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3.
J Comput Assist Tomogr ; 44(5): 667-672, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32842066

RESUMO

OBJECTIVE: The aim of the study was to compare the prevalence of diffuse pulmonary ossification (DPO) in idiopathic pulmonary fibrosis (IPF), systemic sclerosis (SSc)-related interstitial lung disease (ILD), and chronic hypersensitivity pneumonitis (HP). METHODS: High-resolution computed tomography (HRCT) from 71 IPF, 67 SSc-ILD, and 75 HP cases were independently evaluated by 2 thoracic radiologists blinded to patient data. Studies were assessed for the presence of DPO, HRCT scanning pattern, stigmata of granulomatous infection, and honeycombing. RESULTS: The prevalence of DPO was significantly higher in cases of IPF and SSc compared with HP, although there was no significant difference in prevalence between the IPF and SSc groups, even when accounting for the presence of prior granulomatous infection. Interobserver agreement for the presence of DPO was substantial. CONCLUSIONS: Although prevalence DPO on HRCT varies between some forms of ILD, the use of DPO to influence characterization of ILD should be considered with caution.


Assuntos
Fibrose Pulmonar Idiopática/diagnóstico por imagem , Doenças Pulmonares Intersticiais/diagnóstico por imagem , Ossificação Heterotópica/diagnóstico por imagem , Tomografia Computadorizada por Raios X/métodos , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Doença Crônica , Feminino , Humanos , Fibrose Pulmonar Idiopática/patologia , Pulmão/diagnóstico por imagem , Pulmão/patologia , Doenças Pulmonares Intersticiais/etiologia , Doenças Pulmonares Intersticiais/patologia , Masculino , Pessoa de Meia-Idade , Ossificação Heterotópica/patologia , Escleroderma Sistêmico/complicações , Adulto Jovem
4.
Br J Radiol ; 93(1113): 20190121, 2020 Sep 01.
Artigo em Inglês | MEDLINE | ID: mdl-32584606

RESUMO

OBJECTIVE: To analyse delayed contrast dynamics of fibrotic lesions in interstitial lung disease (ILD) using five dimensional (5D) MRI and to correlate contrast dynamics with disease severity. METHODS: 20 patients (mean age: 71 years; M:F, 13:7), with chronic fibrosing ILD: n = 12 idiopathic pulmonary fibrosis (IPF) and n = 8 non-IPF, underwent thin-section multislice CT as part of the standard diagnostic workup and additionally MRI of the lung. 2 min after contrast injection, a radial gradient echo sequence with golden-angle spacing was acquired during 5 min of free-breathing, followed by 5D image reconstruction. Disease was categorized as severe or non-severe according to CT morphological regional severity. For each patient, 10 lesions were analysed. RESULTS: IPF lesions showed later peak enhancement compared to non-IPF (severe: p = 0.01, non-severe: p = 0.003). Severe lesions showed later peak enhancement compared to non-severe lesions, in non-IPF (p = 0.04), but not in IPF (p = 0.35). There was a tendency towards higher accumulation and washout rates in IPF compared to non-IPF in non-severe disease. Severe lesions had lower washout rate than non-severe ones in both IPF (p = 0.003) and non-IPF (p = 0.005). Continuous contrast agent accumulation, without washout, was found only in IPF lesions. CONCLUSIONS: Contrast agent dynamics are influenced by type and severity of pulmonary fibrosis, which might enable a more thorough characterisation of disease burden. The regional impairment is of particular interest in the context of antifibrotic treatments and was characterised using a non-invasive, non-irradiating, free-breathing method. ADVANCES IN KNOWLEDGE: Delayed contrast enhancement patterns allow the assessment of regional lung impairment which could represent different disease stages or phenotypes in ILD.


Assuntos
Meios de Contraste/farmacocinética , Doenças Pulmonares Intersticiais/diagnóstico por imagem , Imagem por Ressonância Magnética/métodos , Compostos Organometálicos/farmacocinética , Fibrose Pulmonar/diagnóstico por imagem , Idoso , Doenças do Tecido Conjuntivo , Feminino , Humanos , Pneumonias Intersticiais Idiopáticas/diagnóstico por imagem , Pneumonias Intersticiais Idiopáticas/metabolismo , Pneumonias Intersticiais Idiopáticas/patologia , Fibrose Pulmonar Idiopática/diagnóstico por imagem , Fibrose Pulmonar Idiopática/metabolismo , Fibrose Pulmonar Idiopática/patologia , Processamento de Imagem Assistida por Computador/métodos , Doenças Pulmonares Intersticiais/metabolismo , Doenças Pulmonares Intersticiais/patologia , Masculino , Pessoa de Meia-Idade , Tomografia Computadorizada Multidetectores , Projetos Piloto , Análise de Componente Principal , Estudos Prospectivos , Fibrose Pulmonar/metabolismo , Fibrose Pulmonar/patologia , Respiração , Índice de Gravidade de Doença , Fatores de Tempo , Capacidade Vital
5.
Int J Cardiovasc Imaging ; 36(9): 1711-1723, 2020 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-32448985

RESUMO

No data are actually available regarding the left atrial (LA) functional assessment by two-dimensional speckle tracking echocardiography (2D-STE) in early-stage idiopathic pulmonary fibrosis (IPF). The primary end-point of our study was to assess whether global LA peak strain (GLAPS), measured by 2D-STE analysis, may detect early alterations in LA function in IPF patients without right heart failure (RHF). Between September 2017 and January 2019, 50 consecutive IPF patients (73.8 ± 6.8 years, 36 males) without chronic RHF and 30 controls matched by age, sex and cardiovascular risk factors, were enrolled in an observational retrospective case-control study. All patients underwent a complete echocardiographic study implemented with 2D-STE analysis. GLAPS, left ventricular (LV) global longitudinal strain (GLS), right atrial (RA) reservoir strain (GSA+) and right ventricular (RV)-GLS were obtained in each patient. LVFP were significantly increased in IPF patients in comparison to controls (average E/e' ratio 14.4 ± 3.0 vs 9.6 ± 1.5, p < 0.0001), while LV-GLS was slightly reduced in IPF patients compared to controls (19.4 ± 3.6% vs 21.0 ± 2.2%, p = 0.03).Moreover, GLAPS was significantly impaired in IPF patients in comparison to controls (18.4 ± 3.7% vs 28.4 ± 5.6%, p < 0.0001).Finally, the two groups of patients did not show any statistically significant difference in both RA-GSA + (23.9 ± 3.7% vs 24.5 ± 4.0%, p = 0.49) and RV-GLS (- 22.6 ± 3.3% vs - 23.5 ± 3.0%, p = 0.22). Notably, LV-GLS was strongly inversely correlated both with RV/LV basal diameter ratio and TRV in IPF patients (r = - 0.87 and - 0.82, respectively) but not in controls (r = - 0.29 and - 0.27, respectively). This finding highlights a likely process of ventricular interdependence in non-advanced IPF, with consequent LV diastolic dysfunction and secondary impairment in LV-GLS and GLAPS. Early LA reservoir dysfunction in IPF patients may be secondary to LV diastolic dysfunction induced by ventricular interdependence and may develop before RV diastolic and systolic dysfunction.


Assuntos
Função do Átrio Esquerdo , Remodelamento Atrial , Ecocardiografia Doppler de Pulso , Fibrose Pulmonar Idiopática/diagnóstico por imagem , Disfunção Ventricular Esquerda/diagnóstico por imagem , Função Ventricular Esquerda , Idoso , Idoso de 80 Anos ou mais , Progressão da Doença , Diagnóstico Precoce , Feminino , Humanos , Fibrose Pulmonar Idiopática/complicações , Fibrose Pulmonar Idiopática/fisiopatologia , Masculino , Pessoa de Meia-Idade , Variações Dependentes do Observador , Valor Preditivo dos Testes , Reprodutibilidade dos Testes , Estudos Retrospectivos , Fatores de Tempo , Disfunção Ventricular Esquerda/etiologia , Disfunção Ventricular Esquerda/fisiopatologia , Função Ventricular Direita
6.
Radiologe ; 60(6): 549-562, 2020 Jun.
Artigo em Alemão | MEDLINE | ID: mdl-32342119

RESUMO

Idiopathic pulmonary fibrosis (IPF) is a specific form of chronic progressive fibrosing interstitial lung disease with a poor prognosis. High-resolution computed tomography (HRCT) plays an important role in the work-up of patients with suspected IPF. In HRCT IPF is characterized by the pattern of usual interstitial pneumonia (UIP). For a long time only supportive or immunosuppressive treatment was possible. The approval of antifibrotic agents in 2012 marked a turning point and triggered further clinical and scientific interest. Based on the recently gained knowledge the revised version of the international guidelines for the diagnosis of IPF was published in 2018, including instructions for HRCT interpretation. In this continued medical education article the relevant signs in HRCT are presented. The specifications given in the guidelines are elucidated.


Assuntos
Fibrose Pulmonar Idiopática , Humanos , Fibrose Pulmonar Idiopática/diagnóstico por imagem , Pulmão , Tomografia Computadorizada por Raios X
7.
Thorax ; 75(8): 648-654, 2020 08.
Artigo em Inglês | MEDLINE | ID: mdl-32345689

RESUMO

AIMS: Patients with idiopathic pulmonary fibrosis (IPF) receiving antifibrotic medication and patients with non-IPF fibrosing lung disease often demonstrate rates of annualised forced vital capacity (FVC) decline within the range of measurement variation (5.0%-9.9%). We examined whether change in visual CT variables could help confirm whether marginal FVC declines represented genuine clinical deterioration rather than measurement noise. METHODS: In two IPF cohorts (cohort 1: n=103, cohort 2: n=108), separate pairs of radiologists scored paired volumetric CTs (acquired between 6 and 24 months from baseline). Change in interstitial lung disease, honeycombing, reticulation, ground-glass opacity extents and traction bronchiectasis severity was evaluated using a 5-point scale, with mortality prediction analysed using univariable and multivariable Cox regression analyses. Both IPF populations were then combined to determine whether change in CT variables could predict mortality in patients with marginal FVC declines. RESULTS: On univariate analysis, change in all CT variables except ground-glass opacity predicted mortality in both cohorts. On multivariate analysis adjusted for patient age, gender, antifibrotic use and baseline disease severity (diffusing capacity for carbon monoxide), change in traction bronchiectasis severity predicted mortality independent of FVC decline. Change in traction bronchiectasis severity demonstrated good interobserver agreement among both scorer pairs. Across all study patients with marginal FVC declines, change in traction bronchiectasis severity independently predicted mortality and identified more patients with deterioration than change in honeycombing extent. CONCLUSIONS: Change in traction bronchiectasis severity is a measure of disease progression that could be used to help resolve the clinical importance of marginal FVC declines.


Assuntos
Fibrose Pulmonar Idiopática/diagnóstico por imagem , Fibrose Pulmonar Idiopática/fisiopatologia , Capacidade Vital/fisiologia , Idoso , Estudos de Coortes , Progressão da Doença , Feminino , Humanos , Fibrose Pulmonar Idiopática/terapia , Masculino , Pessoa de Meia-Idade , Índice de Gravidade de Doença , Fatores de Tempo , Tomografia Computadorizada por Raios X
8.
Respir Res ; 21(1): 75, 2020 Mar 26.
Artigo em Inglês | MEDLINE | ID: mdl-32216814

RESUMO

BACKGROUND: Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive lung disease with poor prognosis and a significant unmet medical need. This study evaluated the safety, pharmacokinetics (PK) and target engagement in the lungs, of GSK3008348, a novel inhaled alpha-v beta-6 (αvß6) integrin inhibitor, in participants with IPF. METHODS: This was a phase 1b, randomised, double-blind (sponsor unblind) study, conducted in the UK (two clinical sites, one imaging unit) between June 2017 and July 2018 (NCT03069989). Participants with a definite or probable diagnosis of IPF received a single nebulised dose of 1000 mcg GSK3008348 or placebo (ratio 5:2) in two dosing periods. In period 1, safety and PK assessments were performed up to 24 h post-dose; in period 2, after a 7-day to 28-day washout, participants underwent a total of three positron emission tomography (PET) scans: baseline, Day 1 (~ 30 min post-dosing) and Day 2 (~ 24 h post-dosing), using a radiolabelled αvß6-specific ligand, [18F]FB-A20FMDV2. The primary endpoint was whole lung volume of distribution (VT), not corrected for air volume, at ~ 30 min post-dose compared with pre-dose. The study success criterion, determined using Bayesian analysis, was a posterior probability (true % reduction in VT > 0%) of ≥80%. RESULTS: Eight participants with IPF were enrolled and seven completed the study. Adjusted posterior median reduction in uncorrected VT at ~ 30 min after GSK3008348 inhalation was 20% (95% CrI: - 9 to 42%). The posterior probability that the true % reduction in VT > 0% was 93%. GSK3008348 was well tolerated with no reports of serious adverse events or clinically significant abnormalities that were attributable to study treatment. PK was successfully characterised showing rapid absorption followed by a multiphasic elimination. CONCLUSIONS: This study demonstrated engagement of the αvß6 integrin target in the lung following nebulised dosing with GSK3008348 to participants with IPF. To the best of our knowledge this is the first time a target-specific PET radioligand has been used to assess target engagement in the lung, not least for an inhaled drug. TRIAL REGISTRATION: clinicaltrials.gov: NCT03069989; date of registration: 3 March 2017.


Assuntos
Butiratos/uso terapêutico , Fibrose Pulmonar Idiopática/tratamento farmacológico , Integrinas/antagonistas & inibidores , Naftiridinas/uso terapêutico , Pirazóis/uso terapêutico , Pirrolidinas/uso terapêutico , Volume de Ventilação Pulmonar/efeitos dos fármacos , Administração por Inalação , Idoso , Antígenos de Neoplasias , Teorema de Bayes , Butiratos/administração & dosagem , Butiratos/farmacocinética , Método Duplo-Cego , Determinação de Ponto Final , Feminino , Humanos , Fibrose Pulmonar Idiopática/diagnóstico por imagem , Masculino , Naftiridinas/administração & dosagem , Naftiridinas/farmacocinética , Nebulizadores e Vaporizadores , Tomografia por Emissão de Pósitrons , Pirazóis/administração & dosagem , Pirazóis/farmacocinética , Pirrolidinas/administração & dosagem , Pirrolidinas/farmacocinética , Resultado do Tratamento
9.
J Bras Pneumol ; 46(5): e20190153, 2020.
Artigo em Português, Inglês | MEDLINE | ID: mdl-32159601

RESUMO

OBJECTIVE: To correlate the prevalence and prognosis of each HRCT pattern of typical, probable, and indeterminate usual interstitial pneumonia (UIP) with the clinical multidisciplinary diagnosis of interstitial lung disease (ILD). METHODS: We included all patients with a multidisciplinary diagnosis of ILD with an HRCT pattern of typical UIP, probable UIP, or indeterminate for UIP. Clinical and histopathological data, pulmonary function tests, and survival status were retrospectively obtained. The final diagnosis was validated by a multidisciplinary team. RESULTS: A total of 244 patients were included in the study, with a mean age of 68 ±13 years and being 52.5% males. In a total of 106 patients with typical UIP pattern, 62% had the multidisciplinary diagnosis of IPF, 20% had chronic hypersensitivity pneumonitis (CHP), and 10% had connective tissue disease-related ILD (CTD-ILD). Out of the 114 cases with probable UIP, CTD-ILD corresponded to 39%, IPF to 31%, desquamative interstitial pneumonia to 11%, drug-related lung disease to 9%, and CHP to 8%. In the 24 patients with CT indeterminate for UIP, CTD-ILD was the final diagnosis in 33%, followed by desquamative interstitial pneumonia (21%), and IPF (13%). Patients with typical UIP were more likely to die or had lung transplantation in the follow-up (17.9% and 11.3%, respectively). CONCLUSION: IPF, CHP, and CTD-ILD were the main differential diagnoses in patients with HRCT patterns of typical, probable and indeterminate UIP. Patients with HRCT typical UIP pattern were more likely to die or had lung transplantation in the follow-up.


Assuntos
Fibrose Pulmonar Idiopática/diagnóstico por imagem , Doenças Pulmonares Intersticiais/diagnóstico por imagem , Pulmão/diagnóstico por imagem , Tomografia Computadorizada por Raios X/métodos , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Fibrose Pulmonar Idiopática/epidemiologia , Doenças Pulmonares Intersticiais/epidemiologia , Masculino , Pessoa de Meia-Idade , Prevalência , Prognóstico , Estudos Retrospectivos
10.
Lancet Respir Med ; 8(6): 573-584, 2020 06.
Artigo em Inglês | MEDLINE | ID: mdl-32061334

RESUMO

BACKGROUND: The observation that patients with idiopathic pulmonary fibrosis (IPF) can have higher than normal expiratory flow rates at low lung volumes led to the conclusion that the airways are spared in IPF. This study aimed to re-examine the hypothesis that airways are spared in IPF using a multiresolution imaging protocol that combines multidetector CT (MDCT), with micro-CT and histology. METHODS: This was a retrospective cohort study comparing explanted lungs from patients with severe IPF treated by lung transplantation with a cohort of unused donor (control) lungs. The donor control lungs had no known lung disease, comorbidities, or structural lung injury, and were deemed appropriate for transplantation on review of the clinical files. The diagnosis of IPF in the lungs from patients was established by a multidisciplinary consensus committee according to existing guidelines, and was confirmed by video-assisted thoracic surgical biopsy or by pathological examination of the contralateral lung. The control and IPF groups were matched for age, sex, height, and bodyweight. Samples of lung tissue were compared using the multiresolution imaging approach: a cascade of clinical MDCT, micro-CT, and histological imaging. We did two experiments: in experiment 1, all the lungs were randomly sampled; in experiment 2, samples were selected from regions of minimal and established fibrosis. The patients and donors were recruited from the Katholieke Universiteit Leuven (Leuven, Belgium) and the University of Pennsylvania Hospital (Philadelphia, PA, USA). The study took place at the Katholieke Universiteit Leuven, and the University of British Columbia (Vancouver, BC, Canada). FINDINGS: Between Oct 5, 2009, and July 22, 2016, explanted lungs from patients with severe IPF (n=11), were compared with a cohort of unused donor (control) lungs (n=10), providing 240 samples of lung tissue for comparison using the multiresolution imaging approach. The MDCT specimen scans show that the number of visible airways located between the ninth generation (control 69 [SD 22] versus patients with IPF 105 [33], p=0·0023) and 14th generation (control 9 [6] versus patients with IPF 49 [28], p<0·0001) of airway branching are increased in patients with IPF, which we show by micro-CT is due to thickening of their walls and distortion of their lumens. The micro-CT analysis showed that compared with healthy (control) lung anatomy (mean 5·6 terminal bronchioles per mL [SD 1·6]), minimal fibrosis in IPF tissue was associated with a 57% loss of the terminal bronchioles (mean 2·4 terminal bronchioles per mL [SD 1·0]; p<0·0001), the appearance of fibroblastic foci, and infiltration of the tissue by inflammatory immune cells capable of forming lymphoid follicles. Established fibrosis in IPF tissue had a similar reduction (66%) in the number of terminal bronchioles (mean 1·9 terminal bronchioles per mL [SD 1·4]; p<0·0001) and was dominated by increased airspace size, Ashcroft fibrosis score, and volume fractions of tissue and collagen. INTERPRETATION: Small airways disease is a feature of IPF, with significant loss of terminal bronchioles occuring within regions of minimal fibrosis. On the basis of these findings, we postulate that the small airways could become a potential therapeutic target in IPF. FUNDING: Katholieke Universiteit Leuven, US National Institutes of Health, BC Lung Association, and Genentech.


Assuntos
Fibrose Pulmonar Idiopática/patologia , Bronquíolos/diagnóstico por imagem , Bronquíolos/patologia , Feminino , Humanos , Fibrose Pulmonar Idiopática/diagnóstico por imagem , Fibrose Pulmonar Idiopática/cirurgia , Pulmão/diagnóstico por imagem , Pulmão/patologia , Transplante de Pulmão , Masculino , Pessoa de Meia-Idade , Tomografia Computadorizada Multidetectores , Imagem Multimodal , Estudos Retrospectivos , Microtomografia por Raio-X
11.
Respir Investig ; 58(3): 185-189, 2020 May.
Artigo em Inglês | MEDLINE | ID: mdl-32102769

RESUMO

BACKGROUND: Pirfenidone suppresses the decline of forced vital capacity (FVC) in patients with idiopathic pulmonary fibrosis (IPF). However, IPF progresses in some patients despite treatment. We analyzed patients with meaningful FVC declines during pirfenidone treatment and explored the factors predictive of disease progression after FVC decline. METHODS: This study was a retrospective, multicenter, observational study conducted by the Okayama Respiratory Disease Study Group. We defined initial decline in %FVC as 5% or greater per 6-month period during pirfenidone treatment. IPF patients who were treated with pirfenidone and experienced an initial decline from December 2008 to September 2017 were enrolled. RESULTS: We analyzed 21 patients with IPF. After the initial decline, 4 (19.0%) patients showed improvement in disease, 11 (52.4%) showed stable disease, and 6 (28.6%) showed progressive disease. There was no significant correlation between %FVC reduction on initial decline and subsequent %FVC change (p = 0.475). Deterioration of high-resolution computed tomography (HRCT) findings on initial decline was observed significantly more often in the progressive versus improved/stable disease groups (100% vs 20.0%, p = 0.009). CONCLUSIONS: We revealed that deterioration of HRCT findings may predict disease progression after the initial decline in %FVC in IPF patients treated with pirfenidone.


Assuntos
Progressão da Doença , Fibrose Pulmonar Idiopática/diagnóstico por imagem , Fibrose Pulmonar Idiopática/tratamento farmacológico , Piridonas/uso terapêutico , Intensificação de Imagem Radiográfica/métodos , Tomografia Computadorizada por Raios X/métodos , Capacidade Vital , Humanos , Fibrose Pulmonar Idiopática/fisiopatologia , Valor Preditivo dos Testes , Estudos Retrospectivos
12.
Jpn J Radiol ; 38(6): 524-532, 2020 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-32103467

RESUMO

PURPOSE: Radiologic diagnosis of chronic hypersensitivity pneumonitis (CHP) presenting a usual interstitial pneumonia (UIP) pattern is challenging. The aim of this study was to identify the high-resolution CT (HRCT) findings which are useful to discriminate CHP-UIP from idiopathic pulmonary fibrosis (IPF). MATERIALS AND METHODS: This study included 49 patients with well-established bird-related CHP-UIP, histologically confirmed, and 49 patients with IPF. Two groups of observers independently assessed HRCT, evaluated the extent of each abnormal HRCT finding. When their radiological diagnosis was CHP-UIP, they noted the HRCT findings inconsistent with IPF. RESULTS: Correct CT diagnoses were made in 79% of CHP-UIP and 53% of IPF. Although no apparent difference was seen in the extent of each HRCT finding, upper or mid-lung predominance, extensive ground-glass abnormality, and profuse micronodules were more frequently pointed out as inconsistent findings in CHP-UIP than IPF (p = 0.007, 0.010, 0.001, respectively). On regression analysis, profuse micronodules [OR 13.34 (2.85-62.37); p = 0.001] and upper or mid-lung predominance of findings [OR 2.86 (1.16-7.01); p = 0.022] remained as variables in the equation. CONCLUSION: In this cohort, some IPF cases were misdiagnosed as CHP-UIP. Profuse micronodules and upper or mid-lung predominance are important clues for the differentiation of CHP-UIP from IPF.


Assuntos
Alveolite Alérgica Extrínseca/diagnóstico por imagem , Fibrose Pulmonar Idiopática/diagnóstico por imagem , Doenças Pulmonares Intersticiais/diagnóstico por imagem , Tomografia Computadorizada por Raios X/métodos , Adulto , Idoso , Alveolite Alérgica Extrínseca/complicações , Diagnóstico Diferencial , Feminino , Humanos , Fibrose Pulmonar Idiopática/complicações , Pulmão/diagnóstico por imagem , Masculino , Pessoa de Meia-Idade
13.
Eur J Radiol ; 124: 108852, 2020 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-32028067

RESUMO

PURPOSE: To investigate the role of a quantitative analysis software (CALIPER) in identifying HRCT thresholds predicting IPF patients' survival and lung function decline and its role in detecting changes of HRCT abnormalities related to treatment and their correlation with Forced Vital Capacity (FVC). METHODS: This retrospective study included 105 patients with a multidisciplinary diagnosis of IPF for whom one HRCT at baseline and concomitant FVC were available. HRCTs were evaluated with CALIPER and the correlation between FVC and radiological features were assessed. Radiological thresholds for survival prediction and functional decline were calculated for all patients. Fifty-nine patients with at least 2 serial HRCTs were classified into two groups based on treatment. For patients for whom a FVC within 3 months of the HRCT was available (n = 44), the correlation of radiological and clinical progression was evaluated. RESULTS: The correlation between FVC and CALIPER-derived features at baseline was significant and strong. A baseline CALIPER-derived interstitial lung disease (ILD%) extent higher than 20 % and pulmonary vascular related structures (PVRS%) score greater than 5 % defined a worse prognosis. A significant progression of CALIPER-derived features in all patients was found with a faster increase in untreated patients. ILD% and PVRS% changes during follow-up demonstrated strong correlations with FVC changes. CONCLUSIONS: CALIPER quantification of fibrosis and vascular involvement could distinguish disease progression in treated versus untreated patients and predict the survival. The changes in CALIPER-derived variables over time were significantly correlated to changes in FVC.


Assuntos
Fibrose Pulmonar Idiopática/diagnóstico por imagem , Interpretação de Imagem Assistida por Computador/métodos , Tomografia Computadorizada por Raios X/métodos , Idoso , Progressão da Doença , Feminino , Humanos , Pulmão/diagnóstico por imagem , Masculino , Reprodutibilidade dos Testes , Estudos Retrospectivos , Índice de Gravidade de Doença , Capacidade Vital
14.
Eur Radiol ; 30(5): 2669-2679, 2020 May.
Artigo em Inglês | MEDLINE | ID: mdl-32006172

RESUMO

OBJECTIVES: To test HRCT with either visual or quantitative analysis in both short-term and long-term follow-up of stable IPF against long-term (transplant-free) survival, beyond 2 years of disease stability. METHODS: Fifty-eight IPF patients had FVC measurements and HRCTs at baseline (HRCT0), 10-14 months (HRCT1) and 22-26 months (HRCT2). Visual scoring, CALIPER quantitative analysis of HRCT measures, and their deltas were evaluated against combined all-cause mortality and lung transplantation by adjusted Cox proportional hazard models at each time interval. RESULTS: At HRCT1, a ≥ 20% relative increase in CALIPER-total lung fibrosis yielded the highest radiological association with outcome (C-statistic 0.62). Moreover, the model combining FVC% drop ≥ 10% and ≥ 20% relative increase of CALIPER-total lung fibrosis improved the stratification of outcome (C-statistic 0.69, high-risk category HR 12.1; landmark analysis at HRCT1 C-statistic 0.66, HR 14.9 and at HRCT2 C-statistic 0.61, HR 21.8). Likewise, at HRCT2, the model combining FVC% decrease trend and ≥ 20% relative increase of CALIPER-pulmonary vessel-related volume (VRS) improved the stratification of outcome (C-statistic 0.65, HR 11.0; landmark analysis at HRCT1 C-statistic 0.62, HR 13.8 and at HRCT2 C-statistic 0.58, HR 12.6). A less robust stratification of outcome distinction was also demonstrated with the categorical visual scoring of disease change. CONCLUSIONS: Annual combined CALIPER -FVC changes showed the greatest stratification of long-term outcome in stable IPF patients, beyond 2 years. KEY POINTS: • Longitudinal high-resolution computed tomography (HRCT) data is more helpful than baseline HRCT alone for stratification of long-term outcome in IPF. • HRCT changes by visual or quantitative analysis can be added with benefit to the current spirometric reference standard to improve stratification of long-term outcome in IPF. • HRCT follow-up at 12-14 months is more helpful than HRCT follow-up at 23-26 months in clinically stable subjects with IPF.


Assuntos
Fibrose Pulmonar Idiopática/diagnóstico por imagem , Fibrose Pulmonar Idiopática/fisiopatologia , Idoso , Feminino , Humanos , Fibrose Pulmonar Idiopática/cirurgia , Pulmão , Transplante de Pulmão/estatística & dados numéricos , Masculino , Pessoa de Meia-Idade , Prognóstico , Modelos de Riscos Proporcionais , Estudos Retrospectivos , Tomografia Computadorizada por Raios X/métodos , Capacidade Vital
15.
PLoS One ; 15(1): e0226084, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-31929532

RESUMO

PURPOSE: To conduct a meta-analysis to determine specific computed tomography (CT) patterns and clinical features that discriminate between nonspecific interstitial pneumonia (NSIP) and usual interstitial pneumonia (UIP). MATERIALS AND METHODS: The PubMed/Medline and Embase databases were searched for studies describing the radiological patterns of UIP and NSIP in chest CT images. Only studies involving histologically confirmed diagnoses and a consensus diagnosis by an interstitial lung disease (ILD) board were included in this analysis. The radiological patterns and patient demographics were extracted from suitable articles. We used random-effects meta-analysis by DerSimonian & Laird and calculated pooled odds ratios for binary data and pooled mean differences for continuous data. RESULTS: Of the 794 search results, 33 articles describing 2,318 patients met the inclusion criteria. Twelve of these studies included both NSIP (338 patients) and UIP (447 patients). NSIP-patients were significantly younger (NSIP: median age 54.8 years, UIP: 59.7 years; mean difference (MD) -4.4; p = 0.001; 95% CI: -6.97 to -1.77), less often male (NSIP: median 52.8%, UIP: 73.6%; pooled odds ratio (OR) 0.32; p<0.001; 95% CI: 0.17 to 0.60), and less often smokers (NSIP: median 55.1%, UIP: 73.9%; OR 0.42; p = 0.005; 95% CI: 0.23 to 0.77) than patients with UIP. The CT findings from patients with NSIP revealed significantly lower levels of the honeycombing pattern (NSIP: median 28.9%, UIP: 73.4%; OR 0.07; p<0.001; 95% CI: 0.02 to 0.30) with less peripheral predominance (NSIP: median 41.8%, UIP: 83.3%; OR 0.21; p<0.001; 95% CI: 0.11 to 0.38) and more subpleural sparing (NSIP: median 40.7%, UIP: 4.3%; OR 16.3; p = 0.005; 95% CI: 2.28 to 117). CONCLUSION: Honeycombing with a peripheral predominance was significantly associated with a diagnosis of UIP. The NSIP pattern showed more subpleural sparing. The UIP pattern was predominantly observed in elderly males with a history of smoking, whereas NSIP occurred in a younger patient population.


Assuntos
Fibrose Pulmonar Idiopática/patologia , Doenças Pulmonares Intersticiais/patologia , Fatores Etários , Humanos , Fibrose Pulmonar Idiopática/diagnóstico por imagem , Fibrose Pulmonar Idiopática/epidemiologia , Pulmão/diagnóstico por imagem , Pulmão/fisiologia , Doenças Pulmonares Intersticiais/diagnóstico por imagem , Doenças Pulmonares Intersticiais/epidemiologia , Prevalência , Fatores Sexuais , Fumar , Tomografia Computadorizada por Raios X
16.
PLoS One ; 15(1): e0228390, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-31999801

RESUMO

BACKGROUND: The safety of pirfenidone on pulmonary fibrosis patients with other kinds of interstitial lung diseases (ILDs) in addition to idiopathic pulmonary fibrosis (IPF) is unknown. Furthermore, its effectiveness-related factors on IPF patients are not quite explored. METHODS: A retrospective study, on patients prescribed pirfenidone for pulmonary fibrosis, was conducted to assess effectiveness on IPF patients and tolerability of all patients with lung fibrosis. The effectiveness of pirfenidone was tested on 110 IPF subjects receiving treatment for ≥3 months by high-resolution computed tomography (HRCT). Response-linked factors and progression-free survival (PFS) were also analyzed. The data about safety outcomes and drug dose adjustments were collected from all included subjects. RESULTS: A total of 176 subjects were included: 117 were IPF, 19 connective tissue disease-associated interstitial lung disease (CTD-ILD), and 40 unclassifiable ILD. Out of the 110 IPF subjects, 89 subjects were assessed as stable and 21 as progressive, out of which 10 died of acute exacerbation and 11 progressed. The effectiveness was significantly related to their baseline body mass index (BMI). IPF subjects with BMI>25kg/m2 or diffusion capacity of carbon monoxide (DLco)>30% had higher PFS rate. The most common adverse events were skin-related and gastrointestinal-related. Drug discontinuation owing to adverse events occurred similarly in these three groups. CONCLUSION: Pirfenidone was well tolerated in most of the lung fibrosis patients besides IPF, with a similar pattern of adverse events. Nearly 80% of IPF subjects were assessed as stable. More benefits were seen in IPF patients with higher BMI or mild-to-moderate disease.


Assuntos
Anti-Inflamatórios não Esteroides/administração & dosagem , Fibrose Pulmonar Idiopática/tratamento farmacológico , Piridonas/administração & dosagem , Idoso , Anti-Inflamatórios não Esteroides/efeitos adversos , Índice de Massa Corporal , Monóxido de Carbono/química , China , Feminino , Humanos , Fibrose Pulmonar Idiopática/classificação , Fibrose Pulmonar Idiopática/diagnóstico por imagem , Masculino , Pessoa de Meia-Idade , Piridonas/efeitos adversos , Estudos Retrospectivos , Tomografia Computadorizada por Raios X , Resultado do Tratamento , Capacidade Vital
17.
J Thorac Imaging ; 35(2): 115-122, 2020 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-31913257

RESUMO

PURPOSE: The purpose of this study was to investigate histogram-based quantitative high-resolution computed tomography (HRCT) indexes in the assessment of lung cancer (LC) development in idiopathic pulmonary fibrosis (IPF) patients. MATERIALS AND METHODS: From IPF databases of 2 national respiratory centers, we retrospectively studied patients with and without LC development-respectively, divided into Group A (n=16) and Group B (n=33). The extent of fibrotic disease was quantified on baseline and follow-up HRCT examinations using kurtosis, skewness, percentage of high attenuation area (HAA%), and percentage of fibrotic area (FA%). These indexes were compared between the 2 groups using the Mann-Whitney U test. In the prediction of LC development, receiver operating characteristic analysis was performed to assess threshold values of HRCT indexes. RESULTS: At baseline, no difference was reported among groups for kurtosis, skewness, HAA%, and FA%, with P-values of 0.0881, 0.0606, 0.0578, and 0.0606, respectively. On follow-up, significant differences were reported, with P-values of 0.0174 for kurtosis, 0.0313 for skewness, 0.0297 for HAA%, and 0.0407 for FA%.On baseline HRCT, in the prediction of LC development, receiver operating characteristic analysis reported sensibility and specificity of 87.5% and 45.45% for kurtosis, 68.75% and 63.64% for skewness, 81.25% and 54.55% for FA%, and 75% and 60.61% for HAA%. CONCLUSIONS: LC development is associated with progression of fibrosis; at baseline, FA% and HAA% reported more convenient sensitivity/specificity ratios in the prediction of LC development.


Assuntos
Fibrose Pulmonar Idiopática/complicações , Fibrose Pulmonar Idiopática/diagnóstico por imagem , Neoplasias Pulmonares/complicações , Neoplasias Pulmonares/diagnóstico por imagem , Tomografia Computadorizada por Raios X/métodos , Idoso , Idoso de 80 Anos ou mais , Progressão da Doença , Feminino , Humanos , Pulmão/diagnóstico por imagem , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Sensibilidade e Especificidade
18.
Am J Physiol Lung Cell Mol Physiol ; 318(2): L276-L286, 2020 02 01.
Artigo em Inglês | MEDLINE | ID: mdl-31774302

RESUMO

Idiopathic pulmonary fibrosis (IPF) is characterized by a profound remodeling of the collagen in the extracellular matrix (ECM), where the fibers become both denser and more highly aligned. However, it is unknown how this reconfiguration of the collagen matrix affects disease progression. Here, we investigate the role of specific alterations in collagen fiber organization on cell migration dynamics by using biomimetic image-based collagen scaffolds representing normal and fibrotic lung, where the designs are derived directly from high-resolution second harmonic generation microscopy images. The scaffolds are fabricated by multiphoton-excited (MPE) polymerization, where the process is akin to three-dimensional printing, except that it is performed at much greater resolution (∼0.5 microns) and with collagen and collagen analogs. These scaffolds were seeded with early passaged primary human normal and IPF fibroblasts to enable the decoupling of the effect of cell-intrinsic characteristics (normal vs. IPF) versus ECM structure (normal vs. IPF) on migration dynamics. We found that the highly aligned IPF collagen structure promoted enhanced cell elongation and F-actin alignment along with increased cell migration speed and straightness relative to the normal tissues. Collectively, the data are consistent with an enhanced contact guidance mechanism on the aligned IPF matrix. Although cell intrinsic effects were observed, the aligned collagen matrix morphology had a larger effect on these metrics. Importantly, these biomimetic models of the lung cannot be synthesized by conventional fabrication methods. We suggest that the MPE image-based fabrication method will enable additional hypothesis-based testing studies of cell-matrix interactions in the context of tissue fibrosis.


Assuntos
Movimento Celular , Matriz Extracelular/metabolismo , Fibroblastos/patologia , Fibrose Pulmonar Idiopática/diagnóstico por imagem , Fibrose Pulmonar Idiopática/patologia , Processamento de Imagem Assistida por Computador , Tecidos Suporte/química , Citoesqueleto de Actina/efeitos dos fármacos , Citoesqueleto de Actina/metabolismo , Actinas/metabolismo , Animais , Movimento Celular/efeitos dos fármacos , Forma Celular/efeitos dos fármacos , Colágeno/farmacologia , Matriz Extracelular/efeitos dos fármacos , Humanos , Fótons , Polimerização , Ratos , Fibras de Estresse/efeitos dos fármacos , Fibras de Estresse/metabolismo
19.
J Thorac Cardiovasc Surg ; 159(3): 1111-1118.e2, 2020 03.
Artigo em Inglês | MEDLINE | ID: mdl-31587890

RESUMO

BACKGROUND: Idiopathic pulmonary fibrosis is defined as a specific form of progressive fibrosing interstitial pneumonia. Postoperative acute exacerbation is considered a lethal comorbidity for patients with lung cancer, particularly when it is accompanied with idiopathic pulmonary fibrosis. Thus, pretherapeutic risk stratification for acute exacerbation has been anticipated. In this study, we aimed to investigate whether the maximum standardized uptake value of F-18 fluorodeoxyglucose is useful for assessing the postoperative risk of acute exacerbation and severe respiratory adverse events in patients with lung cancer after surgical resection. METHODS: A total of 822 patients with lung cancer who underwent preoperative high-resolution computed tomography, fluorodeoxyglucose-positron emission tomography/computed tomography, and pulmonary resection between July 2012 and July 2018 were assessed. Maximum standardized uptake value of the main tumor and that of the noncancerous lung area were measured using a 3-dimensional workstation. Multivariable analyses for acute exacerbation and severe respiratory adverse events were performed using the logistic regression model. RESULTS: Among all patients, 120 (14.6%) had idiopathic pulmonary fibrosis findings on high-resolution computed tomography whereas severe respiratory adverse events were observed in 35 (4.2%) patients, including those with acute exacerbation (n = 15, 1.8%). Maximum standardized uptake value of the main tumor and that of the noncancerous lung area were independently associated with both acute exacerbation and severe respiratory adverse events on multivariable analysis, both in all patients and in the 120 patients with idiopathic pulmonary fibrosis. Risk stratification analysis showed that 19.0% and 30.2% of patients who were positive for idiopathic pulmonary fibrosis on high-resolution computed tomography and with a maximum standardized uptake value of the main tumor and that of the noncancerous lung area 1.69 or greater (the optimal cutoff value relevant to acute exacerbation) experienced acute exacerbation and severe respiratory adverse events, respectively. CONCLUSIONS: Maximum standardized uptake value of the main tumor and that of the noncancerous lung area were independently associated with the incidence of postoperative acute exacerbation and severe respiratory adverse events in patients with lung cancer.


Assuntos
Fluordesoxiglucose F18/administração & dosagem , Fibrose Pulmonar Idiopática/diagnóstico por imagem , Neoplasias Pulmonares/cirurgia , Pneumonectomia/efeitos adversos , Tomografia Computadorizada com Tomografia por Emissão de Pósitrons , Compostos Radiofarmacêuticos/administração & dosagem , Adulto , Idoso , Idoso de 80 Anos ou mais , Progressão da Doença , Feminino , Humanos , Fibrose Pulmonar Idiopática/fisiopatologia , Neoplasias Pulmonares/diagnóstico por imagem , Neoplasias Pulmonares/fisiopatologia , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Reprodutibilidade dos Testes , Respiração , Medição de Risco , Fatores de Risco , Resultado do Tratamento , Adulto Jovem
20.
Respir Investig ; 58(2): 91-101, 2020 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-31882370

RESUMO

BACKGROUND: Idiopathic pulmonary fibrosis (IPF) is a progressive disease often accompanied by skeletal muscle wasting. We investigated whether skeletal muscle mass and muscle attenuation on computed tomography (CT) are predictors of mortality in IPF patients, using a nationwide cloud-based database and web-based multidisciplinary discussion (MDD) system. METHODS: IPF patients diagnosed using MDD from April 2009 to March 2014 were included. We analyzed the cross-sectional area (CSA) of the erector spinae muscle (ESMCSA) and the pectoralis muscle (PMCSA), muscle attenuation of the ESM (ESMMA), and PM (PMMA) on single-slice axial CT. Survival probability was assessed using the Kaplan-Meier method and compared by the log-rank test. Multivariate Cox proportional hazards models were used to evaluate the relationship among the ESMCSA, PMCSA, ESMMA, PMMA, clinical parameters, and prognosis. RESULTS: A total of 199 IPF patients were enrolled. Seventy-four patients died during the study period and the most frequent cause was acute exacerbation (13.1%). The group with the lowest quartile of ESMCSA had significantly worse survival than other groups (P = 0.009). Survival rates of the groups with the lowest quartile of PMCSA, lower ESMMA, and lower PMMA did not differ from those of other groups. According to multivariate analysis, ESMCSA < lower quartile was significantly associated with all-cause mortality (hazards ratio, 1.96; P = 0.030), whereas, ESMMA < median, PMCSA < lower quartile, and PMMA < median were not. CONCLUSIONS: Low ESMCSA on CT images may be a strong risk factor for all-cause mortality in IPF patients based on MDD diagnosis.


Assuntos
Fibrose Pulmonar Idiopática/diagnóstico por imagem , Fibrose Pulmonar Idiopática/patologia , Músculo Esquelético/diagnóstico por imagem , Tomografia Computadorizada por Raios X/métodos , Humanos , Comunicação Interdisciplinar , Japão , Estudos Retrospectivos
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