interstitial lung disease characterized by variable progression. The prealbumin (PA) is a parameter in a routine blood biochemistry examination. We sought to investigate the prognostic value in IPF patients. OBJECTIVES: To evaluate the prognosis value in patients with IPF. METHODS: Blood biochemistry examination, demographics, pulmonary function data from patients with IPF consulted in Beijing Chao-Yang Hospital and China-Japan Friendship Hospital between July 2012 and December 2016 were collected. Infection, liver and kidney dysfunction and lung transplantation are excluded from the cohort. RESULTS: The result of multivariate Cox analysis showed that PA was significant prognostic indicator of survival along with BMI, FVC, serum albumin protein and serum global protein. The patients with PA concentration <0.2 mg/L had shorter survival compared with those whose PA were normal. Although the survival had no significant difference between the patients with PA concentration < 0.2 mg/L and albumin < 35 g/L and those with PA concentration < 0.2 mg/L, the average survival time of patients with PA concentration < 0.2 mg/L and albumin < 35 g/L were shorter. CONCLUSIONS: Our study indicated that IPF patients with PA concentration < 0.2 mg/L have poorer outcome. Further studies are warranted to indentify PA as a predictor for IPF patients outcomes and explore the role of PA in the pathogenesis of IPF.


Assuntos
Fibrose Pulmonar Idiopática/sangue , Fibrose Pulmonar Idiopática/mortalidade , Pré-Albumina/análise , Idoso , China/epidemiologia , Progressão da Doença , Feminino , Humanos , Fibrose Pulmonar Idiopática/fisiopatologia , Japão/epidemiologia , Masculino , Pessoa de Meia-Idade , Prognóstico , Testes de Função Respiratória/métodos , Estudos Retrospectivos , Taxa de Sobrevida
10.
Medicine (Baltimore) ; 98(18): e15140, 2019 May.
Artigo em Inglês | MEDLINE | ID: mdl-31045757

RESUMO

RATIONALE: Idiopathic pulmonary fibrosis (IPF) is a special form of spontaneous, chronic, progressive interstitial fibrotic pneumonia of unknown cause, and treatments for IPF have shown a poor prognosis. This study reports a new treatment, traditional Chinese medicine (TCM) therapy, for tonifying Qi-deficiency of lung-kidney in a 64-year-old patient with IPF. PATIENT CONCERNS: A 64-year-old man, who complained of cough and asthma, was diagnosed as IPF with mild impairment in lung function by thoracic high-resolution computed tomography and pulmonary function test. He received an 18-month N-acetylcysteine monotherapy but had no improvement in lung function. DIAGNOSES: IPF with mild impairment in lung function was diagnosed. INTERVENTIONS: The Chinese herbal medicine composition was decocted in 300 ml water for oral administration with 150 ml decoction twice daily in June 2017. OUTCOMES: The pulmonary function test showed that diffusing capacity for carbon monoxide had increased to 81% of predicted back to normal after 2-month TCM monotherapy. And diffusing capacity for carbon monoxide had increased to 89% of predicted, and forced expiratory volume in 1 s/forced vital capacity ratio increased to 92% at 14-month follow-up. No adverse events occurred during the 14 months of therapy and observation. LESSONS: The treatment by TCM therapy of tonifying lung-kidney's Qi-deficiency for IPF can improve the pulmonary function and reverse disease progression; it may be considered as a complementary treatment for IPF with mild-to-moderate impairment. However, the insights provided in this case report require further exploration and verification.


Assuntos
Fibrose Pulmonar Idiopática/terapia , Rim/efeitos dos fármacos , Pulmão/efeitos dos fármacos , Medicina Tradicional Chinesa/métodos , Acetilcisteína/uso terapêutico , Monóxido de Carbono/metabolismo , Progressão da Doença , Expectorantes/uso terapêutico , Volume Expiratório Forçado/efeitos dos fármacos , Humanos , Fibrose Pulmonar Idiopática/diagnóstico por imagem , Fibrose Pulmonar Idiopática/mortalidade , Fibrose Pulmonar Idiopática/fisiopatologia , Rim/fisiopatologia , Pulmão/fisiopatologia , Masculino , Pessoa de Meia-Idade , Capacidade de Difusão Pulmonar/efeitos dos fármacos , Testes de Função Respiratória/métodos , Resultado do Tratamento , Capacidade Vital/efeitos dos fármacos , Deficiência da Energia Yin
11.
Medicina (Kaunas) ; 55(5)2019 May 13.
Artigo em Inglês | MEDLINE | ID: mdl-31086028

RESUMO

Background and objectives: Idiopathic pulmonary fibrosis (IPF) has a particularly poor prognosis, and most IPF-related deaths are due to acute exacerbation (AE) of this condition. Few reports about biomarkers to predict prognosis of AE-IPF have been published since the release of the new AE-IPF criteria in 2016. The present study investigated relationships between serological markers and in-hospital mortality after the onset of AE-IPF. Methods: Demographic, serological, and imaging data from patients hospitalized at the Maebashi Red Cross Hospital (Gunma, Japan) between 1 January 2013, and 31 December 2017, were retrospectively reviewed. Subjects fulfilling the diagnostic criteria for AE-IPF were divided into those who survived or died; statistical analysis of risk factors was performed using data from these two groups. Results: Diagnostic criteria for AE-IPF were fulfilled by 84 patients (59 males (70.2%)), with a median age of 78 years (range, 56-95 years). IPF was diagnosed before hospitalization in 50 (59.5%) patients and 38 (45.2%) died in hospital. Among the serological markers at hospitalization in the deceased group, C-reactive protein (CRP) was significantly higher than in the survivor group (p = 0.002), while total serum protein (p = 0.031), albumin (p = 0.047) and total cholesterol (p = 0.039) were significantly lower. Cox hazard analysis of factors predicting mortality, corrected for age, sex and BMI, revealed the following: CRP (hazard ratio (HR) 1.080 (95% confidence interval (CI) 1.022-1.141); p = 0.006), LDH (HR 1.003 (95% CI 1.000-1.006); p = 0.037), and total cholesterol (HR 0.985 (95% CI 0.972-0.997); p = 0.018). Conclusions: Our data suggest that CRP, LDH, and total cholesterol may be biomarkers predicting mortality in patients with AE-IPF. However, only prospective controlled studies can confirm or not our observation as a generalizable one.


Assuntos
Biomarcadores/análise , Fibrose Pulmonar Idiopática/sangue , Valor Preditivo dos Testes , Idoso , Idoso de 80 Anos ou mais , Biomarcadores/sangue , Feminino , Humanos , Fibrose Pulmonar Idiopática/mortalidade , Japão , Masculino , Pessoa de Meia-Idade , Modelos de Riscos Proporcionais , Estudos Prospectivos , Estudos Retrospectivos , Estatísticas não Paramétricas
12.
Respir Res ; 20(1): 105, 2019 May 30.
Artigo em Inglês | MEDLINE | ID: mdl-31142314

RESUMO

BACKGROUND: Idiopathic pulmonary fibrosis (IPF) is a progressive disease with a variable clinical course and high mortality. We used data from a large national US registry of patients with IPF to investigate relationships between patient characteristics, including markers of disease severity, and mortality. METHODS: The analysis cohort comprised patients enrolled in the IPF-PRO Registry from its inception on 5 June 2014 to 26 October 2017. The primary criterion for inclusion in this registry is that patients must be diagnosed or confirmed with IPF at the enrolling centre within 6 months. Associations between patient characteristics and markers of disease severity at enrolment and mortality outcomes were investigated using univariable, multivariable and adjustment models. RESULTS: Among 662 patients enrolled, 111 patients died or had a lung transplant over a follow-up period of 30 months. The probability of being free of both events at month 30 was 50.6% (95% CI: 40.0, 60.2). When patient characteristics and markers of disease severity were jointly examined in a multivariable analysis, oxygen use at rest (hazard ratio [HR] 2.44 [95% CI: 1.45, 4.10]), lower forced vital capacity (FVC) % predicted (HR 1.28 [95% CI: 1.10, 1.49] per 10% decrease) and diffusion capacity for carbon monoxide (DLco) % predicted (HR 1.25 [95% CI: 1.04, 1.51] per 10% decrease) were significantly associated with increased risk of death or lung transplant. The risk of death or lung transplant increased with increasing age in patients ≥62 years old (HR 1.18 [95% CI: 0.99, 1.40] per 5-year increase), and decreased with increasing age in patients <62 years old (HR 0.60 [95% CI: 0.39, 0.92] per 5-year increase). CONCLUSIONS: In an observational US registry of patients with IPF, oxygen use at rest, lower FVC % predicted, and lower DLco % predicted were associated with risk of death or lung transplant. An audio podcast of the lead author discussing these data can be downloaded from: http://www.usscicomms.com/respiratory/snyder/IPF-PROsurvival1/ . TRIAL REGISTRATION: ClinicalTrials.gov number: NCT01915511 .


Assuntos
Fibrose Pulmonar Idiopática/mortalidade , Fibrose Pulmonar Idiopática/cirurgia , Transplante de Pulmão/mortalidade , Sistema de Registros , Idoso , Idoso de 80 Anos ou mais , Estudos de Coortes , Feminino , Seguimentos , Humanos , Fibrose Pulmonar Idiopática/diagnóstico , Transplante de Pulmão/tendências , Masculino , Pessoa de Meia-Idade , Mortalidade/tendências , Valor Preditivo dos Testes
13.
J Immunol Res ; 2019: 1845128, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31098385

RESUMO

Rationale: Idiopathic pulmonary fibrosis (IPF) is a progressive fibrotic interstitial lung disease, with high mortality. Currently, the aetiology and the pathology of IPF are poorly understood, with both innate and adaptive responses previously being implicated in the disease pathogenesis. Heat shock proteins (Hsp) and antibodies to Hsp in patients with IPF have been suggested as therapeutic targets and prognostic biomarkers, respectively. We aimed to study the relationship between the expression of Hsp72 and anti-Hsp72 antibodies in the BAL fluid and serum Aw disease progression in patients with IPF. Methods: A novel indirect ELISA to measure anti-Hsp72 IgG was developed and together with commercially available ELISAs used to detect Hsp72 IgG, Hsp72 IgGAM, and Hsp72 antigen, in the serum and BALf of a cohort of IPF (n = 107) and other interstitial lung disease (ILD) patients (n = 66). Immunohistochemistry was used to detect Hsp72 in lung tissue. The cytokine expression from monocyte-derived macrophages was measured by ELISA. Results: Anti-Hsp72 IgG was detectable in the serum and BALf of IPF (n = 107) and other ILDs (n = 66). Total immunoglobulin concentrations in the BALf showed an excessive adaptive response in IPF compared to other ILDs and healthy controls (p = 0.026). Immunohistochemistry detection of C4d and Hsp72 showed that these antibodies may be targeting high expressing Hsp72 type II alveolar epithelial cells. However, detection of anti-Hsp72 antibodies in the BALf revealed that increasing concentrations were associated with improved patient survival (adjusted HR 0.62, 95% CI 0.45-0.85; p = 0.003). In vitro experiments demonstrate that anti-Hsp72 complexes stimulate macrophages to secrete CXCL8 and CCL18. Conclusion: Our results indicate that intrapulmonary anti-Hsp72 antibodies are associated with improved outcomes in IPF. These may represent natural autoantibodies, and anti-Hsp72 IgM and IgA may provide a beneficial role in disease pathogenesis, though the mechanism of action for this has yet to be determined.


Assuntos
Células Epiteliais Alveolares/metabolismo , Autoanticorpos/metabolismo , Proteínas de Choque Térmico HSP72/metabolismo , Fibrose Pulmonar Idiopática/imunologia , Pulmão/imunologia , Macrófagos/fisiologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Células Cultivadas , Quimiocinas CC/metabolismo , Progressão da Doença , Feminino , Proteínas de Choque Térmico HSP72/genética , Proteínas de Choque Térmico HSP72/imunologia , Humanos , Fibrose Pulmonar Idiopática/mortalidade , Interleucina-8/metabolismo , Masculino , Pessoa de Meia-Idade , Análise de Sobrevida
14.
Respir Res ; 20(1): 71, 2019 Apr 11.
Artigo em Inglês | MEDLINE | ID: mdl-30971229

RESUMO

BACKGROUND: Given the broad definition of an acute exacerbation of IPF, it is likely that acute exacerbations are heterogeneous in their aetiology, severity and clinical course. We used pooled data from the INPULSIS® trials of nintedanib versus placebo to investigate whether acute exacerbations reported as serious adverse events were associated with higher mortality than those reported as non-serious adverse events and to assess the effect of nintedanib on these types of events. METHODS: Adverse events considered by an investigator to be an acute exacerbation were adjudicated as a confirmed acute exacerbation, suspected acute exacerbation, or not an acute exacerbation. Time to first investigator-reported acute exacerbation or confirmed/suspected acute exacerbation reported as a serious adverse event or non-serious adverse event over the 52-week treatment period was assessed post-hoc. Deaths were assessed based on data collected over the 52-week treatment period. RESULTS: Of 63 patients who had ≥1 investigator-reported acute exacerbation, 48 (76.2%) had a first acute exacerbation reported as a serious adverse event. Thirty-six (3.4%) patients had ≥1 confirmed/suspected acute exacerbation, of whom 31 had a first event reported as a serious adverse event. Investigator-reported acute exacerbations reported as serious adverse events occurred in 23 patients in the nintedanib group and 26 in the placebo group. Confirmed/suspected acute exacerbations reported as serious adverse events occurred in 10 and 21 patients in these groups, respectively. Nintedanib significantly reduced the risk of a first acute exacerbation reported as a serious adverse event (HR 0.57 [95% CI: 0.32, 0.99]; p = 0.0476) and the risk of a first confirmed/suspected acute exacerbation reported as a serious adverse event (HR 0.30 [95% CI: 0.14, 0.64]; p = 0.0019) versus placebo. A higher proportion of patients with investigator-reported acute exacerbations reported as serious adverse events died than patients with acute exacerbations reported as non-serious adverse events (61.2% versus 7.1%). CONCLUSION: Different severities of acute exacerbation of IPF may exist. Acute exacerbations reported as serious adverse events in the INPULSIS® trials were associated with high mortality. Nintedanib significantly reduced the risk of acute exacerbations reported as serious adverse events. TRIAL REGISTRATION: ClinicalTrials.gov NCT01335464 and NCT01335477 .


Assuntos
Progressão da Doença , Inibidores Enzimáticos/uso terapêutico , Fibrose Pulmonar Idiopática/tratamento farmacológico , Indóis/uso terapêutico , Índice de Gravidade de Doença , Doença Aguda , Idoso , Inibidores Enzimáticos/farmacologia , Feminino , Humanos , Fibrose Pulmonar Idiopática/diagnóstico , Fibrose Pulmonar Idiopática/mortalidade , Indóis/farmacologia , Masculino , Pessoa de Meia-Idade , Capacidade Vital/efeitos dos fármacos , Capacidade Vital/fisiologia
15.
BMC Pulm Med ; 19(1): 75, 2019 Apr 11.
Artigo em Inglês | MEDLINE | ID: mdl-30971235

RESUMO

BACKGROUND: Idiopathic pulmonary fibrosis (IPF) is a fatal lung disease, with a median survival of 2-3 years and variable natural history, characterized by gradual and progressive deterioration. Acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) is a severe complication, associated with poor survival and a mortality > 50%. To date, no treatment has proven effective in AE-IPF, with cyclophosphamide (CYC) the only therapy suggested to be effective on survival, primarily based on retrospective series. Considering the high fatality rates of AE-IPF, evaluating the efficacy of immunosuppressive agents in a randomized controlled trial proves crucial, as the results could significantly impact treatment and prognosis of AE-IPF. METHODS: The EXAFIP study is a French national multicenter double-blind placebo-controlled randomized trial. Its primary objective is to evaluate the efficacy of CYC compared to placebo on early survival in patients treated with corticosteroids. We hypothesize that adding CYC to high-dose corticosteroids would reduce 3-month mortality in AE-IPF patients. The primary outcome is all-cause mortality rate at Month 3; secondary objectives are to evaluate the efficacy of CYC compared to placebo on overall survival at Months 6 and 12, respiratory disease-specific mortality, respiratory morbidity, and chest high-resolution computed tomography features, and to determine prognostic factors in AE-IPF and compare the safety of the two treatment arms during 6 months' follow-up. DISCUSSION: There is an urgent unmet clinical need for effective AE-IPF treatment. The EXAFIP study is the first large Phase III placebo-controlled randomized trial evaluating the efficacy and safety of CYC added to corticosteroids in treating AE-IPF. The results of this study could significantly impact treatment strategy and prognosis of AE-IPF. TRIAL REGISTRATION: Clinical trials, NCT02460588 ; Date: June 2, 2015, prospectively; Issue date: 14/11/2017; Protocole Amendment Number: 03.


Assuntos
Corticosteroides/uso terapêutico , Ciclofosfamida/uso terapêutico , Fibrose Pulmonar Idiopática/tratamento farmacológico , Fibrose Pulmonar Idiopática/mortalidade , Causas de Morte , Ensaios Clínicos Fase III como Assunto , Progressão da Doença , Método Duplo-Cego , Quimioterapia Combinada , França , Humanos , Estudos Multicêntricos como Assunto , Prognóstico , Ensaios Clínicos Controlados Aleatórios como Assunto , Fatores de Tempo , Resultado do Tratamento
16.
BMC Pulm Med ; 19(1): 81, 2019 Apr 18.
Artigo em Inglês | MEDLINE | ID: mdl-30999878

RESUMO

BACKGROUND: In many clinical disorders, there is a relationship between the ratio of the diameter of the main pulmonary artery (mPA) to that of the aorta (Ao) on chest computed tomography (CT). The aim of this study was to determine if the mPA/Ao ratio at diagnosis is associated with the clinical characteristics and outcomes in patients with idiopathic pulmonary fibrosis (IPF). METHODS: We retrospectively reviewed the diameters of the pulmonary artery and aorta on chest CT, clinical characteristics, and results of other examinations in 303 patients at the time of initial diagnosis of IPF at our tertiary care center between 2011 and 2015. The primary outcomes were death and lung transplantation. The patients were followed up until June 2017. RESULTS: One hundred and eight patients (35.6%) died and 58 (19.1%) underwent lung transplantation during follow-up. The mean mPA and Ao diameters were 28.3 mm and 34.0 mm, respectively, and the mean mPA/Ao ratio was 0.84. Thirty-one patients (10.2%) had an mPA/Ao ratio > 1.0 and 182 (60.1%) had an mPA/Ao ratio > 0.8. Patients with an mPA/Ao ratio > 0.8 had a lower DLco value than those with an mPA/Ao ratio ≤ 0.8. In Kaplan-Meier analysis, patients with an mPA/Ao ratio > 1.0 or > 0.8 had worse outcomes than those with an mPA/Ao ratio ≤ 1.0 and ≤ 0.8, respectively. CONCLUSIONS: A higher mPA/Ao ratio based on 1.0 and 0.8 is associated with unfavorable prognosis in patients with IPF.


Assuntos
Aorta/patologia , Fibrose Pulmonar Idiopática/diagnóstico por imagem , Artéria Pulmonar/patologia , Idoso , Aorta/diagnóstico por imagem , Ecocardiografia , Feminino , Humanos , Fibrose Pulmonar Idiopática/mortalidade , Fibrose Pulmonar Idiopática/cirurgia , Estimativa de Kaplan-Meier , Transplante de Pulmão , Masculino , Pessoa de Meia-Idade , Prognóstico , Artéria Pulmonar/diagnóstico por imagem , Testes de Função Respiratória , Estudos Retrospectivos , Tomografia Computadorizada por Raios X
18.
Med Sci Monit ; 25: 2329-2336, 2019 Mar 30.
Artigo em Inglês | MEDLINE | ID: mdl-30927543

RESUMO

BACKGROUND The aim of this study was to assess the association between pulmonary vessel-related structures and cryptogenic fibrosing alveolitis (CFA) in a drug trial in a Chinese population using derived computed tomography (dCT) to evaluate functional reduction and survival. MATERIAL AND METHODS Discovery and validation cohorts were chosen separately by fulfilment of drug trial entry criteria, and we enrolled 269 and 292 consecutive patients, respectively. CFA patients who had undergone imaging based on volumetric non-contrast CT at our hospital were subjected to pulmonary vessel-related structure (PVS) measures and dCT to forecast mortality and reduction in reduced forced vital capacity of CFA. RESULTS The best forecaster of survival and reduction in terms of reduced forced vital capacity were found to be the dCT-generated outcomes in terms of PVS scores. Patients having less extensive disease highlighted the dCT outcomes through outperformance of CFA measures. When we used the cohort enhancement device, we found reduction in the requisite sample size of a CFA drug trial by 31% with the use of more than 5.0% dCT PVS score. CONCLUSIONS We found an association between CFA and PVS using dCT and it is far better than the results achieved so far by use of criterion standard measures. Additionally, reduction in the restrictive trial costs was also achieved by using cohort enhancement in a CFA drug trial setting, as PVS scores forced us to decrease the size of required CFA drug trial population by 30%. Interestingly, patients who had to take antifibrotic medication for longer periods had longer survival and less decreases forced vital capacity, as identified by PVS scores.


Assuntos
Fibrose Pulmonar Idiopática/fisiopatologia , Tomografia Computadorizada Multidetectores/métodos , Capacidade Vital/fisiologia , Idoso , Idoso de 80 Anos ou mais , China , Feminino , Humanos , Fibrose Pulmonar Idiopática/metabolismo , Fibrose Pulmonar Idiopática/mortalidade , Masculino , Pessoa de Meia-Idade , Testes de Função Respiratória/métodos , Tomografia Computadorizada por Raios X/métodos
19.
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
20.
PLoS One ; 14(3): e0214278, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-30897161

RESUMO

BACKGROUND: Honeycombing on high-resolution computed tomography (HRCT) images is a key finding in idiopathic pulmonary fibrosis (IPF). In IPF, honeycombing area determined by quantitative CT analysis is correlated with pulmonary function test findings. We hypothesized that quantitative CT-derived honeycombing area (HA) might predict mortality in patients with IPF. MATERIALS AND METHODS: Chest HRCT images of 52 IPF patients with definite usual interstitial pneumonia (UIP) pattern were retrospectively evaluated. Mortality data up to July 31, 2016, were recorded. Using a computer-aided system, HA and percentage of HA (%HA) were measured quantitatively. Predictors of 3-year mortality were evaluated using logistic regression models. RESULTS: The median %HA, %predicted forced vital capacity (FVC) and composite physiologic index (CPI) were 3.8%, 83.6%, and 33.6, respectively. According to GAP (gender, age, and physiology) stage, 20, 14, and 5 patients were classified under stages I-II-III, respectively. Percentage of HA was significantly correlated with %FVC, CPI, and GAP stage (all, p < 0.001). In univariate analysis, %HA, %FVC, and CPI were statistically significant predictors of mortality. In multivariate analysis using the stepwise regression method, only %HA (odds ratio [OR], 1.27; p = 0.011) was a significant independent predictors of mortality. Patients with %HA ≥ 4.8% had significantly lower survival rates than those with lesser %HA (median survival time, 1.3 vs 5.0 years; log-rank test; p < 0.001). CONCLUSION: Quantitative CT-derived HA might be an important and independent predictor of mortality in IPF patients with definite UIP pattern.


Assuntos
Fibrose Pulmonar Idiopática/diagnóstico por imagem , Fibrose Pulmonar Idiopática/mortalidade , Tomografia Computadorizada por Raios X/métodos , Idoso , Idoso de 80 Anos ou mais , Estudos de Avaliação como Assunto , Feminino , Humanos , Masculino , Prognóstico , Curva ROC , Estudos Retrospectivos , Análise de Sobrevida , Taxa de Sobrevida
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