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Thomas V Colby - One of the best experts on this subject based on the ideXlab platform.
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Utility of a Molecular Classifier as a Complement to High-Resolution Computed Tomography to Identify Usual Interstitial Pneumonia.
American journal of respiratory and critical care medicine, 2021Co-Authors: Luca Richeldi, Jeffrey L Myers, Thomas V Colby, David A Lynch, Mary Beth Scholand, Steve D Groshong, Jonathan H. Chung, Sadia Benzaquen, Steven D. Nathan, J Russell DavisAbstract:Rationale: Usual Interstitial Pneumonia (UIP) is the defining morphology of idiopathic pulmonary fibrosis (IPF). Guidelines for IPF diagnosis conditionally recommend surgical lung biopsy for histop...
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use of a molecular classifier to identify Usual Interstitial Pneumonia in conventional transbronchial lung biopsy samples a prospective validation study
The Lancet Respiratory Medicine, 2019Co-Authors: Ganesh Raghu, Kevin R Flaherty, Jeffrey L Myers, Thomas V Colby, David A Lynch, Steve D Groshong, Jonathan H. Chung, David J Lederer, Brandon T Larsen, Mark P SteeleAbstract:Summary Background In the appropriate clinical setting, the diagnosis of idiopathic pulmonary fibrosis (IPF) requires a pattern of Usual Interstitial Pneumonia to be present on high-resolution chest CT (HRCT) or surgical lung biopsy. A molecular Usual Interstitial Pneumonia signature can be identified by a machine learning algorithm in less-invasive transbronchial lung biopsy samples. We report prospective findings for the clinical validity and utility of this molecular test. Methods We prospectively recruited 237 patients for this study from those enrolled in the Bronchial Sample Collection for a Novel Genomic Test (BRAVE) study in 29 US and European sites. Patients were undergoing evaluation for Interstitial lung disease and had had samples obtained by clinically indicated surgical or transbronchial biopsy or cryobiopsy for pathology. Histopathological diagnoses were made by experienced pathologists. Available HRCT scans were reviewed centrally. Three to five transbronchial lung biopsy samples were collected from all patients specifically for this study, pooled by patient, and extracted for transcriptomic sequencing. After exclusions, diagnostic histopathology and RNA sequence data from 90 patients were used to train a machine learning algorithm (Envisia Genomic Classifier, Veracyte, San Francisco, CA, USA) to identify a Usual Interstitial Pneumonia pattern. The primary study endpoint was validation of the classifier in 49 patients by comparison with diagnostic histopathology. To assess clinical utility, we compared the agreement and confidence level of diagnosis made by central multidisciplinary teams based on anonymised clinical information and radiology results plus either molecular classifier or histopathology results. Findings The classifier identified Usual Interstitial Pneumonia in transbronchial lung biopsy samples from 49 patients with 88% specificity (95% CI 70–98) and 70% sensitivity (47–87). Among 42 of these patients who had possible or inconsistent Usual Interstitial Pneumonia on HRCT, the classifier showed 81% positive predictive value (95% CI 54–96) for underlying biopsy-proven Usual Interstitial Pneumonia. In the clinical utility analysis, we found 86% agreement (95% CI 78–92) between clinical diagnoses using classifier results and those using histopathology data. Diagnostic confidence was improved by the molecular classifier results compared with histopathology results in 18 with IPF diagnoses (proportion of diagnoses that were confident or provisional with high confidence 89% vs 56%, p=0·0339) and in all 48 patients with non-diagnostic pathology or non-classifiable fibrosis histopathology (63% vs 42%, p=0·0412). Interpretation The molecular test provided an objective method to aid clinicians and multidisciplinary teams in ascertaining a diagnosis of IPF, particularly for patients without a clear radiological diagnosis, in samples that can be obtained by a less invasive method. Further prospective clinical validation and utility studies are planned. Funding Veracyte.
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Usual Interstitial Pneumonia can be detected in transbronchial biopsies using machine learning
Annals of the American Thoracic Society, 2017Co-Authors: Daniel Pankratz, Kevin K Brown, Jeffrey L Myers, Thomas V Colby, David A Lynch, Jessica D Anderson, Yoonha Choi, Urooj Imtiaz, Grazyna Fedorowicz, Kevin R FlahertyAbstract:Rationale: Usual Interstitial Pneumonia (UIP) is the histopathologic hallmark of idiopathic pulmonary fibrosis. Although UIP can be detected by high-resolution computed tomography of the chest, the results are frequently inconclusive, and pathology from transbronchial biopsy (TBB) has poor sensitivity. Surgical lung biopsy may be necessary for a definitive diagnosis.Objectives: To develop a genomic classifier in tissue obtained by TBB that distinguishes UIP from non-UIP, trained against central pathology as the reference standard.Methods: Exome enriched RNA sequencing was performed on 283 TBBs from 84 subjects. Machine learning was used to train an algorithm with high rule-in (specificity) performance using specimens from 53 subjects. Performance was evaluated by cross-validation and on an independent test set of specimens from 31 subjects. We explored the feasibility of a single molecular test per subject by combining multiple TBBs from upper and lower lobes. To address whether classifier accuracy depend...
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radiologic pathologic discordance in biopsy proven Usual Interstitial Pneumonia
European Respiratory Journal, 2016Co-Authors: Kunihiro Yagihashi, Henry D. Tazelaar, Thomas V Colby, Jason Huckleberry, Baskaran Sundaram, Sudhakar Pipavath, Marvin I. Schwarz, Jordan Zach, David A LynchAbstract:The aim of this study was to compare the clinical, radiological and histological findings in a large population of subjects enrolled during a multicentre study of idiopathic pulmonary fibrosis, with a focus on discordance between imaging and histologic diagnoses of Usual Interstitial Pneumonia (UIP).Two independent radiologists retrospectively reviewed 241 subjects who underwent high-resolution computed tomography (HRCT) and surgical lung biopsies. HRCT findings were classified as UIP, possible UIP and inconsistent with UIP. Histological findings were classified as definite, probable, possible and not UIP.Of the 241 cases, 102 (42.3%) had HRCT findings of UIP, 64 (26.6%) had possible UIP and 75 (31.1%) were inconsistent with UIP. Among those with UIP on HRCT, 99 (97.1%) had histologically definite or probable UIP (concordant group), and 71 (94.7%) of those with "inconsistent" HRCT features had histologically definite or probable UIP (discordant group). Discordant subjects were slightly younger and less likely to be smokers than concordant subjects, but no survival differences were identified.In this population of patients enrolled with a diagnosis of idiopathic pulmonary fibrosis, 94.7% of those with HRCT findings "inconsistent with UIP" demonstrated histological UIP. This suggests that the term "inconsistent with UIP" is misleading.
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IgG4-Related Lung Disease Associated with Usual Interstitial Pneumonia
The open rheumatology journal, 2016Co-Authors: Frank Schneider, Kristen L. Veraldi, Marc C. Levesque, Thomas V ColbyAbstract:We report a case of immunoglobulin(Ig)G4-related disease with the radiologic and histopathological manifestations resembling Usual Interstitial Pneumonia (UIP). The patient was a 62-year-old man who presented with progressive dyspnea of insidious onset. High resolution computed tomography of the chest showed lower-lobe predominant peripheral reticulation and traction bronchiectasis but no honeycomb change. Microscopic examination of the surgical lung biopsy showed characteristic features of UIP including architectural distortion by fibrosis with peripheral and paraseptal accentuation, scattered fibroblast foci and microscopic honeycomb change. In addition there were prominent multifocal lymphoplasmacytic infiltrates with a marked increase of IgG4-positive plasma cells (79 per high power field in hot spots) and high IgG4/IgG ratio (up to 67%). The serum IgG4 level was elevated at 760 mg/dl (reference range 9-89), with normal levels for the other IgG subclasses and negative serologic markers for autoimmune diseases. The patient’s symptoms improved significantly with oral corticosteroid treatment.
David A Lynch - One of the best experts on this subject based on the ideXlab platform.
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Utility of a Molecular Classifier as a Complement to High-Resolution Computed Tomography to Identify Usual Interstitial Pneumonia.
American journal of respiratory and critical care medicine, 2021Co-Authors: Luca Richeldi, Jeffrey L Myers, Thomas V Colby, David A Lynch, Mary Beth Scholand, Steve D Groshong, Jonathan H. Chung, Sadia Benzaquen, Steven D. Nathan, J Russell DavisAbstract:Rationale: Usual Interstitial Pneumonia (UIP) is the defining morphology of idiopathic pulmonary fibrosis (IPF). Guidelines for IPF diagnosis conditionally recommend surgical lung biopsy for histop...
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use of a molecular classifier to identify Usual Interstitial Pneumonia in conventional transbronchial lung biopsy samples a prospective validation study
The Lancet Respiratory Medicine, 2019Co-Authors: Ganesh Raghu, Kevin R Flaherty, Jeffrey L Myers, Thomas V Colby, David A Lynch, Steve D Groshong, Jonathan H. Chung, David J Lederer, Brandon T Larsen, Mark P SteeleAbstract:Summary Background In the appropriate clinical setting, the diagnosis of idiopathic pulmonary fibrosis (IPF) requires a pattern of Usual Interstitial Pneumonia to be present on high-resolution chest CT (HRCT) or surgical lung biopsy. A molecular Usual Interstitial Pneumonia signature can be identified by a machine learning algorithm in less-invasive transbronchial lung biopsy samples. We report prospective findings for the clinical validity and utility of this molecular test. Methods We prospectively recruited 237 patients for this study from those enrolled in the Bronchial Sample Collection for a Novel Genomic Test (BRAVE) study in 29 US and European sites. Patients were undergoing evaluation for Interstitial lung disease and had had samples obtained by clinically indicated surgical or transbronchial biopsy or cryobiopsy for pathology. Histopathological diagnoses were made by experienced pathologists. Available HRCT scans were reviewed centrally. Three to five transbronchial lung biopsy samples were collected from all patients specifically for this study, pooled by patient, and extracted for transcriptomic sequencing. After exclusions, diagnostic histopathology and RNA sequence data from 90 patients were used to train a machine learning algorithm (Envisia Genomic Classifier, Veracyte, San Francisco, CA, USA) to identify a Usual Interstitial Pneumonia pattern. The primary study endpoint was validation of the classifier in 49 patients by comparison with diagnostic histopathology. To assess clinical utility, we compared the agreement and confidence level of diagnosis made by central multidisciplinary teams based on anonymised clinical information and radiology results plus either molecular classifier or histopathology results. Findings The classifier identified Usual Interstitial Pneumonia in transbronchial lung biopsy samples from 49 patients with 88% specificity (95% CI 70–98) and 70% sensitivity (47–87). Among 42 of these patients who had possible or inconsistent Usual Interstitial Pneumonia on HRCT, the classifier showed 81% positive predictive value (95% CI 54–96) for underlying biopsy-proven Usual Interstitial Pneumonia. In the clinical utility analysis, we found 86% agreement (95% CI 78–92) between clinical diagnoses using classifier results and those using histopathology data. Diagnostic confidence was improved by the molecular classifier results compared with histopathology results in 18 with IPF diagnoses (proportion of diagnoses that were confident or provisional with high confidence 89% vs 56%, p=0·0339) and in all 48 patients with non-diagnostic pathology or non-classifiable fibrosis histopathology (63% vs 42%, p=0·0412). Interpretation The molecular test provided an objective method to aid clinicians and multidisciplinary teams in ascertaining a diagnosis of IPF, particularly for patients without a clear radiological diagnosis, in samples that can be obtained by a less invasive method. Further prospective clinical validation and utility studies are planned. Funding Veracyte.
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Usual Interstitial Pneumonia can be detected in transbronchial biopsies using machine learning
Annals of the American Thoracic Society, 2017Co-Authors: Daniel Pankratz, Kevin K Brown, Jeffrey L Myers, Thomas V Colby, David A Lynch, Jessica D Anderson, Yoonha Choi, Urooj Imtiaz, Grazyna Fedorowicz, Kevin R FlahertyAbstract:Rationale: Usual Interstitial Pneumonia (UIP) is the histopathologic hallmark of idiopathic pulmonary fibrosis. Although UIP can be detected by high-resolution computed tomography of the chest, the results are frequently inconclusive, and pathology from transbronchial biopsy (TBB) has poor sensitivity. Surgical lung biopsy may be necessary for a definitive diagnosis.Objectives: To develop a genomic classifier in tissue obtained by TBB that distinguishes UIP from non-UIP, trained against central pathology as the reference standard.Methods: Exome enriched RNA sequencing was performed on 283 TBBs from 84 subjects. Machine learning was used to train an algorithm with high rule-in (specificity) performance using specimens from 53 subjects. Performance was evaluated by cross-validation and on an independent test set of specimens from 31 subjects. We explored the feasibility of a single molecular test per subject by combining multiple TBBs from upper and lower lobes. To address whether classifier accuracy depend...
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radiologic pathologic discordance in biopsy proven Usual Interstitial Pneumonia
European Respiratory Journal, 2016Co-Authors: Kunihiro Yagihashi, Henry D. Tazelaar, Thomas V Colby, Jason Huckleberry, Baskaran Sundaram, Sudhakar Pipavath, Marvin I. Schwarz, Jordan Zach, David A LynchAbstract:The aim of this study was to compare the clinical, radiological and histological findings in a large population of subjects enrolled during a multicentre study of idiopathic pulmonary fibrosis, with a focus on discordance between imaging and histologic diagnoses of Usual Interstitial Pneumonia (UIP).Two independent radiologists retrospectively reviewed 241 subjects who underwent high-resolution computed tomography (HRCT) and surgical lung biopsies. HRCT findings were classified as UIP, possible UIP and inconsistent with UIP. Histological findings were classified as definite, probable, possible and not UIP.Of the 241 cases, 102 (42.3%) had HRCT findings of UIP, 64 (26.6%) had possible UIP and 75 (31.1%) were inconsistent with UIP. Among those with UIP on HRCT, 99 (97.1%) had histologically definite or probable UIP (concordant group), and 71 (94.7%) of those with "inconsistent" HRCT features had histologically definite or probable UIP (discordant group). Discordant subjects were slightly younger and less likely to be smokers than concordant subjects, but no survival differences were identified.In this population of patients enrolled with a diagnosis of idiopathic pulmonary fibrosis, 94.7% of those with HRCT findings "inconsistent with UIP" demonstrated histological UIP. This suggests that the term "inconsistent with UIP" is misleading.
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Radiologic–pathologic discordance in biopsy-proven Usual Interstitial Pneumonia
The European respiratory journal, 2016Co-Authors: Kunihiro Yagihashi, Henry D. Tazelaar, Thomas V Colby, Jason Huckleberry, Jordan A. Zach, Baskaran Sundaram, Sudhakar Pipavath, Marvin I. Schwarz, David A LynchAbstract:The aim of this study was to compare the clinical, radiological and histological findings in a large population of subjects enrolled during a multicentre study of idiopathic pulmonary fibrosis, with a focus on discordance between imaging and histologic diagnoses of Usual Interstitial Pneumonia (UIP).Two independent radiologists retrospectively reviewed 241 subjects who underwent high-resolution computed tomography (HRCT) and surgical lung biopsies. HRCT findings were classified as UIP, possible UIP and inconsistent with UIP. Histological findings were classified as definite, probable, possible and not UIP.Of the 241 cases, 102 (42.3%) had HRCT findings of UIP, 64 (26.6%) had possible UIP and 75 (31.1%) were inconsistent with UIP. Among those with UIP on HRCT, 99 (97.1%) had histologically definite or probable UIP (concordant group), and 71 (94.7%) of those with "inconsistent" HRCT features had histologically definite or probable UIP (discordant group). Discordant subjects were slightly younger and less likely to be smokers than concordant subjects, but no survival differences were identified.In this population of patients enrolled with a diagnosis of idiopathic pulmonary fibrosis, 94.7% of those with HRCT findings "inconsistent with UIP" demonstrated histological UIP. This suggests that the term "inconsistent with UIP" is misleading.
Takateru Izumi - One of the best experts on this subject based on the ideXlab platform.
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Cell profiles of bronchoalveolar lavage fluid as prognosticators of idiopathic pulmonary fibrosis/Usual Interstitial Pneumonia among Japanese Patients.
Respiration; international review of thoracic diseases, 2005Co-Authors: Rollin P. Tabuena, Sonoko Nagai, Michio Shigematsu, Takeo Tsutsumi, Takateru Izumi, Tomohiro Handa, Takeuchi Minoru, Takeshi Mikuniya, Kunio Hamada, Michiaki MishimaAbstract:Background: The role of bronchoalveolar lavage fluid (BALF) cell profiles in predicting the clinical outcome of idiopathic pulmonary fibrosis (IPF)/Usual Interstitial Pneumonia (UIP
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Serial Evaluation of High-Resolution Computed Tomography Findings in Patients with Idiopathic Pulmonary Fibrosis in Usual Interstitial Pneumonia
Respiration; international review of thoracic diseases, 2002Co-Authors: Taishi Nagao, Sonoko Nagai, Michio Hayashi, Michio Shigematsu, Takateru Izumi, Kunio Hamada, Yoshihide Hiramoto, Michiaki MishimaAbstract:Background: The development of well-matured fibrosis in Usual Interstitial Pneumonia (UIP) is strongly associated with an unfavorable outcome of patients with idiopathic pulmonary f
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Usual Interstitial Pneumonia: Idiopathic Pulmonary Fibrosis versus Collagen Vascular Diseases
Respiration; international review of thoracic diseases, 2001Co-Authors: Taishi Nagao, Masanori Kitaichi, Sonoko Nagai, Michio Hayashi, Michio Shigematsu, Takeo Tsutsumi, Satake N, Takateru IzumiAbstract:Background: Bronchoalveolar lavage fluid (BALF) lymphocytosis was found in patients with Usual Interstitial Pneumonia (UIP) associated with collagen vascular diseases (CVD) other th
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Usual Interstitial Pneumonia histologic correlation with high resolution ct
Radiology, 1992Co-Authors: K Nishimura, Masanori Kitaichi, Sonoko Nagai, Takateru Izumi, M Kanaoka, Harumi ItohAbstract:The authors reviewed 46 cases of idiopathic pulmonary fibrosis with Usual Interstitial Pneumonia (UIP), correlating findings on high-resolution computed tomographic (HRCT) scans with findings in specimens obtained at open lung biopsy and autopsy. The following HRCT findings were observed: (a) an accumulation of small cystic spaces with thick walls, (b) air bronchiolograms within areas of intense lung attenuation, (c) rugged pleural surfaces, (d) irregularly thickened pulmonary vessels, (e) bronchial wall thickening, and (f) slightly increased lung attenuation. Macroscopic honeycombing correlating with small cystic spaces was demonstrated at HRCT and pathologic examination. Air bronchiolograms in the areas of intense lung attenuation (ie, microscopic honeycombing) corresponded to dilated bronchioles (greater than 1 mm in diameter) with fibrosis. Irregularly thickened vessels and bronchial walls and irregular pleural surfaces were the result of fibrosis in the periphery of the secondary pulmonary lobules. A...
Kevin R Flaherty - One of the best experts on this subject based on the ideXlab platform.
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use of a molecular classifier to identify Usual Interstitial Pneumonia in conventional transbronchial lung biopsy samples a prospective validation study
The Lancet Respiratory Medicine, 2019Co-Authors: Ganesh Raghu, Kevin R Flaherty, Jeffrey L Myers, Thomas V Colby, David A Lynch, Steve D Groshong, Jonathan H. Chung, David J Lederer, Brandon T Larsen, Mark P SteeleAbstract:Summary Background In the appropriate clinical setting, the diagnosis of idiopathic pulmonary fibrosis (IPF) requires a pattern of Usual Interstitial Pneumonia to be present on high-resolution chest CT (HRCT) or surgical lung biopsy. A molecular Usual Interstitial Pneumonia signature can be identified by a machine learning algorithm in less-invasive transbronchial lung biopsy samples. We report prospective findings for the clinical validity and utility of this molecular test. Methods We prospectively recruited 237 patients for this study from those enrolled in the Bronchial Sample Collection for a Novel Genomic Test (BRAVE) study in 29 US and European sites. Patients were undergoing evaluation for Interstitial lung disease and had had samples obtained by clinically indicated surgical or transbronchial biopsy or cryobiopsy for pathology. Histopathological diagnoses were made by experienced pathologists. Available HRCT scans were reviewed centrally. Three to five transbronchial lung biopsy samples were collected from all patients specifically for this study, pooled by patient, and extracted for transcriptomic sequencing. After exclusions, diagnostic histopathology and RNA sequence data from 90 patients were used to train a machine learning algorithm (Envisia Genomic Classifier, Veracyte, San Francisco, CA, USA) to identify a Usual Interstitial Pneumonia pattern. The primary study endpoint was validation of the classifier in 49 patients by comparison with diagnostic histopathology. To assess clinical utility, we compared the agreement and confidence level of diagnosis made by central multidisciplinary teams based on anonymised clinical information and radiology results plus either molecular classifier or histopathology results. Findings The classifier identified Usual Interstitial Pneumonia in transbronchial lung biopsy samples from 49 patients with 88% specificity (95% CI 70–98) and 70% sensitivity (47–87). Among 42 of these patients who had possible or inconsistent Usual Interstitial Pneumonia on HRCT, the classifier showed 81% positive predictive value (95% CI 54–96) for underlying biopsy-proven Usual Interstitial Pneumonia. In the clinical utility analysis, we found 86% agreement (95% CI 78–92) between clinical diagnoses using classifier results and those using histopathology data. Diagnostic confidence was improved by the molecular classifier results compared with histopathology results in 18 with IPF diagnoses (proportion of diagnoses that were confident or provisional with high confidence 89% vs 56%, p=0·0339) and in all 48 patients with non-diagnostic pathology or non-classifiable fibrosis histopathology (63% vs 42%, p=0·0412). Interpretation The molecular test provided an objective method to aid clinicians and multidisciplinary teams in ascertaining a diagnosis of IPF, particularly for patients without a clear radiological diagnosis, in samples that can be obtained by a less invasive method. Further prospective clinical validation and utility studies are planned. Funding Veracyte.
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Usual Interstitial Pneumonia can be detected in transbronchial biopsies using machine learning
Annals of the American Thoracic Society, 2017Co-Authors: Daniel Pankratz, Kevin K Brown, Jeffrey L Myers, Thomas V Colby, David A Lynch, Jessica D Anderson, Yoonha Choi, Urooj Imtiaz, Grazyna Fedorowicz, Kevin R FlahertyAbstract:Rationale: Usual Interstitial Pneumonia (UIP) is the histopathologic hallmark of idiopathic pulmonary fibrosis. Although UIP can be detected by high-resolution computed tomography of the chest, the results are frequently inconclusive, and pathology from transbronchial biopsy (TBB) has poor sensitivity. Surgical lung biopsy may be necessary for a definitive diagnosis.Objectives: To develop a genomic classifier in tissue obtained by TBB that distinguishes UIP from non-UIP, trained against central pathology as the reference standard.Methods: Exome enriched RNA sequencing was performed on 283 TBBs from 84 subjects. Machine learning was used to train an algorithm with high rule-in (specificity) performance using specimens from 53 subjects. Performance was evaluated by cross-validation and on an independent test set of specimens from 31 subjects. We explored the feasibility of a single molecular test per subject by combining multiple TBBs from upper and lower lobes. To address whether classifier accuracy depend...
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variable prostaglandin e2 resistance in fibroblasts from patients with Usual Interstitial Pneumonia
American Journal of Respiratory and Critical Care Medicine, 2008Co-Authors: Steven K Huang, Scott H Wettlaufer, Cory M Hogaboam, Kevin R Flaherty, Jeffrey L Myers, Galen B Toews, William D Travis, Thomas V Colby, Fernando J. Martinez, Marc PetersgoldenAbstract:Rationale: Prostaglandin (PG) E2, a cyclooxygenase-derived lipid mediator, is a potent down-regulator of fibroblast activation in normal lung fibroblasts. Although fibroblasts from patients with idiopathic pulmonary fibrosis are known to exhibit a defect in PGE2 synthesis, there is little information about their responsiveness to this lipid mediator.Objectives: To compare responses to PGE2 in normal, Usual Interstitial Pneumonia (UIP), and other diffuse parenchymal lung disease (DPLD) fibroblasts.Methods: Fibroblasts were grown in vitro from well characterized control (n = 7), UIP (n = 17), or other DPLD (n = 13) lung tissue. The effects of PGE2 on fibroblast proliferation and collagen expression were determined.Measurements and Main Results: Only 3 of 12 UIP fibroblast lines exhibited PGE2-mediated inhibition of both collagen synthesis and cell proliferation, as opposed to 6 of 6 nonfibrotic control cell lines. The degree of PGE2 resistance in DPLD fibroblasts was quite variable, with UIP cells exhibitin...
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fibroblastic foci in Usual Interstitial Pneumonia idiopathic versus collagen vascular disease
American Journal of Respiratory and Critical Care Medicine, 2003Co-Authors: Kevin R Flaherty, Galen B Toews, William D Travis, Thomas V Colby, Jeanette A Mumford, Susan Murray, Victor J Thannickal, Ella A Kazerooni, Barry H Gross, Joseph P. LynchAbstract:A histologic feature of Usual Interstitial Pneumonia is the presence gators have noted a prognostic value of quantifying fibroof fibroblastic foci. As some patients with Usual Interstitial pneumo- blastic foci (FF) in patients with idiopathic Interstitial pneunia and an underlying collagen vascular disease have a better prog- monia (19, 20). Furthermore, several groups have suggested nosis, we hypothesized that they would have fewer fibroblastic foci. differences in the alveolar microenvironment and fibroblast Pathologists reviewed surgical lung biopsies from 108 patients with phenotype between patients with idiopathic and collagenUsual Interstitial Pneumonia (nine with collagen vascular disease) vascular associated pulmonary fibrosis (21‐24). We hypotheand assigned a score (absent 0, mild 1, moderate 2, and marked sized that patients with CVD-associated UIP would have an 3) for fibroblastic foci. Patients with idiopathic Usual Interstitial improved survival compared with patients with idiopathic Pneumonia had a higher median profusion of fibroblastic foci (1.75 UIP and that this improved survival would correlate with vs. 1.0, p 0.003). Baseline characteristics were similar, although the profusion of FF. patients with a collagen vascular disease were younger, had a shorter duration of symptoms, and had a higher percentage of METHODS predicted total lung capacity. Profusion of fibroblastic foci was the most discriminative feature for separating idiopathic from collagen Patient Recruitment and Therapy vascular disease–associated Usual Interstitial Pneumonia (odds ratio This study used information from the University of Michigan Special8.31; 95% confidence interval, 1.98, 59.42; p 0.002 for a one- ized Center of Research in the Pathobiology of Fibrotic Lung Disease unit increase in fibroblastic foci score). No deaths were noted in the database. Patients were referred for enrollment in study protocols for collagen vascular disease–associated Usual Interstitial Pneumonia suspected IPF and underwent surgical lung biopsy between October group; 52 deaths occurred in the idiopathic Usual Interstitial pneu- 1989 and February 2000. In these patients, a suspicion of IPF was based monia group (log rank; p 0.005). We conclude that patients with on symptoms, physiologic abnormalities, and radiographic findings (3). collagen vascular disease–associated Usual Interstitial Pneumonia Patients were excluded if they were found to have a disease other have fewer fibroblastic foci and improved survival. than IPF during the enrollment evaluation. Diseases that were exclusionary included pneumoconiosis, sarcoidosis, carcinoma, lymphoma, Langer
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Usual Interstitial Pneumonia.
Seminars in respiratory and critical care medicine, 2001Co-Authors: Joseph P. Lynch, Kevin R Flaherty, Fernando J. Martinez, Mark Wurfel, Eric S. White, William Travis, Ganesh RaghuAbstract:Usual Interstitial Pneumonia (UIP) is a distinct histological lesion observed in idiopathic pulmonary fibrosis (IPF), but can be found in other etiologies. The diagnosis of UIP can be established by surgical lung biopsy or by high resolution thin section computed tomographic scans (provided the radiographic features are classical). Historically, patients labeled as "IPF'' encompassed a group of disorders including UIP as well as other idiopathic Interstitial Pneumonias, which differ from UIP in prognosis and responsiveness to therapy. Current recommendations from international consensus statements restrict the term IPF to patients with idiopathic UIP. The inciting cause(s) and pathogenesis of UIP have not been elucidated, but alveolar epithelial cell injury and dysregulation or altered phenotypic expression of fibroblasts are key elements. Inflammatory cells may play minor roles in initiating or propagating the fibrotic process. The prognosis of UIP is poor. Mean survival following diagnosis approximates 3 years. Current therapies are of unproven value. Corticosteroids or immunosuppressive agents have been most often used, but data affirming benefit are lacking. Single-lung transplantation is a viable option for patients failing medical therapy. Novel therapeutic strategies based upon inhibiting fibroproliferation or enhancing alveolar reepithelialization are desperately needed. In this article, we discuss diagnostic criteria for UIP (both histopathological and radiographic), natural history and clinical course, and therapeutic approaches (both current and future).
Takeshi Johkoh - One of the best experts on this subject based on the ideXlab platform.
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pathologically proved nonspecific Interstitial Pneumonia ct pattern analysis as compared with Usual Interstitial Pneumonia ct pattern
Radiology, 2014Co-Authors: Hiromitsu Sumikawa, Kensuke Kataoka, Takeshi Johkoh, Junya Fukuoka, Hiroyuki Taniguchi, Thomas V Colby, Kiminori Fujimoto, Yasuhiro Kondoh, Hiroaki Arakawa, Takashi OguraAbstract:Although patients with pathologically proved idiopathic pulmonary fibrosis/Usual Interstitial Pneumonia showed variable CT patterns, those with pathologically proved nonspecific Interstitial Pneumonia showed less variable CT patterns.
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Nonspecific Interstitial Pneumonia and Usual Interstitial Pneumonia: is differentiation possible by high-resolution computed tomography?
Seminars in Ultrasound Ct and Mri, 2013Co-Authors: Takeshi JohkohAbstract:Abstract Nonspecific Interstitial Pneumonia (NSIP) is a form of idiopathic Interstitial Pneumonia characterized histologically by varying degrees of Interstitial inflammation and fibrosis that are temporally and morphologically homogeneous in comparison with Usual Interstitial Pneumonia (UIP). Differentiation from UIP is very important because treatment and prognosis are different between NSIP and UIP. Although there are limitations for the differentiation between NSIP and UIP on computed tomography, some computed tomography findings contribute to it. Relatively peribronchovascular distribution, wide extent of areas with ground-glass attenuation, and subpleural sparing are more highly seen in patients with NSIP, whereas wide extent of honeycombing and subpleural distribution are more common characteristics in patients with UIP.
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Chronic hypersensitivity pneumonitis and pulmonary sarcoidosis: differentiation from Usual Interstitial Pneumonia using high-resolution computed tomography.
Seminars in ultrasound CT and MR, 2013Co-Authors: Yeon Joo Jeong, Takeshi Johkoh, Man Pyo Chung, Joungho Han, Kyung Soo Lee, Kazuya IchikadoAbstract:The distinction of chronic hypersensitivity pneumonitis (HP) or advanced-stage sarcoidosis from idiopathic pulmonary fibrosis or Usual Interstitial Pneumonia is important because each disease is managed differently and may have a different prognosis. The analyses of pattern and distribution of lung parenchymal abnormalities on high-resolution computed tomography scans help differentiate among the 3 diseases. In chronic HP, the presence of lobular areas of decreased attenuation and centrilobular small nodules and the absence of lower lung zone predominance are characteristically observed. In advanced-stage sarcoidosis, patchy areas of reticulation, traction bronchiectasis, architectural distortion, honeycomblike cysts, bullae, and paracicatricial emphysema are observed in the upper and middle lung zones. Lung bases are Usually spared. In idiopathic pulmonary fibrosis or Usual Interstitial Pneumonia, however, the presence of honeycombing with lower lung zone predominance and the absence of centrilobular small nodules are important findings that allow us to differentiate the disease from chronic HP or advanced-stage sarcoidosis. In the 3 diseases, most important prognosis-predicting factor is the extent of fibrotic score (the extent of honeycombing and reticulation) calculated on high-resolution computed tomography scans or fibrosis estimated on chest radiographs.
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Do you really know precise radiologic-pathologic correlation of Usual Interstitial Pneumonia?
European journal of radiology, 2013Co-Authors: Takeshi Johkoh, Hiromitsu Sumikawa, Junya Fukuoka, Kiminori Fujimoto, Tomonori Tanaka, Masashi Takahashi, Noriyuki Tomiyama, Yasuhiro Kondo, Hiroyuki TaniguchiAbstract:Although Usual Interstitial Pneumonia (UIP) is the most common chronic Interstitial Pneumonia, understanding of pathologic backgrounds of CT findings has still not been enough. Since honeycombing on either scanning microgram or CT is essential for diagnosis of UIP in 2010 ATS-ERS-JRS-ALAT guide line, the role of radiologists has become much more important. We will summarize common and uncommon CT findings with radiologic-pathological correlation.
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Computed tomography findings in pathological Usual Interstitial Pneumonia: relationship to survival.
American journal of respiratory and critical care medicine, 2007Co-Authors: Hiromitsu Sumikawa, Takeshi Johkoh, Takashi Ogura, Hiroyuki Taniguchi, Thomas V Colby, Yasuhiro Kondoh, Hiroaki Arakawa, Kazuya Ichikado, Moritaka Suga, Kiminori FujimotoAbstract:Rationale: Patients with a clinicopathological diagnosis of idiopathic pulmonary fibrosis (IPF) may have typical findings of Usual Interstitial Pneumonia (UIP) on computed tomography (CT) or nonspecific or atypical findings, including those often seen in nonspecific Interstitial Pneumonia.Objectives: The aims of this study were to revisit the high-resolution CT findings of IPF and to clarify the correlation between the CT findings and mortality.Methods: The study included 98 patients with a histologic diagnosis of UIP and a clinical diagnosis of IPF. Two observers evaluated the CT findings independently and classified each case into one of the following three categories: (1) definite UIP, (2) consistent with UIP, or (3) suggestive of alternative diagnosis. The correlation between the CT categories and mortality was evaluated using the Kaplan-Meier method and the log-rank test, as well as Cox proportional hazards regression models.Measurements and Main Results: Thirty-three of the 98 CT scans were classifi...
