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INTRODUCTION: Pleuroparenchymal fibroelastosis (PPFE) findings are associated with poor prognosis in interstitial lung disease (ILD). However, the effect of PPFE findings on the development of immune checkpoint inhibitor-related pneumonitis (ICI-pneumonitis), a life-threatening adverse event, in lung cancer patients with ILD has not been elucidated. We aimed to determine whether PPFE findings are a risk factor for ICI-pneumonitis in lung cancer patients with ILD. METHODS: We retrospectively examined 712 lung cancer patients, including 173 patients with background ILDs, who received ICI therapy in our institute between December 2015 and May 2021. Background ILDs were radiologically classified into three types: lone PPFE, other ILDs with PPFE, and other ILDs without PPFE. The cumulative ICI-pneumonitis incidence curves and median overall survival (mOS) were compared between the three radiological types, and risk factors for ICI-pneumonitis were evaluated. RESULTS: Of 173 eligible patients with ILD, 23 patients (13.3%) experienced ICI-pneumonitis. The Kaplan-Meier method and the log-rank test showed that lone PPFE patients had significantly lower incidence of ICI-pneumonitis (p = 0.024) and longer mOS (575 vs. 326 days; p = 0.0096) than other ILDs patients. ICI-pneumonitis (p = 0.35) and mOS (p = 0.29) were not significantly different between other ILDs with and without PPFE. A multivariate Cox proportional hazards regression analysis revealed that lone PPFE pattern was an independent predictive factor for ICI-pneumonitis (hazard ratio, 0.20; 95% confidence interval, 0.043-0.93; p = 0.040). CONCLUSION: ICI therapy could be safer in lone PPFE patients than in other ILDs patients with lung cancer.
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Doenças Pulmonares Intersticiais , Neoplasias Pulmonares , Pneumonia , Humanos , Inibidores de Checkpoint Imunológico/efeitos adversos , Estudos Retrospectivos , Tomografia Computadorizada por Raios X/métodos , Doenças Pulmonares Intersticiais/induzido quimicamente , Doenças Pulmonares Intersticiais/diagnóstico por imagem , Neoplasias Pulmonares/complicações , Neoplasias Pulmonares/tratamento farmacológico , Pulmão/diagnóstico por imagemRESUMO
BACKGROUND: Osteoblastic bone reaction (OBR) refers to an increase in bone density at the site of bone metastasis or the appearance of new sclerotic bone lesions after anticancer treatment. OBR can be misunderstood as disease progression. In this study, we aimed to investigate the prevalence and details of OBR and its association with clinical outcomes in patients with epidermal growth factor receptor (EGFR)-mutant non-small cell lung cancer (NSCLC) treated with osimertinib. METHODS: This was a single-center, retrospective cohort study. We reviewed patients who were diagnosed with EGFR-mutant NSCLC with bone metastasis and received osimertinib as a first-line treatment between February 2018 and October 2022. The OBR was evaluated by comparing baseline computed tomography (CT) scans with the first CT scan after treatment initiation. RESULTS: A total of 45 patients were included in this study. Thirty-seven patients (82%) developed OBR. OBR developed in 94% (n = 16) of patients with sclerotic bone lesions (n = 17) at baseline. Similarly, OBR developed in lytic and mixed bone lesions in 76% and 82% of patients with lytic and mixed lesions, respectively. Progression-free survival (PFS) did not differ significantly between patients with (OBR group) and without OBR (non-OBR group) (median PFS, 24 months vs. 17 months; hazard ratio (HR), 0.62; 95% CI, 0.24-1.6; p = 0.31). In univariate analysis, the OBR group showed a trend toward longer skeletal-related events-free survival (SRE-FS) than the non-OBR group (median SRE-FS, 26 months vs. 12 months; HR, 0.53; 95% CI, 0.21-1.33; p = 0.16). Multivariate analysis showed OBR was a significant independent predictor of SRE-FS (HR, 0.35; 95% CI, 0.13-0.92; p = 0.034). CONCLUSIONS: OBR developed in most patients with NSCLC and bone metastasis who received osimertinib treatment. The increased incidence of OBR in patients with EGFR-mutant NSCLC with bone metastasis treated with osimertinib should not be confused with disease progression, and treatment decisions should be made carefully.
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Doenças Ósseas , Carcinoma Pulmonar de Células não Pequenas , Neoplasias Pulmonares , Humanos , Carcinoma Pulmonar de Células não Pequenas/tratamento farmacológico , Carcinoma Pulmonar de Células não Pequenas/genética , Estudos Retrospectivos , Neoplasias Pulmonares/tratamento farmacológico , Neoplasias Pulmonares/genética , Progressão da Doença , Receptores ErbB/genética , MutaçãoRESUMO
BACKGROUND: Idiopathic non-specific interstitial pneumonia (iNSIP), idiopathic pleuroparenchymal fibroelastosis (iPPFE), and unclassifiable idiopathic interstitial pneumonia (IIP) are IIPs with chronic fibrotic phenotypes, and unlike idiopathic pulmonary fibrosis, they have often been treated with anti-inflammatory drugs, including corticosteroids and immunosuppressants. However, the impact of bronchoalveolar lavage (BAL) lymphocytosis on the effects of anti-inflammatory therapy has never been evaluated. This study aimed to elucidate whether BAL lymphocytosis can be used to predict the efficacy of anti-inflammatory drugs for iNSIP, iPPFE, and unclassifiable IIP. METHODS: Japanese patients diagnosed with iNSIP, iPPFE, and unclassifiable IIP by multidisciplinary discussion were identified using the nationwide registry. Eligible patients were stratified into four groups with and without BAL lymphocytosis and anti-inflammatory therapy to compare overall survival (OS) and changes in lung function. BAL lymphocytosis was defined as a lymphocyte differential count > 15%, and the cut-off was corroborated by survival classification and regression tree analysis. RESULTS: Overall, 186 patients (37 iNSIP, 16 iPPFE, and 133 unclassifiable IIP) were analyzed. Limited to patients treated with anti-inflammatory drugs (n = 123), patients with BAL lymphocytosis had a better prognosis [hazard ratio (HR), 0.26; 95% confidence interval (CI), 0.11-0.63; P = 0.003], higher slope of forced vital capacity (FVC) % predicted for 2 years, and longer OS (log-rank test, P = 0.012) than those without BAL lymphocytosis. On multivariate analysis, BAL lymphocytosis (HR 0.31; 95% CI 0.13-0.75; P = 0.009) was a prognostic factor for OS, along with age and FVC % predicted. Conversely, for patients managed without anti-inflammatory therapy (n = 63), the presence or absence of BAL lymphocytosis had no prognostic value. CONCLUSIONS: BAL lymphocytosis is associated with good outcomes in patients treated with anti-inflammatory drugs, but has no prognostic value when anti-inflammatory drugs are not used. BAL lymphocytosis may provide a predictive biomarker for identifying patients with iNSIP, iPPFE and unclassifiable IIP who are likely to benefit from anti-inflammatory drugs.
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Anti-Inflamatórios/uso terapêutico , Pneumonias Intersticiais Idiopáticas/tratamento farmacológico , Fibrose Pulmonar Idiopática/tratamento farmacológico , Pulmão/efeitos dos fármacos , Linfocitose/imunologia , Idoso , Anti-Inflamatórios/efeitos adversos , Líquido da Lavagem Broncoalveolar/imunologia , Feminino , Humanos , Pneumonias Intersticiais Idiopáticas/imunologia , Pneumonias Intersticiais Idiopáticas/mortalidade , Pneumonias Intersticiais Idiopáticas/fisiopatologia , Fibrose Pulmonar Idiopática/imunologia , Fibrose Pulmonar Idiopática/mortalidade , Fibrose Pulmonar Idiopática/fisiopatologia , Japão , Pulmão/imunologia , Pulmão/fisiopatologia , Masculino , Pessoa de Meia-Idade , Sistema de Registros , Estudos Retrospectivos , Medição de Risco , Fatores de Risco , Fatores de Tempo , Resultado do Tratamento , Capacidade VitalRESUMO
BACKGROUND: Pleuroparenchymal fibroelastosis (PPFE) is characterised by predominant upper lobe pleural and subpleural lung parenchymal fibrosis. Radiological PPFE-like lesion has been associated with various types of interstitial lung diseases. However, the prevalence and clinical significance of radiological PPFE-like lesion in patients with idiopathic interstitial pneumonias (IIPs) are not fully understood. We aimed to determine the prevalence and clinical impact on survival of radiological PPFE-like lesion in patients with IIPs. METHODS: A post-hoc analysis was conducted using data from the Japanese nationwide cloud-based database of patients with IIPs. All the patients in the database were diagnosed as having IIPs by multidisciplinary discussion. Patients diagnosed with idiopathic PPFE were excluded. Clinical data and chest computed tomography (CT) image of 419 patients with IIPs were analysed. The presence of radiological PPFE-like lesion was independently evaluated by two chest radiologists blind to the clinical data. RESULTS: Of the 419 patients with IIPs, radiological PPFE-like lesions were detected in 101 (24.1%) patients, mainly in idiopathic pulmonary fibrosis (IPF) and unclassifiable IIPs, but less in idiopathic nonspecific interstitial pneumonia. Prognostic analyses revealed that radiological PPFE-like lesion was significantly associated with poor outcome in patients with IIPs, which was independent of age, IPF diagnosis and %FVC. In survival analyses, the patients with radiological PPFE-like lesions had poor survival compared with those without (log-rank, p < 0.0001). Subgroup analyses demonstrated that radiological PPFE-like lesion was significantly associated with poor survival both in patients with IPF and those with unclassifiable IIPs. CONCLUSION: Radiological PPFE-like lesion is a condition that could exist in IIPs, mainly in IPF and unclassifiable IIPs. Importantly, the radiological PPFE-like lesion is a non-invasive marker to predict poor outcome in patients with IIPs, which should be carefully considered in clinical practice.
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Pneumonias Intersticiais Idiopáticas/diagnóstico , Fibrose Pulmonar Idiopática/diagnóstico , Pulmão/diagnóstico por imagem , Radiografia Torácica/métodos , Tomografia Computadorizada por Raios X/métodos , Idoso , Feminino , Seguimentos , Humanos , Pneumonias Intersticiais Idiopáticas/complicações , Fibrose Pulmonar Idiopática/etiologia , Masculino , Pessoa de Meia-Idade , Prognóstico , Estudos RetrospectivosRESUMO
Recent studies have suggested that in patients with an idiopathic interstitial pneumonia (IIP), a probable usual interstitial pneumonia (UIP) pattern on chest computed tomography (CT) is sufficient to diagnose idiopathic pulmonary fibrosis (IPF) without histopathology.We retrospectively compared the prognosis and time to first acute exacerbation (AE) in IIP patients with a UIP and a probable UIP pattern on initial chest CT.One hundred and sixty IIP patients with a UIP pattern and 242 with a probable UIP pattern were identified. Probable UIP pattern was independently associated with longer survival time (adjusted hazard ratio 0.713, 95% CI 0.536-0.950; p=0.021) and time to first AE (adjusted hazard ratio 0.580, 95% CI 0.389-0.866; p=0.008). In subjects with a probable UIP pattern who underwent surgical lung biopsy, the probability of a histopathological UIP pattern was 83%. After multidisciplinary discussion and the inclusion of longitudinal behaviour, a diagnosis of IPF was made in 66% of cases. In IPF patients, survival time and time to first AE were not associated with CT pattern. Among subjects with a probable UIP pattern, compared to non-IPF patients, survival time and time to first AE were shorter in IPF patients.In conclusion, IIP patients with a probable UIP pattern on initial chest CT had a better prognosis and longer time to first AE than those with a UIP pattern. However, when baseline data and longitudinal behaviour provided a final diagnosis of IPF, CT pattern was not associated with these outcomes. This suggests diagnostic heterogeneity among patients with a probable UIP pattern.
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Pneumonias Intersticiais Idiopáticas , Fibrose Pulmonar Idiopática , Humanos , Pneumonias Intersticiais Idiopáticas/diagnóstico por imagem , Fibrose Pulmonar Idiopática/diagnóstico por imagem , Pulmão/diagnóstico por imagem , Prognóstico , Modelos de Riscos Proporcionais , Estudos Retrospectivos , Tomografia Computadorizada por Raios XRESUMO
Multidisciplinary discussion (MDD) requiring close communication between specialists (clinicians, radiologists and pathologists) is the gold standard for the diagnosis of idiopathic interstitial pneumonias (IIPs). However, MDD by specialists is not always feasible because they are often separated by time and location. An online database would facilitate data sharing and MDD. Our aims were to develop a nationwide cloud-based integrated database containing clinical, radiological and pathological data of patients with IIPs along with a web-based MDD system, and to validate the diagnostic utility of web-based MDD in IIPs.Clinical data, high-resolution computed tomography images and lung biopsy slides from patients with IIPs were digitised and uploaded to separate servers to develop a cloud-based integrated database. Web-based MDD was performed using the database and video-conferencing to reach a diagnosis.Clinical, radiological and pathological data of 524 patients in 39 institutions were collected, uploaded and incorporated into the cloud-based integrated database. Subsequently, web-based MDDs with a pulmonologist, radiologist and pathologist using the database and video-conferencing were successfully performed for the 465 cases with adequate data. Overall, the web-based MDD changed the institutional diagnosis in 219 cases (47%). Notably, the MDD diagnosis yielded better prognostic separation among the IIPs than did the institutional diagnosis.This is the first study of developing a nationwide cloud-based integrated database containing clinical, radiological and pathological data for web-based MDD in patients with IIPs. The database and the web-based MDD system that we built made MDD more feasible in practice, potentially increasing accurate diagnosis of IIPs.
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Computação em Nuvem , Gerenciamento de Dados/organização & administração , Pneumonias Intersticiais Idiopáticas/diagnóstico , Comunicação Interdisciplinar , Idoso , Biópsia , Bases de Dados Factuais , Diagnóstico Diferencial , Feminino , Humanos , Pneumonias Intersticiais Idiopáticas/mortalidade , Japão/epidemiologia , Masculino , Pessoa de Meia-Idade , Patologistas , Padrões de Prática Médica , Pneumologistas , Radiologistas , Análise de Sobrevida , Tomografia Computadorizada por Raios XRESUMO
BACKGROUND AND OBJECTIVE: Pneumothorax is a co-morbidity in patients with idiopathic pulmonary fibrosis (IPF). However, its incidence, risk factors and prognostic significance in IPF remain unclear. The aim of this study was to clarify the incidence and prognostic significance of pneumothorax in patients with IPF, and to further investigate the risk factors for its onset. METHODS: Eighty-four consecutive patients with IPF based on the consensus guideline were included in this study. We retrospectively reviewed the medical records, pulmonary function tests and chest high-resolution computed tomography images, and determined the incidence of pneumothorax. The prognostic significance of pneumothorax was evaluated using the Cox proportional hazards model analysis with time-dependent covariates. We also assessed the cumulative incidence and the risk factors for pneumothorax. RESULTS: Of the 84 patients, 17 (20.2%) developed pneumothorax. The cumulative incidence of pneumothorax was 8.5%, 12.5% and 17.7% at 1, 2 and 3 years, respectively. Univariate analysis demonstrated that pneumothorax was significantly related to poor prognosis (hazards ratio, 2.99; P = 0.002). Multivariate analysis, adjusting for sex, age and forced vital capacity (% predicted), revealed that pneumothorax was an independent predictor of poor outcome in IPF (hazards ratio, 2.85; P = 0.006). Lower BMI and the presence of extensive reticular abnormalities were significantly associated with developing pneumothorax. CONCLUSION: These results confirm that patients with IPF often develop pneumothorax during their clinical course and that the onset of pneumothorax predicts a poor outcome.
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Fibrose Pulmonar Idiopática/epidemiologia , Pneumotórax/epidemiologia , Idoso , Idoso de 80 Anos ou mais , Índice de Massa Corporal , Comorbidade , Feminino , Humanos , Fibrose Pulmonar Idiopática/diagnóstico por imagem , Fibrose Pulmonar Idiopática/fisiopatologia , Incidência , Masculino , Pessoa de Meia-Idade , Pneumotórax/diagnóstico por imagem , Pneumotórax/fisiopatologia , Prognóstico , Modelos de Riscos Proporcionais , Estudos Retrospectivos , Fatores de Risco , Fatores de Tempo , Tomografia Computadorizada por Raios XRESUMO
BACKGROUND: Chronic eosinophilic pneumonia (CEP) is characterized by the accumulation of eosinophils in the lung with unknown etiology. Although systemic corticosteroid administration leads to dramatic improvement, nearly half the patients with CEP experience relapse and some develop persistent impairment of pulmonary function. However, predictive factors for this persistent impairment have not been determined. OBJECTIVE: To investigate the occurrence of persistent impairment of pulmonary function in CEP and determine its predictive factors. METHODS: This observational study consisted of 133 consecutive patients with CEP who were followed for longer than 1 year. Spirometry was performed at the time of diagnosis and at follow-up. RESULTS: During the observational period (6.1 ± 4.1 years), relapse occurred in 75 patients (56.4%). Remarkably, 42 patients (31.6%) had a persistent pulmonary function defect (27 obstructive, 10 restrictive, and 4 obstructive and restrictive cases) at the last evaluation. Logistic analyses showed that the relapse was associated with neither persistent obstructive nor restrictive defects. Persistent obstructive defect was significantly associated with the comorbidity of asthma and obstructive defect at the initial CEP diagnosis, whereas persistent restrictive defect was significantly related to reticulation at high-resolution computer tomography and restrictive defect at diagnosis. CONCLUSION: Persistent impairment of pulmonary function is common in CEP. Concurrent asthma and obstructive defects at diagnosis were predictors for persistent obstructive impairments, whereas reticulation at high-resolution computer tomography and restrictive defect at diagnosis predicted persistent restrictive impairment. Attention should be paid to these persistent impairments in the management of CEP. TRIAL REGISTRATION: http://www.umin.ac.jp/ctr/index-j.htm Identifier: UMIN000019092 (principal investigator, Takafumi Suda, MD, PhD).
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Asma/epidemiologia , Eosinófilos/imunologia , Pulmão/fisiologia , Eosinofilia Pulmonar/diagnóstico , Adulto , Idoso , Idoso de 80 Anos ou mais , Doença Crônica , Comorbidade , Feminino , Glucocorticoides/uso terapêutico , Humanos , Japão/epidemiologia , Estudos Longitudinais , Masculino , Pessoa de Meia-Idade , Prednisolona/uso terapêutico , Prognóstico , Eosinofilia Pulmonar/tratamento farmacológico , Eosinofilia Pulmonar/epidemiologia , Recidiva , Espirometria , Adulto JovemRESUMO
PURPOSE: Small airway disease (SAWD) in patients with interstitial lung disease (ILD) is often assessed by high-resolution computed tomography (HRCT). However, frequent HRCT examinations result in a high level of radiographic exposure. This study investigated the utility of the forced oscillation technique (FOT) to evaluate SAWD in patients with ILD. METHODS: Broadband FOT using a commercially available device (MostGraph-01) and pulmonary function tests (PFT) were performed in 90 patients with ILD. HRCT images taken within 3 months were reviewed. The patients were divided into two groups according to the presence or absence of SAWD findings detected by HRCT. Clinical characteristics, PFT, and FOT between the two groups were compared. RESULTS: Of the 90 patients with ILD, 19 were classified as having SAWD findings (the presence group) and 71 as not having SAWD findings (the absence group). There were no significant differences in parameters of PFT between the two groups. The presence group had higher absolute values of reactance at 5 Hz (X5), resonant frequency (Fres), and low-frequency reactance area (ALX) than did the absence group. A within-breath change analysis demonstrated that the change in X5, Fres, and ALX between expiration and inspiration (ΔX5, ΔFres, ΔALX, respectively) was significantly different between the groups. A univariate analysis revealed that X5, Fres, ALX, ΔX5, ΔFres, ΔALX were significantly associated with the presence of SAWD findings. Multivariate analysis validated that Fres was related to the presence of SAWD findings. CONCLUSIONS: The FOT may be useful in detecting and evaluating SAWD in patients with ILD. TRIAL REGISTRATION: UMIN 000020733.
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Doenças Pulmonares Intersticiais/fisiopatologia , Pneumopatias Obstrutivas/fisiopatologia , Mecânica Respiratória , Adulto , Idoso , Idoso de 80 Anos ou mais , Resistência das Vias Respiratórias , Área Sob a Curva , Expiração , Feminino , Humanos , Inalação , L-Lactato Desidrogenase/sangue , Doenças Pulmonares Intersticiais/complicações , Doenças Pulmonares Intersticiais/diagnóstico por imagem , Pneumopatias Obstrutivas/diagnóstico por imagem , Pneumopatias Obstrutivas/etiologia , Masculino , Pessoa de Meia-Idade , Mucina-1/sangue , Valor Preditivo dos Testes , Proteína D Associada a Surfactante Pulmonar/sangue , Curva ROC , Testes de Função Respiratória , Tomografia Computadorizada por Raios X , Adulto JovemRESUMO
PURPOSE: To perform volumetric analysis of stage I lung adenocarcinomas by using an automated computer program and to determine value of volumetric computed tomographic (CT) measurements associated with prognostic factors and outcome. MATERIALS AND METHODS: Consecutive patients (n = 145) with stage I lung adenocarcinoma who underwent surgery after preoperative chest CT were enrolled. By using volumetric automated computer-assisted analytic program, nodules were classified into three subgroups: pure ground glass, part solid, or solid. Total tumor volume, solid tumor volume, and percentage of solid volume of each cancer were calculated after eliminating vessel components. One radiologist measured the longest diameter of the solid tumor component and of total tumor with their ratio, which was defined as solid proportion. The value of these quantitative data by examining associations with pathologic prognostic factors and outcome measures (disease-free survival and overall survival) were analyzed with logistic regression and Cox proportional hazards regression models, respectively. Significant parameters identified at univariate analysis were included in the multiple analyses. RESULTS: All 22 recurrences occurred in patients with nodules classified as part solid or solid. Multiple logistic regression analysis revealed that percentage of solid volume of 63% or greater was an independent indicator associated with pleural invasion (P = .01). Multiple Cox proportional hazards regression analysis revealed that percentage of solid volume of 63% or greater was a significant indicator of lower disease-free survival (hazard ratio, 18.45 [95% confidence interval: 4.34, 78.49]; P < .001). Both solid tumor volume of 1.5 cm(3) or greater and percentage of solid volume of 63% or greater were significant indicators of decreased overall survival (hazard ratio, 5.92 and 9.60, respectively [95% confidence interval: 1.17, 30.33 and 1.17, 78.91, respectively]; P = .034 and .036, respectively). CONCLUSION: Two volumetric measurements (solid volume, ≥1.5 cm(3); percentage of solid volume, ≥63%) were found to be independent indicators associated with increased likelihood of recurrence and/or death in patients with stage I adenocarcinoma.
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Adenocarcinoma/patologia , Neoplasias Pulmonares/patologia , Tomografia por Emissão de Pósitrons , Tomografia Computadorizada por Raios X , Adenocarcinoma/diagnóstico por imagem , Adulto , Idoso , Idoso de 80 Anos ou mais , Feminino , Fluordesoxiglucose F18 , Humanos , Neoplasias Pulmonares/diagnóstico por imagem , Masculino , Pessoa de Meia-Idade , Estadiamento de Neoplasias , Prognóstico , Interpretação de Imagem Radiográfica Assistida por Computador , Compostos Radiofarmacêuticos , Software , Taxa de Sobrevida , Carga TumoralRESUMO
PURPOSE: To assess the variability of computed tomography (CT) patterns in patients with pathologic nonspecific interstitial pneumonia (NSIP) and to evaluate correlation of CT patterns with new idiopathic pulmonary fibrosis (IPF) classification guidelines, including pathologic diagnosis and predicted mortality. MATERIALS AND METHODS: The ethical review boards of the five institutions that contributed cases waived the need for informed consent for retrospective review of patient records and images. The study included 114 patients with (a) a pathologic diagnosis of idiopathic NSIP (n = 39) or (b) a pathologic diagnosis of usual interstitial pneumonia (UIP) and a clinical diagnosis of IPF (n = 75). Two groups of independent observers evaluated the extent and distribution of various CT findings and identified the following five patterns: UIP, possible UIP, indeterminate (either UIP or NSIP), NSIP, and suggestive of an alternative diagnosis. CT findings were compared with pathologic diagnoses and outcome from clinical findings by using the log-rank test and Kaplan-Meier curves. RESULTS: Radiologists classified 17 cases as UIP, 24 as possible UIP, 13 as indeterminate (either UIP or NSIP), and 56 as NSIP. In 35 of 39 patients with pathologic NSIP, a diagnosis of NSIP was made with CT. On the basis of CT interpretations, the mean overall survival time of patients with UIP, possible UIP, indeterminate findings, or NSIP was 33.5, 73.0, 101.0, and 140.2 months, respectively. Outcome of patients with a CT diagnosis of UIP was significantly worse than that of patients with a pattern of possible UIP, indeterminate findings, or NSIP (log-rank test: P = .013, P = .018, and P < .001, respectively). CONCLUSION: CT pattern in patients with pathologic NSIP is more uniform than that in patients with pathologic UIP, and CT NSIP pattern is associated with better patient outcome than is CT UIP pattern.
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Pneumonias Intersticiais Idiopáticas/diagnóstico por imagem , Tomografia Computadorizada por Raios X/métodos , Adulto , Idoso , Idoso de 80 Anos ou mais , Biópsia , Diagnóstico Diferencial , Feminino , Humanos , Pneumonias Intersticiais Idiopáticas/mortalidade , Pneumonias Intersticiais Idiopáticas/patologia , Pulmão/patologia , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Interpretação de Imagem Radiográfica Assistida por Computador , Testes de Função Respiratória , Análise de SobrevidaRESUMO
PURPOSE: We retrospectively analyzed patients with clinically diagnosed interstitial pneumonia to investigate the factors which contribute to the difference in prognosis from the initiation of long-term oxygen therapy (LTOT) among subtypes. METHODS: Seventy-six patients with clinically diagnosed idiopathic interstitial pneumonia (IIP; n = 49) or interstitial pneumonia associated with collagen vascular disease (CVD-IP; n = 27) in whom LTOT was initiated in our facility from January 1999 to December 2012 were analyzed. RESULTS: Patients with CVD-IP had significantly longer survival time from the initiation of LTOT than those with IIP with the median survival of 51.7 months versus 18.8 months, respectively. The 1-year survival rate was 92.4% for patients with CVD-IP versus 76.5% for those with IIP, and 2-year survival was 88.6 versus 36.0%, respectively. The patterns classified with high-resolution computed tomography (HRCT) were not associated with prognosis. The association between pulmonary hypertension and prognosis was unclear. In results of the multivariate Cox analysis which included factors demonstrating p < 0.1 in the univariate Cox analysis, male gender, low body mass index, and the absence of collagen vascular disease (CVD) were significantly associated with poor prognosis. CONCLUSIONS: After the initiation of LTOT, patients with IIP had poor prognosis regardless of the patterns classified with HRCT, while those with CVD-IP survived longer. Male gender, low body mass index, and the absence of CVD were the independent negative prognostic factors in patients with interstitial pneumonia receiving LTOT.
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Doenças do Colágeno/terapia , Doenças Pulmonares Intersticiais/terapia , Oxigenoterapia , Doenças Vasculares/terapia , Idoso , Índice de Massa Corporal , Doenças do Colágeno/diagnóstico , Doenças do Colágeno/mortalidade , Doenças do Colágeno/fisiopatologia , Feminino , Humanos , Japão , Estimativa de Kaplan-Meier , Pulmão/fisiopatologia , Doenças Pulmonares Intersticiais/diagnóstico , Doenças Pulmonares Intersticiais/mortalidade , Doenças Pulmonares Intersticiais/fisiopatologia , Masculino , Pessoa de Meia-Idade , Análise Multivariada , Oxigenoterapia/efeitos adversos , Oxigenoterapia/mortalidade , Modelos de Riscos Proporcionais , Estudos Retrospectivos , Fatores de Risco , Fatores Sexuais , Fatores de Tempo , Tomografia Computadorizada por Raios X , Resultado do Tratamento , Doenças Vasculares/diagnóstico , Doenças Vasculares/mortalidade , Doenças Vasculares/fisiopatologiaRESUMO
BACKGROUND: Anti-synthetase syndrome-associated interstitial lung disease (ASS-ILD) may occur without myositis. Although a recent Japanese guide proposed a watch-and-wait approach for chronic ASS-ILD without obvious progression, the natural history of this subgroup and the appropriateness of the watch-and-wait approach remain unclear. We aimed to describe the natural history of ASS-ILD, that is sufficiently indolent to be a candidate for the watch-and-wait approach. METHODS: Among consecutive patients with ASS-ILD, we retrospectively identified those without myositis, acute/subacute onset, and significant lung function impairment, which qualified them as indolent-ASS-ILD cases, and described their natural course. Additionally, we evaluated the risk factors for fibrosis progression on computed tomography (CT) using the Cox proportional hazards model. RESULTS: Among 80 patients with ASS-ILD, we identified 33 with indolent-ASS-ILD, all of whom were initially followed up with a watch-and-wait approach. Among 30 patients with sufficient follow-up data, 27 (90%) showed a stable course without treatment over 24 months. Subsequently, four patients experienced ≥10% relative forced vital capacity (FVC) decline without treatment during a median follow-up duration of 81 months. Seven patients showed fibrosis progression with >10% increase in the total lung area on CT. Higher levels of Krebs von den Lungen-6 (KL-6) and surfactant protein-D (SP-D) were associated with fibrosis progression on CT. CONCLUSION: Most patients with indolent-ASS-ILD did not experience ≥10% relative FVC decline over five years without treatment. However, fibrosis progression on CT, which seemed to precede significant FVC decline, occurred more frequently, especially in patients with higher KL-6 and SP-D levels.
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Progressão da Doença , Doenças Pulmonares Intersticiais , Tomografia Computadorizada por Raios X , Humanos , Doenças Pulmonares Intersticiais/etiologia , Masculino , Feminino , Estudos Retrospectivos , Pessoa de Meia-Idade , Idoso , Capacidade Vital , Mucina-1/sangue , Fatores de Risco , Miosite/complicaçõesRESUMO
Idiopathic inflammatory myopathies (IIM) are a heterogeneous group of autoimmune diseases characterized by muscle involvement and various extramuscular manifestations. Interstitial lung disease (ILD) is one of the most common extramuscular manifestations of IIM and is associated with significant mortality and morbidity. The clinical phenotypes, treatment responses, and prognosis of IIM-ILD are significantly related to myositis-specific antibody (MSA) profiles, with some racial differences. The features associated with MSA in IIM-ILD could also be relevant to cases of ILD where MSA is present but does not meet the criteria for IIM. The anti-melanoma differentiation-associated gene 5 antibody is highly associated with rapidly progressive ILD (RP-ILD), especially in Asian populations, and with characteristic cutaneous manifestations, such as skin ulcers. Radiologically, ground-glass opacities, consolidations, and nonsegmental linear opacities were more predominant than reticular opacities and honeycombing. While the mortality rate is still around 30%, the prognosis can be improved with early intensive therapy with corticosteroids and multiple immunosuppressants. In contrast, anti-aminoacyl-tRNA synthetase (ARS) antibodies are associated with chronic ILD, although RP-ILD is also common. Patients with anti-ARS antibodies often show lung-predominant presentations, with subtle muscle and skin involvement. Radiologically, reticular opacities, with or without consolidation, are predominant and may progress to honeycombing over time. Combination therapy with corticosteroids and a single immunosuppressant is recommended to prevent relapses, which often lead to a decline in lung function and fatal long-term outcomes. Significant advances in immunology and genetics holds promise for fostering more personalized approaches to managing IIM-ILD.
RESUMO
BACKGROUND: Usual interstitial pneumonia (UIP) cases without honeycombing (possible UIP) included various CT features and was often difficult to diagnose. PURPOSE: This study aimed to classify the cases with possible UIP on CT features using cluster analysis and evaluate the features of subsets of participants and the correlation of prognosis. MATERIALS AND METHODS: The study included 85 patients with possible UIP in the 2011 idiopathic pulmonary fibrosis (IPF) guideline with radiological diagnosis. All cases underwent surgical biopsies and were diagnosed by multidisciplinary discussion (MDD) from the nationwide registry in Japan. The readers evaluated pulmonary opacity, nodules, cysts, and predominant distribution which were reclassified by IPF guidelines in 2018. Additionally, cases were classified into four groups by cluster analysis based on CT findings. The differences in survival among IPF classification and the clusters were evaluated. RESULTS: Cases were diagnosed as IPF (n = 55), NSIP (n = 4), unclassifiable (n = 23), and others (n = 3) by MDD. Cluster analysis revealed 4 clusters by CT features (n = 47, 16, 19 and 3, respectively). Cluster 1 had fewer lesions overall. Cluster 2 have many pure ground-glass opacities and ground-glass opacities with reticulation. Cluster 3 had many reticular opacities and nodules with few lower predominant distributions. Cluster 4 was characterized by peribronchovascular consolidation.The mean survival time of cluster 1 (4518 days) was significantly better than cluster 2, 3, and 4 (1843, 2196, and 1814 days, respectively) (p = 0.03). CONCLUSION: In conclusion, UIP without honeycombing included various CT patterns and MDD diagnoses. Significangly differences in prognosis were observed among clusters classified by CT findings.
Assuntos
Fibrose Pulmonar Idiopática , Tomografia Computadorizada por Raios X , Humanos , Masculino , Feminino , Tomografia Computadorizada por Raios X/métodos , Idoso , Fibrose Pulmonar Idiopática/diagnóstico por imagem , Pessoa de Meia-Idade , Prognóstico , Japão , Idoso de 80 Anos ou mais , Análise por ConglomeradosRESUMO
BACKGROUND AND PURPOSE: Idiopathic dendriform pulmonary ossification (DPO) is mostly asymptomatic, and detected incidentally in lung CT. There have been no reports on the precise CT-pathologic correlation and the prevalence of idiopathic DPO. This study aimed to clarify the histological background and prevalence of idiopathic DPO. MATERIALS AND METHODS: Sixteen patients with histologically confirmed idiopathic DPO (12 men and 4 women; mean age, 38.8 years; range 22-56 years) were identified in a nationwide epidemiological survey. Local HRCT findings of pre-biopsy examinations, such as branching, round, linear structures with or without high attenuation were compared side by side with histological findings. The attenuation of branching, round, and linear structures was classified into three-point levels on bone window images (width, 2500 HU; level, 500 HU). Furthermore, we collected continuous pulmonary CT images of 8111 cases for checking up metastasis from extrathoracic malignancy at a single institution, and evaluated the prevalence of interstitial lung abnormalities (ILAs) and DPO. RESULTS: In all 16 cases, branching (n = 15, 93%), round (n = 5, 31%), or linear (n = 5, 31%) structures were identified, histologically corresponding to dendriform ossification and cicatricial organizing pneumonia (OP)/fibrosis. Histologically, ossification was confirmed in all the 16 patients. However, in two cases, a highly attenuated structure could not be detected on the pre-biopsy CT of the same area. Regarding the prevalence of idiopathic DPO, 283 (3.5%) of 8111 patients had ILAs, of which a total of 26 (0.3% of all cases, 9.2% of ILAs cases) had DPO. CONCLUSION: Idiopathic DPO showed linear or branching structures with or without high attenuation on CT, corresponded to ossification, cicatricial OP/fibrosis. DPO was seen in 9.2% of ILAs cases. Idiopathic DPO is one of pathologic phenotypes of ILAs.
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Doenças Pulmonares Intersticiais , Ossificação Heterotópica , Fenótipo , Tomografia Computadorizada por Raios X , Humanos , Feminino , Masculino , Pessoa de Meia-Idade , Adulto , Tomografia Computadorizada por Raios X/métodos , Ossificação Heterotópica/diagnóstico por imagem , Ossificação Heterotópica/patologia , Prevalência , Doenças Pulmonares Intersticiais/diagnóstico por imagem , Doenças Pulmonares Intersticiais/patologia , Adulto Jovem , Pulmão/diagnóstico por imagem , Pulmão/patologiaRESUMO
BACKGROUND: The diagnosis of fibrotic hypersensitivity pneumonitis (fHP) from other interstitial lung diseases, particularly idiopathic pulmonary fibrosis (IPF), is often difficult. This study aimed to examine computed tomography (CT) findings that were useful for differentiating between fHP and IPF and to develop and validate a radiological diagnostic model. METHODS: In this study, 246 patients (fHP, n = 104; IPF, n = 142) from two institutions were included and randomly divided into the test (n = 164) and validation (n = 82) groups (at a 2:1 ratio). Three radiologists evaluated CT findings, such as pulmonary fibrosis, small airway disease, and predominant distribution, and compared them between fHP and IPF using binomial logistic regression and multivariate analysis. A prognostic model was developed from the test group and validated with the validation group. RESULTS: Ground-glass opacity (GGO) with traction bronchiectasis (TB), honeycombing, hypoattenuation area, three-density pattern, diffuse craniocaudal distribution, peribronchovascular opacities in the upper lung, and random distribution were more common in fHP than in IPF. In multivariate analysis, GGO with TB, peribronchovascular opacities in the upper lung, and random distribution were significant features. The area under the curve of the fHP diagnostic model with the three aforementioned CT features was 0.733 (95% confidence interval [CI], 0.655-0.811, p < 0.001) in the test group and 0.630 (95% CI, 0.504-0.755, p < 0.047) in the validation group. CONCLUSION: GGO with TB, peribronchovascular opacities in the upper lung, and random distribution were important CT features for differentiating fHP from IPF.
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Alveolite Alérgica Extrínseca , Fibrose Pulmonar Idiopática , Tomografia Computadorizada por Raios X , Humanos , Alveolite Alérgica Extrínseca/diagnóstico por imagem , Alveolite Alérgica Extrínseca/diagnóstico , Fibrose Pulmonar Idiopática/diagnóstico por imagem , Fibrose Pulmonar Idiopática/patologia , Tomografia Computadorizada por Raios X/métodos , Diagnóstico Diferencial , Masculino , Feminino , Idoso , Pessoa de Meia-IdadeRESUMO
BACKGROUND: Patients with idiopathic interstitial pneumonia (IIP) have a favourable prognosis when they have interstitial pneumonia with autoimmune features (IPAF). However, precise IPAF-related findings from high-resolution computed tomography (HRCT) and lung histopathological specimens and the treatment response have not been fully determined. Therefore, this study was conducted to evaluate the relationship between findings on HRCT or lung histopathological specimens and the progression of interstitial pneumonia in patients with IPAF. METHODS: This multicentre cohort study prospectively enrolled consecutive patients with IIP. At the diagnosis of IIP, we systematically evaluated 74 features suggestive of connective tissue diseases and followed them up. HRCT, lung specimens, serum antibodies, and the clinical course were also evaluated. RESULTS: Among 222 patients with IIP, 26 (11.7%) fulfilled the IPAF criteria. During a median observation period of 36 months, patients with IPAF showed better survival than those without IPAF (p = 0.034). While histopathological findings were not related to IPAF, nonspecific interstitial pneumonia (NSIP) with organizing pneumonia (OP) overlap was the most prevalent HRCT pattern (p < 0.001) and the consolidation opacity was the most common radiological finding in IPAF (p = 0.017). Furthermore, in patients with IPAF, the diagnosis of COP or NSIP with OP overlap was associated with a higher increase in %FVC in 1 year than in those with idiopathic pulmonary fibrosis, NSIP, or unclassifiable IIP (p = 0.002). CONCLUSIONS: This study shows the presence of consolidation opacity on HRCT and the diagnosis of COP or NSIP with OP overlap are associated with IPAF and its favourable treatment response in patients with IPAF.
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Doenças Autoimunes , Doenças do Tecido Conjuntivo , Pneumonias Intersticiais Idiopáticas , Doenças Pulmonares Intersticiais , Humanos , Estudos de Coortes , Estudos Prospectivos , Doenças Autoimunes/complicações , Doenças Autoimunes/diagnóstico por imagem , Estudos Retrospectivos , Doenças Pulmonares Intersticiais/diagnóstico , Pneumonias Intersticiais Idiopáticas/diagnóstico , Doenças do Tecido Conjuntivo/complicações , Doenças do Tecido Conjuntivo/diagnóstico por imagemRESUMO
BACKGROUND AND OBJECTIVE: Despite significant recent progress in the understanding of idiopathic pulmonary fibrosis (IPF), the early phase of the disease is still poorly understood. We studied patients with IPF without pulmonary function impairment in order to determine the clinical features, natural history and key findings for physiological progression. METHODS: Twenty-five patients without pulmonary function impairment were identified from among patients with previously diagnosed IPF who underwent surgical lung biopsies between January 1997 and December 2006 at our institutions. 'Without pulmonary function impairment' was defined as both forced vital capacity (FVC) and diffusing capacity for carbon monoxide (DLco) >80% predicted. Patients diagnosed with IPF through multidisciplinary discussion based on the new IPF guidelines were the subjects of this study. RESULTS: Sixteen patients had a confirmed diagnosis of IPF. Eleven patients presented with chest X-ray abnormality found during an annual health examination. Seven patients were asymptomatic. Eleven patients showed physiological disease progression (median time; 19.9 ± 12.3 months) defined by a decline of at least 10% in FVC or at least 15% in DLco. Univariate analysis revealed that both usual interstitial pneumonia pattern and extent of honeycombing on high-resolution computed tomography (HRCT) were factors associated with disease progression (odds ratio 5.634, 95% confidence interval 1.364-23.278; odds ratio 2.371/5%, 95% confidence interval 1.042-5.395). CONCLUSIONS: IPF patients without pulmonary function impairment have a progressive but slow clinical course. The existence and extent of honeycombing on HRCT are predictive of disease progression.