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1.
Respir Res ; 20(1): 287, 2019 Dec 18.
Artigo em Inglês | MEDLINE | ID: mdl-31852459

RESUMO

BACKGROUND: Acute exacerbation of chronic fibrosing idiopathic interstitial pneumonias (AE-IIPs) is associated with a high mortality rate. In 2016, an international working group proposed a revised diagnostic criteria for AE-IIPs, suggesting that it be classified as idiopathic or triggered. Many factors are known to trigger AE-IIPs, including surgery, infection, and drugs. However, it is unknown which AE-IIPs triggers have a worse prognosis. We aimed to investigate the prognosis of patients with various clinical types of AE-IIPs, particularly infection-triggered, non-infection triggered, and idiopathic AE-IIPs. METHODS: We retrospectively collected data from 128 chronic fibrosing IIPs (CF-IIPs) patients who were hospitalized by respiratory failure between April 2009 and March 2019 at Juntendo University Hospital. Among these patients, we evaluated 79 patients who developed AE-IIPs and 21 who developed pneumonia superimposed on CF-IIPs. Patients with AE-IIPs were classified into three types: idiopathic, infection-triggered, and non-infection-triggered AE-IIPs. We analyzed differences in patient characteristics, examination findings; level of serum markers, results of pulmonary function, and radiological findings, prior treatment for baseline CF-IIPs, and prognosis. We then evaluated the risk factor for early death (death within 30 days from the onset of AE-IIPs) associated with AE-IIPs. RESULTS: Among the patients who developed AE-IIPs, 34 were characterized as having idiopathic, 25 were characterized as having infection-triggered, and 20 were categorized as having non-infection-triggered AE-IIPs. Survival time for pneumonia superimposed on IIPs was significantly longer than that for any AE-IIPs. Survival time for bacterial pneumonia superimposed on CF-IIPs was significantly longer than that for AE-IIPs (for each idiopathic and all triggered IIPs). Thereafter, survival time for infection-triggered was significantly longer than for idiopathic or non-infection-triggered AE-IIPs. The mortality rate was significantly lower in infection-triggered AE-IIPs than in other types of AE-IIPs. Furthermore, the incidence of infection-triggered AE-IIPs in winter was significantly higher than that in other seasons. Moreover, the clinical AE-IIPs types and radiological findings at AE-IIP onset were significant risk factors for AE-IIPs-induced early death. CONCLUSIONS: Our findings suggest that patients with infection-triggered AE-IIPs can expect a better prognosis than can patients with other clinical types of AE-IIPs.


Assuntos
Efeitos Colaterais e Reações Adversas Relacionados a Medicamentos/epidemiologia , Doença Iatrogênica/epidemiologia , Pneumonias Intersticiais Idiopáticas/epidemiologia , Pulmão , Pneumonia Bacteriana/epidemiologia , Idoso , Idoso de 80 Anos ou mais , Progressão da Doença , Efeitos Colaterais e Reações Adversas Relacionados a Medicamentos/diagnóstico , Efeitos Colaterais e Reações Adversas Relacionados a Medicamentos/mortalidade , Efeitos Colaterais e Reações Adversas Relacionados a Medicamentos/terapia , Feminino , Interações Hospedeiro-Patógeno , Humanos , Pneumonias Intersticiais Idiopáticas/diagnóstico , Pneumonias Intersticiais Idiopáticas/mortalidade , Pneumonias Intersticiais Idiopáticas/terapia , Incidência , Japão/epidemiologia , Pulmão/efeitos dos fármacos , Pulmão/microbiologia , Pulmão/cirurgia , Masculino , Pessoa de Meia-Idade , Pneumonia Bacteriana/diagnóstico , Pneumonia Bacteriana/mortalidade , Pneumonia Bacteriana/terapia , Prognóstico , Estudos Retrospectivos , Medição de Risco , Fatores de Risco , Estações do Ano , Fatores de Tempo
2.
PLoS One ; 14(9): e0221322, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31487292

RESUMO

BACKGROUND: In Korea, several household humidifier disinfectants (HDs) were clinically confirmed to cause HD-associated lung injury (HDLI). Polyhexamethylene guanidine (PHMG) phosphate is the main ingredient of the HDs found to be associated with lung disease. However, the association of HDs with other interstitial lung disease including idiopathic interstitial pneumonia (IIP) is not clear. We examined the relationship between HD exposure and IIP in a family-based study. METHODS: This case-control study included 244 IIP cases and 244 family controls who lived with the IIP patients. The IIP cases were divided into two groups, HDLI and other IIP, and were matched to family controls based on age and gender. Information on exposure to HDs was obtained from a structured questionnaire and field investigations. Conditional logistic regression was used to estimate odds ratio (ORs) and their corresponding 95% confidence interval (CI), investigating the association of HD-related exposure characteristics with IIP risk. RESULTS: The risks of IIP increased two-fold or more in the highest compared with the lowest quartile of several HD use characteristics, including average total use hours per day, cumulative sleep hours, use of HD during sleep, and cumulative exposure level. In analyses separated by HDLI and other IIP, the risks of HDLI were associated with airborne HD concentrations (adjusted OR = 3.01, 95% CI = 1.34-6.76; Q4 versus Q1) and cumulative exposure level (adjusted OR = 3.57, 95% CI = 1.59-8.01; Q4 versus Q1), but this relationship was not significant in the patients with other IIP. In comparison between HDLI and other IIP, the odds ratios of average total use hours, cumulative use hours, and cumulative sleeps hours was higher for other IIP. CONCLUSION: The use of household HDs is associated not only with HDLI but also with other IIP.


Assuntos
Desinfetantes/efeitos adversos , Umidificadores/estatística & dados numéricos , Pneumonias Intersticiais Idiopáticas/etiologia , Exposição por Inalação/efeitos adversos , Adulto , Estudos de Casos e Controles , Características da Família , Feminino , Humanos , Pneumonias Intersticiais Idiopáticas/epidemiologia , Pneumonias Intersticiais Idiopáticas/patologia , Incidência , Masculino , Pessoa de Meia-Idade , República da Coreia/epidemiologia , Fatores de Risco , Inquéritos e Questionários , Adulto Jovem
3.
Jpn J Clin Oncol ; 49(12): 1126-1133, 2019 Dec 27.
Artigo em Inglês | MEDLINE | ID: mdl-31411689

RESUMO

OBJECTIVE: Identifying risk factors for cancer treatment-related acute exacerbations (AEs) of idiopathic interstitial pneumonia (IIP) in patients with lung cancer. METHODS: We retrospectively reviewed clinical records of 98 patients with concurrent lung cancer and IIPs diagnosed and treated at the Sapporo Medical University Hospital from January 2010 to December 2014. RESULTS: Of the 98 patients with concurrent lung cancer and IIPs, 14 patients (14.3%) had AEs. A total of 10 patients died. The univariate analysis revealed that the patients with idiopathic pulmonary fibrosis (IPF) or usual interstitial pneumonia (UIP) patterns on chest computed tomography (CT) had significantly higher rates of AE than those with non-IPF or non-UIP patterns, respectively. Further, those with a reduced percentage of forced vital capacity (%FVC) predictive values or elevated Krebs von den Lungen-6 (KL-6) presented significantly higher rates of AE. Our multivariate analysis identified that UIP pattern on chest CT and each 10% decrease in %FVC were significant independent risk factors for AEs. Of the 14 patients who experienced AEs, 10 cases were associated with cancer treatment. The treatment-specific incidences were 3/40 (7.5%) for surgery, 5/50 (10.0%) for chemotherapy, and 2/26 (7.7%) for radiation therapy. After comparing the AE incidences in 55 cases receiving one treatment (monotherapy group) and in 29 cases receiving two types of treatment or more (multitherapy group), we found no significant differences. CONCLUSIONS: Chest CT UIP patterns and reduced %FVC are independent risk factors for AE. Moreover, AE incidence did not increase in the multitherapy group compared with the monotherapy group.


Assuntos
Pneumonias Intersticiais Idiopáticas/epidemiologia , Pneumonias Intersticiais Idiopáticas/patologia , Neoplasias Pulmonares/epidemiologia , Neoplasias Pulmonares/terapia , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Pneumonias Intersticiais Idiopáticas/complicações , Pneumonias Intersticiais Idiopáticas/terapia , Incidência , Pulmão/patologia , Pulmão/fisiopatologia , Neoplasias Pulmonares/complicações , Neoplasias Pulmonares/patologia , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Fatores de Risco , Exacerbação dos Sintomas
4.
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
6.
PLoS One ; 13(6): e0199659, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-29928060

RESUMO

OBJECTIVE: Although a possible association among myeloperoxidase-anti-neutrophil cytoplasmic antibody (MPO-ANCA), microscopic polyangiitis (MPA), and idiopathic pulmonary fibrosis (IPF) has been suggested, the clinical significance of MPO-ANCA in idiopathic interstitial pneumonias (IIPs), including IPF and non-IPF, remains unclear. We aimed to investigate the frequency of MPO-ANCA positivity, as well as MPA incidence and risk factors for development in patients initially diagnosed with IIP. METHODS: We retrospectively analysed 305 consecutive patients who were initially diagnosed as IIP and had MPO-ANCA results available. RESULTS: Of the 305 patients, 26 (8.5%) were MPO-ANCA-positive. Baseline characteristics were similar between the MPO-ANCA-positive and -negative patients. The cumulative 5-year MPA incidence was 24.3% in the MPO-ANCA-positive patients and 0% in the -negative patients (P < 0.0001). MPO-ANCA was positive in 15 of 133 (11.3%) patients initially diagnosed with IPF and in 11 of 172 (6.3%) patients initially diagnosed with non-IPF (P = 0.56), with cumulative 5-year MPA incidence of 6.2% and 1.0%, respectively (P = 0.10). Multivariate analysis revealed that UIP pattern on HRCT (HR = 3.20, P < 0.01) and no treatment for IIP (HR = 3.52, P < 0.01) were independently associated with MPA development in MPO-ANCA-positive patients. CONCLUSION: MPO-ANCA positivity was uncommon, but was associated with subsequent MPA development in patients initially diagnosed with IIP, including both IPF and non-IPF cases. The study suggested that attention should be paid to MPA development in MPO-ANCA-positive IIP patients with UIP pattern on HRCT and those without treatment for IIP.


Assuntos
Anticorpos Anticitoplasma de Neutrófilos/sangue , Pneumonias Intersticiais Idiopáticas/sangue , Poliangiite Microscópica/sangue , Peroxidase/sangue , Idoso , Progressão da Doença , Feminino , Seguimentos , Humanos , Pneumonias Intersticiais Idiopáticas/complicações , Pneumonias Intersticiais Idiopáticas/epidemiologia , Incidência , Masculino , Poliangiite Microscópica/complicações , Poliangiite Microscópica/epidemiologia , Pessoa de Meia-Idade , Estudos Retrospectivos , Fatores de Risco
7.
Respir Res ; 18(1): 204, 2017 12 06.
Artigo em Inglês | MEDLINE | ID: mdl-29212510

RESUMO

BACKGROUND: The effects of corticosteroid-based therapy in patients with idiopathic nonspecific interstitial pneumonia (iNSIP), and factors affecting treatment outcome, are not fully understood. We aimed to investigate the long-term treatment response and factors affecting the treatment outcome in iNSIP patients from a multi-center study in Korea. METHODS: The Korean interstitial lung disease (ILD) Study Group surveyed ILD patients from 2003 to 2007. Patients were divided into two groups to compare the treatment response: response group (forced vital capacity (FVC) improves ≥10% after 1 year) and non-response group (FVC <10%). Factors affecting treatment response were evaluated by multivariate logistic regression analysis. RESULTS: A total of 261 patients with iNSIP were enrolled, and 95 patients were followed-up for more than 1 year. Corticosteroid treatment was performed in 86 patients. The treatment group showed a significant improvement in lung function after 1-year: FVC, 10.0%; forced expiratory volume (FEV1), 9.8%; diffusing capacity of the lung for carbon monoxide (DLco), 8.4% (p < 0.001). Sero-negative anti-nuclear antibody (ANA) was significantly related with lung function improvement. Sero-positivity ANA was significantly lower in the response group (p = 0.013), compared to that in the non-response group. A shorter duration of respiratory symptoms at diagnosis was significantly associated with a good response to treatment (p = 0.018). CONCLUSION: Treatment with corticosteroids and/or immunosuppressants improved lung function in iNSIP patients, which was more pronounced in sero-negative ANA and shorter symptom duration patients. These findings suggest that early treatment should be considered in iNSIP patients, even in an early disease stage.


Assuntos
Pneumonias Intersticiais Idiopáticas/tratamento farmacológico , Pneumonias Intersticiais Idiopáticas/epidemiologia , Corticosteroides/administração & dosagem , Idoso , Estudos de Coortes , Feminino , Seguimentos , Humanos , Pneumonias Intersticiais Idiopáticas/diagnóstico , Imunossupressores/administração & dosagem , Masculino , Pessoa de Meia-Idade , República da Coreia/epidemiologia , Resultado do Tratamento
8.
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
9.
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
10.
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
11.
Rev Pneumol Clin ; 73(1): 13-26, 2017 Feb.
Artigo em Francês | MEDLINE | ID: mdl-27789161

RESUMO

Idiopathic interstitial pneumonias comprise 8 clinicopathological entities, most of them with a chronic course and various prognosis. Idiopathic pulmonary fibrosis is the most frequent and most severe of these. Computed tomography has an important role for its diagnosis. It can identify the corresponding pathological pattern of usual interstitial pneumonia in about 50 percent of cases. It can suggest differential diagnosis in other cases, most frequently fibrosing nonspecific interstitial pneumonia and chronic hypersensitivity pneumonitis. Imaging features should be integrated to clinical and available pathologic data during multidisciplinary team meetings involving physicians with a good knowledge of interstitial diseases. Some cases may be unclassifiable, but these could later be reclassified as new data may occur or imaging features may change. Surgical lung biopsy is being less frequently performed and an emerging less invasive technique, lung cryobiopsy, is under evaluation. Pleuroparenchymal fibroelastosis is a distinct entity only recently described, with uncertain prevalence and prognosis that seems being quite often associated to another pattern of interstitial pneumonia.


Assuntos
Pneumonias Intersticiais Idiopáticas/diagnóstico , Alveolite Alérgica Extrínseca/classificação , Alveolite Alérgica Extrínseca/diagnóstico , Alveolite Alérgica Extrínseca/epidemiologia , Alveolite Alérgica Extrínseca/patologia , Biópsia , Diagnóstico Diferencial , Humanos , Pneumonias Intersticiais Idiopáticas/classificação , Pneumonias Intersticiais Idiopáticas/epidemiologia , Pneumonias Intersticiais Idiopáticas/patologia , Fibrose Pulmonar Idiopática/diagnóstico , Fibrose Pulmonar Idiopática/epidemiologia , Fibrose Pulmonar Idiopática/patologia , Doenças Pulmonares Intersticiais/diagnóstico , Doenças Pulmonares Intersticiais/epidemiologia , Doenças Pulmonares Intersticiais/patologia , Valor Preditivo dos Testes , Prognóstico
12.
Respirology ; 21(8): 1431-1437, 2016 11.
Artigo em Inglês | MEDLINE | ID: mdl-27460223

RESUMO

BACKGROUND AND OBJECTIVE: Acute exacerbations (AEs) of idiopathic pulmonary fibrosis (IPF) and other idiopathic interstitial pneumonia (IIP) have a poor prognosis. This study aims to clarify the incidence and prognosis of AE in IPF and the other IIP. METHODS: A total of 229 patients were enrolled, of whom 92 had IPF and 137 had 'IIP other than IPF' based on the American Thoracic Society/European Respiratory Society/Japanese Respiratory Society/Latin American Thoracic Association (ATS/ERS/JRS/ALAT) 2011 IPF Guidelines. IIP other than IPF included 11 patients with a surgical lung biopsy (SLB) and the remainder without such a biopsy. IIP other than IPF was further classified into IIP with a 'possible usual interstitial pneumonia (UIP)' pattern on HRCT (n = 75) and IIP with 'inconsistent with UIP' pattern (n = 62) based on published guidelines. Predictors of AE and the prognosis after AE were examined in these groups. RESULTS: The 1-year incidence of AE in IPF, IIP with possible UIP HRCT patterns and IIP with inconsistent with UIP HRCT patterns was 16.5%, 8.9% and 4.0%, respectively. AE occurred significantly more frequently in IPF than in IIP with possible UIP and inconsistent with UIP HRCT patterns after adjustment for BMI, modified Medical Research Council score and %forced vital capacity. Prognosis of AE-IIP with possible UIP HRCT pattern was significantly worse than that of AE-IPF. CONCLUSION: Although AE occurred significantly less frequently in IIP with possible UIP and inconsistent with UIP HRCT patterns than in IPF, the prognosis of AE-IIP with possible UIP HRCT patterns might be worse than that of AE-IPF.


Assuntos
Pneumonias Intersticiais Idiopáticas , Pulmão , Idoso , Biópsia/métodos , Feminino , Humanos , Pneumonias Intersticiais Idiopáticas/diagnóstico , Pneumonias Intersticiais Idiopáticas/epidemiologia , Pneumonias Intersticiais Idiopáticas/fisiopatologia , Fibrose Pulmonar Idiopática/patologia , Incidência , Japão/epidemiologia , Pulmão/patologia , Pulmão/fisiopatologia , Masculino , Pessoa de Meia-Idade , Prognóstico , Testes de Função Respiratória/métodos , Testes de Função Respiratória/estatística & dados numéricos , Estudos Retrospectivos , Exacerbação dos Sintomas , Tomografia Computadorizada por Raios X/métodos
13.
Int J Tuberc Lung Dis ; 20(7): 978-84, 2016 07.
Artigo em Inglês | MEDLINE | ID: mdl-27287654

RESUMO

SETTING: Although the incidence and prevalence of idiopathic interstitial pneumonia (IIP) and idiopathic pulmonary fibrosis (IPF) have been assessed in Western countries, their epidemiology has not been analysed in Asian countries, including the Republic of Korea. OBJECTIVE: To estimate the prevalence and incidence of IIP, including IPF, in Korea, using a large, nationwide database. DESIGN: The Health Insurance Review and Assessment Services claims database, which includes information on every patient with diagnostic codes for IPF and IIP from 2010 to 2013, was reviewed. Age- and sex-specific IPF and IIP prevalence and incidence rates were estimated. RESULTS: Among Korean males and females, IPF prevalence from 2010 to 2013 was estimated at respectively 39.7 and 24.3 per 100 000 population, while IIP prevalence was estimated at respectively 97.1 and 66.5/100 000. The annual incidence rates among Korean males and females during 2011 and 2012 were respectively 16.4 and 9.7/100 000, for IPF, and respectively 42.3 and 27.5/100 000 for IIP. CONCLUSIONS: IPF is more prevalent in Korea than previously reported; its prevalence may be similar to or higher than in the United States and in European countries.


Assuntos
Pneumonias Intersticiais Idiopáticas/epidemiologia , Fibrose Pulmonar Idiopática/epidemiologia , Distribuição por Idade , Bases de Dados Factuais , Feminino , Humanos , Pneumonias Intersticiais Idiopáticas/diagnóstico , Fibrose Pulmonar Idiopática/diagnóstico , Incidência , Masculino , Prevalência , República da Coreia/epidemiologia , Distribuição por Sexo , Fatores de Tempo
14.
Respir Investig ; 53(2): 51-9, 2015 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-25745849

RESUMO

BACKGROUND: There have been no prospective large-scale multicenter epidemiological studies on the clinical course and treatment from the time of diagnosis of idiopathic interstitial pneumonias (IIPs) in Japan. The purpose of this study was to clarify the current clinical situation of IIP in Japan. METHODS: This study was supported by a grant from the Ministry of Health, Labour and Welfare to the Diffuse Lung Diseases Research Group. Data including clinical findings, course, and treatment of IIP from a web database created by a collaborative effort of medical institutions across Japan that specialize in the care of interstitial pneumonias were collected and analyzed. RESULTS: A total of 436 IIP patients from 19 institutions were newly registered during a 5-year period. Idiopathic pulmonary fibrosis (IPF) was the most frequently encountered IIP, and 28% of the IPF cases were initially diagnosed by abnormal chest X-ray or CT in asymptomatic patients. Until the 2008 fiscal year, no treatment was given for most cases of IPF. After the end of 2008, when pirfenidone was approved for manufacture, the number of patients for whom no treatment was recommended declined, and pirfenidone therapy was initiated in 32.9% of cases in 2009. The median survival times for IPF from the onset symptoms and from the initial visit were 105 months and 69 months, respectively. CONCLUSIONS: This study should provide valuable information for understanding the current state of IIP in Japan.


Assuntos
Pneumonias Intersticiais Idiopáticas/epidemiologia , Internet , Sistema de Registros/estatística & dados numéricos , Idoso , Feminino , Humanos , Pneumonias Intersticiais Idiopáticas/diagnóstico , Pneumonias Intersticiais Idiopáticas/tratamento farmacológico , Japão/epidemiologia , Masculino , Pessoa de Meia-Idade , Estudos Multicêntricos como Assunto , Prognóstico , Estudos Prospectivos , Piridonas/uso terapêutico , Taxa de Sobrevida , Fatores de Tempo
15.
Sarcoidosis Vasc Diffuse Lung Dis ; 31(4): 266-74, 2015 Jan 05.
Artigo em Inglês | MEDLINE | ID: mdl-25591137

RESUMO

BACKGROUND: Idiopathic interstitial pneumonias (IIP) are associated with an increased lung cancer (LC) risk. However, data on the prognostic and therapeutic impact are limited. We therefore aimed to analyze the outcome of IIP patients with LC under different treatment modalities. METHODS: Patients with IIPs diagnosed in a tertiary interstitial lung diseases (ILD) center were reviewed for LC diagnosis. RESULTS: Of 265 patients with idiopathic pulmonary fibrosis (IPF), 142 with non-specific interstitial pneumonia (NSIP), and 71 with cryptogenic organizing pneumonia (COP), 16%, 4%, and 6% were affected byLC, respectively. Patient characteristics were: IPF: 93% male, median age 67 years, forced vital capacity (FVC) 82%, diffusion capacity for Carbon monoxide (DLCO) 41%, mean survival 20 months. NSIP: 67% male, median age 70 years, FVC 72%, DLCO 43%, mean survival 35 months. COP: 50% male, median age 66 years, FVC 93%, DLCO 77%, mean survival 88 months. Significant treatment-related toxicities occurred in 55% IPF, 20% NSIP und 0% COP patients. 30-days postoperative mortality was 25% in IPF, and 0% in NSIP/COP while rate of radiation pneumonitis was 24% in IPF. CONCLUSIONS: LC is a frequent comorbidity in IIP, with a higher incidence and reduced survival in IPF compared to other IIPs. LC treatment is associated with significant toxicity, specifically in IPF. Interdisciplinary evaluation of therapeutic options in IIP patients diagnosed with LC is therefore mandatory.


Assuntos
Pneumonias Intersticiais Idiopáticas/epidemiologia , Neoplasias Pulmonares/terapia , Pulmão , Adulto , Idoso , Idoso de 80 Anos ou mais , Biópsia , Comorbidade , Feminino , Alemanha/epidemiologia , Humanos , Pneumonias Intersticiais Idiopáticas/diagnóstico , Pneumonias Intersticiais Idiopáticas/mortalidade , Pneumonias Intersticiais Idiopáticas/fisiopatologia , Estimativa de Kaplan-Meier , Pulmão/patologia , Pulmão/fisiopatologia , Pulmão/cirurgia , Neoplasias Pulmonares/diagnóstico , Neoplasias Pulmonares/mortalidade , Neoplasias Pulmonares/fisiopatologia , Masculino , Pessoa de Meia-Idade , Estadiamento de Neoplasias , Estudos Retrospectivos , Fatores de Risco , Centros de Atenção Terciária , Fatores de Tempo , Tomografia Computadorizada por Raios X , Resultado do Tratamento
16.
Eur J Radiol ; 84(1): 163-171, 2015 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-25439009

RESUMO

BACKGROUND: Because of growing body of interest on the association between fibrosing idiopathic interstitial pneumonias (f-IIP) and ischaemic heart disease, we initiated this prospective study to evaluate the prevalence of asymptomatic coronary artery disease (CAD) in patients with f-IIP. METHODS: Forty-two patients with f-IIP underwent noninvasive screening for CAD that included (a) a chest CT examination enabling calculation of the coronary artery calcium (CAC) score, then depiction of coronary artery stenosis; and (b) stress myocardial perfusion scintigraphy (MPS). Patients with significant coronary abnormalities, defined by a CAC score >400 or coronary artery stenosis >50% at CT and/or perfusion defect >5% at MPS, were referred to the cardiologist. Coronary angiography was indicated in presence of a perfusion defect >10% at MPS or significant left main or proximal left anterior descending stenosis whatever MPS findings. RESULTS: Combining CT and MPS, significant abnormalities were detected in 32/42 patients (76%). The cardiologist: (a) did not consider further investigation in 21 patients (CT abnormalities but no ischaemia at MPS: 12/21; false-positive findings at MPS: 3/21; poor respiratory condition: 6/21); (b) proceeded to coronary angiography in 11 patients which confirmed significant stenoses in 5 patients (5/42; 12%). In the worst-case-scenario (i.e., inclusion of 6 patients with significant coronary artery abnormalities who were not investigated due to poor respiratory condition), the prevalence of CAD reached 26% (11/42). CONCLUSION: In the studied population of patients with f-IIP, asymptomatic CAD ranged between 12% and 26%.


Assuntos
Doença da Artéria Coronariana/diagnóstico por imagem , Doença da Artéria Coronariana/epidemiologia , Pneumonias Intersticiais Idiopáticas/epidemiologia , Idoso , Comorbidade , Angiografia Coronária/métodos , Estenose Coronária/complicações , Estenose Coronária/diagnóstico por imagem , Feminino , Humanos , Masculino , Prevalência , Estudos Prospectivos , Tomografia Computadorizada por Raios X/métodos
17.
Eur Respir J ; 44(3): 594-602, 2014 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-25063244

RESUMO

Cigarette smoking is a key factor in the development of numerous pulmonary diseases. An international group of clinicians, radiologists and pathologists evaluated patients with previously identified idiopathic interstitial pneumonia (IIP) to determine unique features of cigarette smoking. Phase 1 (derivation group) identified smoking-related features in patients with a history of smoking (n=41). Phase 2 (validation group) determined if these features correctly predicted the smoking status of IIP patients (n=100) to participants blinded to smoking history. Finally, the investigators sought to determine if a new smoking-related interstitial lung disease phenotype could be defined. Phase 1 suggested that preserved forced vital capacity with disproportionately reduced diffusing capacity of the lung for carbon monoxide, and various radiographic and histopathological findings were smoking-related features. In phase 2, the kappa coefficient among clinicians was 0.16 (95% CI 0.11-0.21), among the pathologists 0.36 (95% CI 0.32-0.40) and among the radiologists 0.43 (95% CI 0.35-0.52) for smoking-related features. Eight of the 100 cases were felt to represent a potential smoking-related interstitial lung disease. Smoking-related features of interstitial lung disease were identified in a minority of smokers and were not specific for smoking. This study is limited by its retrospective design, the potential for recall bias in smoking history and lack of information on second-hand smoke exposure. Further research is needed to understand the relationship between smoking and interstitial lung disease.


Assuntos
Pneumonias Intersticiais Idiopáticas/induzido quimicamente , Pneumonias Intersticiais Idiopáticas/epidemiologia , Fumar/efeitos adversos , Adulto , Idoso , Monóxido de Carbono/química , Feminino , Humanos , Cooperação Internacional , Masculino , Rememoração Mental , México , Pessoa de Meia-Idade , Modelos Organizacionais , Prognóstico , Pneumologia/organização & administração , Pneumologia/normas , Radiologia , República da Coreia , Estudos Retrospectivos , Poluição por Fumaça de Tabaco , Reino Unido , Estados Unidos
18.
Ann Thorac Cardiovasc Surg ; 18(4): 314-7, 2012.
Artigo em Inglês | MEDLINE | ID: mdl-22446955

RESUMO

PURPOSE: Several small studies have reported that acute exacerbation (AE) of idiopathic interstitial pneumonia (IIP) can occur after lung resection for patients with non-small cell lung cancer, though the incidence rate is unclear. METHODS: We examined our institutional data and performed a search of the MEDLINE database for publications regarding AE of IIP following surgery for lung cancer. Studies reporting the incidence rates of IIP and AE were included. RESULTS: Eleven studies including our institutional data were determined to be eligible. Seven studies designated the incidence of IIP. Of 4749 patients (from 7 studies) who underwent lung resection for NSCLC, 277 had IIP, for an incidence rate of 5.8% (range 1.1%-11.7%). Eleven studies designated the incidence of AE from IIP patient, 67 (15.8%) of 424 IIP patients (from 11 studies) developed AE after surgery, of whom 38 (56.7%) died during the postoperative course. CONCLUSION: Coexistent IIP in patients with lung cancer increases the risk of lung cancer surgery. Furthermore, AE of IIP may be a major cause of operation-related death.


Assuntos
Carcinoma Pulmonar de Células não Pequenas/cirurgia , Pneumonias Intersticiais Idiopáticas/epidemiologia , Neoplasias Pulmonares/cirurgia , Pneumonectomia/efeitos adversos , Doença Aguda , Carcinoma Pulmonar de Células não Pequenas/epidemiologia , Carcinoma Pulmonar de Células não Pequenas/mortalidade , Comorbidade , Progressão da Doença , Humanos , Pneumonias Intersticiais Idiopáticas/mortalidade , Fibrose Pulmonar Idiopática/epidemiologia , Incidência , Japão/epidemiologia , Neoplasias Pulmonares/epidemiologia , Neoplasias Pulmonares/mortalidade , Pneumonectomia/mortalidade , Medição de Risco , Fatores de Risco , Fatores de Tempo , Resultado do Tratamento
19.
Arch. bronconeumol. (Ed. impr.) ; 47(supl.8): 15-19, dic. 2011. tab
Artigo em Espanhol | IBECS | ID: ibc-148040

RESUMO

Las neumonías intersticiales idiopáticas (NII) constituyen un reto diagnóstico para neumólogos, radiólogos y patólogos, comenzando por su clasificación, objeto de controversia en la actualidad. El estudio funcional respiratorio supone un elemento básico para establecer el diagnóstico, orientar en el pronóstico, controlar la evolución y monitorizar la respuesta al tratamiento. La exploración radiológica imprescindible para el diagnóstico de las NII es la tomografía computarizada de alta resolución (TCAR), que además puede ofrecer datos de la actividad y del pronóstico de la enfermedad (AU)


Idiopathic interstitial pneumonias (IIPs) are a diagnostic challenge for pneumologists, radiologists and pathologists alike. The classification of IPPs is currently controversial. Pulmonary function testing is an essential component in order to establish a diagnosis and is required to guide prognosis and follow-up and monitor treatment outcome. High-resolution computed tomography is also essential for the diagnosis of IPPs and may also provide data on disease activity and prognosis (AU)


Assuntos
Humanos , Pneumonias Intersticiais Idiopáticas/classificação , Pneumonias Intersticiais Idiopáticas/diagnóstico , Pneumonias Intersticiais Idiopáticas/epidemiologia , Tomografia Computadorizada por Raios X/métodos , Teste de Esforço , Medidas de Volume Pulmonar , Testes de Função Respiratória
20.
Eur Respir J ; 38(2): 384-91, 2011 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-21273390

RESUMO

Recent evidence suggests that idiopathic nonspecific interstitial pneumonia (iNSIP) is a distinct clinical entity amongst other idiopathic interstitial pneumonias, and some data seem to suggest a possible pathogenetic role of autoimmunity. The aim of the present study was to assess if iNSIP might represent an early lung manifestation of an autoimmune disease. After initial review of cases found in the medical records database by searching for the term "NSIP" (n = 63), 37 iNSIP cases were identified, and were re-evaluated using a dynamic integrated multidisciplinary approach. 27 cases with iNSIP were selected for the study. Mean ± sd age at first respiratory symptom was 54.2 ± 8 yrs, 70% were females, and 59% were never-smokers. At follow-up (mean ± sd 59.7 ± 29 months, range 12-138 months), autoimmune diseases occurred in 14 (52%) patients, with seven (26%) cases of autoimmune thyroiditis, six (22%) of undifferentiated connective tissue disease and three (11%) of connective tissue disease. Patients developing autoimmune diseases were older and more frequently never-smoking females. In >50% of patients diagnosed with iNSIP, evidence of autoimmune diseases develops within 2 yrs, suggesting a probable link between the clinical entity of iNSIP and autoimmune disorders.


Assuntos
Doenças Autoimunes/epidemiologia , Pneumonias Intersticiais Idiopáticas/epidemiologia , Doenças Autoimunes/diagnóstico , Doenças Autoimunes/diagnóstico por imagem , Doenças do Tecido Conjuntivo/diagnóstico , Doenças do Tecido Conjuntivo/diagnóstico por imagem , Doenças do Tecido Conjuntivo/epidemiologia , Feminino , Seguimentos , Humanos , Pneumonias Intersticiais Idiopáticas/diagnóstico , Pneumonias Intersticiais Idiopáticas/diagnóstico por imagem , Incidência , Masculino , Pessoa de Meia-Idade , Prevalência , Fibrose Pulmonar/diagnóstico , Fibrose Pulmonar/diagnóstico por imagem , Fibrose Pulmonar/epidemiologia , Radiografia , Estudos Retrospectivos , Fumar/epidemiologia , Tireoidite Autoimune/diagnóstico , Tireoidite Autoimune/diagnóstico por imagem , Tireoidite Autoimune/epidemiologia
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