• Title/Summary/Keyword: Fibrotic lung disease

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Occupational Lung Diseases: Spectrum of Common Imaging Manifestations

  • Alexander W. Matyga;Lydia Chelala;Jonathan H. Chung
    • Korean Journal of Radiology
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    • v.24 no.8
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    • pp.795-806
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    • 2023
  • Occupational lung diseases (OLD) are a group of preventable conditions caused by noxious inhalation exposure in the workplace. Workers in various industries are at a higher risk of developing OLD. Despite regulations contributing to a decreased incidence, OLD remain among the most frequently diagnosed work-related conditions, contributing to significant morbidity and mortality. A multidisciplinary discussion (MDD) is necessary for a timely diagnosis. Imaging, particularly computed tomography, plays a central role in diagnosing OLD and excluding other inhalational lung diseases. OLD can be broadly classified into fibrotic and non-fibrotic forms. Imaging reflects variable degrees of inflammation and fibrosis involving the airways, parenchyma, and pleura. Common manifestations include classical pneumoconioses, chronic granulomatous diseases (CGD), and small and large airway diseases. Imaging is influenced by the type of inciting exposure. The findings of airway disease may be subtle or solely uncovered upon expiration. High-resolution chest CT, including expiratory-phase imaging, should be performed in all patients with suspected OLD. Radiologists should familiarize themselves with these imaging features to improve diagnostic accuracy.

Recurrent Desquamative Interstitial Pneumonia with Fibrotic Lung Disease (폐섬유화를 동반한 재발성 박리성 간질성 폐렴)

  • Kim, Won Jin;Choi, Jeong Hee;Park, Yong Bum;Cho, Sung Woo;Nam, Eun Sook;Mo, Eun Kyung
    • Tuberculosis and Respiratory Diseases
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    • v.65 no.4
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    • pp.328-333
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    • 2008
  • Desquamative interstitial pneumonia is an uncommon form of interstitial lung diseases and it has a good prognosis compared with other types of idiopathic interstitial pneumonia. A 69-year old man was admitted to our hospital because of a 3-month history of dyspnea. The patient presented with hypoxemia. High-resolution computerized tomography of the patient showed ground glass opacity and traction bronchiectasis with subpleural early honeycombing on the both lung fields. The pathologic findings of the video-assisted thoracoscopy lung biopsy were compatible with desquamative interstitial pneumonia, and irregularly distributed interstitial fibrosis and inflammation were observed at the peripheral parenchyme. Oral predinsolone was started; his symptoms and chest x-ray were improved, and so he stopped taking the prednisolone. Ten months later, the desquamative interstitial pneumonia recurred. We report here on a case of recurrent desquamative interstitial pneumonia with fibrotic lung disease.

Role of Krebs von den Lungen-6 (KL-6) in Assessing Hypersensitivity Pneumonitis

  • Mostafa, Amira Ismail;Salem, Ayman Elsayed;Ahmed, Heba Allah Moussa;Bayoumi, Aml Ibrahim;Halim, Radwa M. Abdel;Samie, Rasha M. Abdel
    • Tuberculosis and Respiratory Diseases
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    • v.84 no.3
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    • pp.200-208
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    • 2021
  • Background: Hypersensitivity pneumonitis (HP) is an increasingly recognized form of diffuse parenchymal lung disease. Krebs von den Lungen-6 (KL-6) is now classified as a human MUC1 mucin protein, and regenerating type II pneumocytes are the primary cellular source of KL-6/MUC1 in the affected lungs of patients with interstitial lung diseases (ILD). Serum KL-6/MUC1 levels have been demonstrated to be useful for the evaluation of various ILD. To determine the role of circulating KL-6 in evaluating the disease activity and management of HP. Methods: An observational cross-sectional study was conducted on 51 patients with HP and 20 healthy controls. Serum KL-6 levels were measured in both groups. Patients were further assessed based on chest high-resolution computed tomography (HRCT), pulmonary function test, 6-minute walk test, echocardiography, bronchioalveolar lavage, and/or transbronchial biopsy. Patients were divided into the fibrotic and non-fibrotic groups according to the HRCT findings. Results: The median serum KL-6 levels were significantly higher in HP patients as compared to the control group. The median serum KL-6 levels were found to be higher in the non-fibrotic HP group (1,900 IU/mL) as compared to the fibrotic group (1,200 IU/mL). There was a significant inverse correlation between serum KL-6 serum level and the dose of steroids as well as the duration of steroid therapy. Conclusion: The presence of higher KL-6 levels in the non-fibrotic HP group implies its enhanced production by regenerating pneumocytes in response to alveolar injury. The significant association between serum KL-6 levels and the dose and the duration of steroid therapy emphasizes the significant role of steroids in the stabilization of the disease.

Genistein alleviates pulmonary fibrosis by inactivating lung fibroblasts

  • Seung-hyun Kwon;Hyunju Chung;Jung-Woo Seo;Hak Su Kim
    • BMB Reports
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    • v.57 no.3
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    • pp.143-148
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    • 2024
  • Pulmonary fibrosis is a serious lung disease that occurs predominantly in men. Genistein is an important natural soybean-derived phytoestrogen that affects various biological functions, such as cell migration and fibrosis. However, the antifibrotic effects of genistein on pulmonary fibrosis are largely unknown. The antifibrotic effects of genistein were evaluated using in vitro and in vivo models of lung fibrosis. Proteomic data were analyzed using nano-LC-ESI-MS/MS. Genistein significantly reduced transforming growth factor (TGF)-β1-induced expression of collagen type I and α-smooth muscle actin (SMA) in MRC-5 cells and primary fibroblasts from patients with idiopathic pulmonary fibrosis (IPF). Genistein also reduced TGF-β1-induced expression of p-Smad2/3 and p-p38 MAPK in fibroblast models. Comprehensive protein analysis confirmed that genistein exerted an anti-fibrotic effect by regulating various molecular mechanisms, such as unfolded protein response, epithelial mesenchymal transition (EMT), mammalian target of rapamycin complex 1 (mTORC1) signaling, cell death, and several metabolic pathways. Genistein was also found to decrease hydroxyproline levels in the lungs of BLM-treated mice. Genistein exerted an anti-fibrotic effect by preventing fibroblast activation, suggesting that genistein could be developed as a pharmacological agent for the prevention and treatment of pulmonary fibrosis.

Postinfectious bronchiolitis obliterans in children: lessons from bronchiolitis obliterans after lung transplantation and hematopoietic stem cell transplantation

  • Yu, Jinho
    • Clinical and Experimental Pediatrics
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    • v.58 no.12
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    • pp.459-465
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    • 2015
  • Postinfectious bronchiolitis obliterans (PIBO) is an irreversible obstructive lung disease characterized by subepithelial inflammation and fibrotic narrowing of the bronchioles after lower respiratory tract infection during childhood, especially early childhood. Although diagnosis of PIBO should be confirmed by histopathology, it is generally based on history and clinical findings. Irreversible airway obstruction is demonstrated by decreased forced expiratory volume in 1 second with an absent bronchodilator response, and by mosaic perfusion, air trapping, and/or bronchiectasis on computed tomography images. However, lung function tests using spirometry are not feasible in young children, and most cases of PIBO develop during early childhood. Further studies focused on obtaining serial measurements of lung function in infants and toddlers with a risk of bronchiolitis obliterans (BO) after lower respiratory tract infection are therefore needed. Although an optimal treatment for PIBO has not been established, corticosteroids have been used to target the inflammatory component. Other treatment modalities for BO after lung transplantation or hematopoietic stem cell transplantation have been studied in clinical trials, and the results can be extrapolated for the treatment of PIBO. Lung transplantation remains the final option for children with PIBO who have progressed to end-stage lung disease.

Relationship between Incidental Abnormalities on Screening Thoracic Computed Tomography and Mortality: A Long-Term Follow-Up Analysis

  • Jong Eun Lee;Won Gi Jeong;Hyo-Jae Lee;Yun-Hyeon Kim;Kum Ju Chae;Yeon Joo Jeong
    • Korean Journal of Radiology
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    • v.23 no.10
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    • pp.998-1008
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    • 2022
  • Objective: The present study aimed to assess the relationship between incidental abnormalities on thoracic computed tomography (CT) and mortality in a general screening population using a long-term follow-up analysis. Materials and Methods: We retrospectively collected the medical records and CT images of 840 participants (mean age ± standard deviation [SD], 58.5 ± 6.7 years; 564 male) who underwent thoracic CT at a single health promotion center between 2007 and 2010. Two thoracic radiologists independently reviewed all CT images and evaluated any incidental abnormalities (interstitial lung abnormality [ILA], emphysema, coronary artery calcification [CAC], aortic valve [AV] calcification, and pulmonary nodules). Kaplan-Meier analysis with log-rank and z-tests was performed to assess the relationship between incidental CT abnormalities and all-cause mortality in the subsequent follow-up. Cox proportional hazards regression was performed to further identify risk factors of all-cause mortality among the incidental CT abnormalities and clinical factors. Results: Among the 840 participants, 55 (6%), 171 (20%), 288 (34%), 396 (47%), and 97 (11%) had findings of ILA, emphysema, CAC, pulmonary nodule, and AV calcification, respectively, on initial CT. The participants were followed up for a mean period ± SD of 10.9 ± 1.4 years. All incidental CT abnormalities were associated with all-cause mortality in univariable analysis (p < 0.05). However, multivariable analysis further revealed fibrotic ILA as an independent risk factor for all-cause mortality (hazard ratio, 2.52 [95% confidence interval, 1.02-6.22], p = 0.046). ILA were also identified as an independent risk factor for lung cancer or respiratory disease-related deaths. Conclusion: Incidental abnormalities on screening thoracic CT were associated with increased mortality during the long-term follow-up. Among incidental CT abnormalities, fibrotic ILA were independently associated with increased mortality. Appropriate management and surveillance may be required for patients with fibrotic ILA on thoracic CT obtained for general screening purposes.

Therapeutic potential of targeting kinase inhibition in patients with idiopathic pulmonary fibrosis

  • Kim, Suji;Lim, Jae Hyang;Woo, Chang-Hoon
    • Journal of Yeungnam Medical Science
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    • v.37 no.4
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    • pp.269-276
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    • 2020
  • Fibrosis is characterized by excessive accumulation of extracellular matrix components. The fibrotic process ultimately leads to organ dysfunction and failure in chronic inflammatory and metabolic diseases such as pulmonary fibrosis, advanced kidney disease, and liver cirrhosis. Idiopathic pulmonary fibrosis (IPF) is a common form of progressive and chronic interstitial lung disease of unknown etiology. Pathophysiologically, the parenchyma of the lung alveoli, interstitium, and capillary endothelium becomes scarred and stiff, which makes breathing difficult because the lungs have to work harder to transfer oxygen and carbon dioxide between the alveolar space and bloodstream. The transforming growth factor beta (TGF-β) signaling pathway plays an important role in the pathogenesis of pulmonary fibrosis and scarring of the lung tissue. Recent clinical trials focused on the development of pharmacological agents that either directly or indirectly target kinases for the treatment of IPF. Therefore, to develop therapeutic targets for pulmonary fibrosis, it is essential to understand the key factors involved in the pathogenesis of pulmonary fibrosis and the underlying signaling pathway. The objective of this review is to discuss the role of kinase signaling cascades in the regulation of either TGF-β-dependent or other signaling pathways, including Rho-associated coiled-coil kinase, c-jun N-terminal kinase, extracellular signal-regulated kinase 5, and p90 ribosomal S6 kinase pathways, and potential therapeutic targets in IPF.

The Clinical Assessment of Protease-Activated Receptor-2 Expression in Inflammatory Cells from Peripheral Blood and Bronchoalveolar Lavage Fluid in Idiopathic Pulmonary Fibrosis

  • Park, Young Sik;Yoo, Chul-Gyu
    • Tuberculosis and Respiratory Diseases
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    • v.74 no.6
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    • pp.264-268
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    • 2013
  • Background: Idiopathic pulmonary fibrosis (IPF) is a lethal pulmonary fibrotic disease. In general, the exaggerated activation of the coagulation cascade has been observed during initiation or maintenance of the fibrotic disease. In our recent study, immunohistochemical expression of protease-activated receptor-2 (PAR-2), which plays a key role in coagulation cascade, was observed in surgical specimen of IPF patients, and associated with poor clinical outcome. The aim of this study was to evaluate the overexpression of PAR-2 in inflammatory cells from peripheral blood and bronchoalveolar lavage fluid in IPF patients. Methods: From May 2011 to March 2012, IPF patients and controls were enrolled in Seoul National University Hospital. Peripheral blood and bronchoalveolar lavage fluid were collected for analysis of PAR-2 expression. Flow cytometry and reverse transcription polymerase chain reaction were used for PAR-2 receptor and mRNA assessment. Results: Twelve IPF patients and 14 controls were included in this study. Among them, flow cytometry analysis was conducted from 26 peripheral blood (patient group, 11; control group, 13) and 7 bronchoalveolar lavage fluid (patient group, 5; control group, 2). The expression of PAR-2 receptor was not different between patient and control groups (p=0.074). Among all 24 population, PAR-2 mRNA assessment was performed in 19 persons (patient group, 10; control group, 9). The mRNA expression of PAR-2 was not significant different (p=0.633). Conclusion: In IPF patients, PAR-2 receptor and mRNA expression were not different from control group.

Eupatilin treatment inhibits transforming growth factor beta-induced endometrial fibrosis in vitro

  • Lee, Chang-Jin;Hong, Seon-Hwa;Yoon, Min-Ji;Lee, Kyung-Ah;Choi, Dong Hee;Kwon, Hwang;Ko, Jung-Jae;Koo, Hwa Seon;Kang, Youn-Jung
    • Clinical and Experimental Reproductive Medicine
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    • v.47 no.2
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    • pp.108-113
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    • 2020
  • Objective: Endometrial fibrosis, the primary pathological feature of intrauterine adhesion, may lead to disruption of endometrial tissue structure, menstrual abnormalities, infertility, and recurrent pregnancy loss. At present, no ideal therapeutic strategy exists for this fibrotic disease. Eupatilin, a major pharmacologically active flavone from Artemisia, has been previously reported to act as a potent inducer of dedifferentiation of fibrotic tissue in the liver and lung. However, the effects of eupatilin on endometrial fibrosis have not yet been investigated. In this study, we present the first report on the impact of eupatilin treatment on transforming growth factor beta (TGF-β)-induced endometrial fibrosis. Methods: The efficacy of eupatilin on TGF-β-induced endometrial fibrosis was assessed by examining changes in morphology and the expression levels of fibrosis markers using immunofluorescence staining and quantitative real-time reverse-transcription polymerase chain reaction. Results: Eupatilin treatment significantly reduced the fibrotic activity of TGF-β-induced endometrial fibrosis in Ishikawa cells, which displayed more circular shapes and formed more colonies. Additionally, the effects of eupatilin on fibrotic markers including alpha-smooth muscle actin, hypoxia-inducible factor 1 alpha, collagen type I alpha 1 chain, and matrix metalloproteinase-2, were evaluated in TGF-β-induced endometrial fibrosis. The expression of these markers was highly upregulated by TGF-β pretreatment and recovered to the levels of control cells in response to eupatilin treatment. Conclusion: Our findings suggest that suppression of TGF-β-induced signaling by eupatilin might be an effective therapeutic strategy for the treatment of endometrial fibrosis.

Medical Imaging and Nuclear Molecular Imaging Probes for Pulmonary Fibrosis Diagnosis

  • Heesu Ahn;Yong Jin Lee
    • Journal of Radiopharmaceuticals and Molecular Probes
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    • v.8 no.2
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    • pp.103-111
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    • 2022
  • Idiopathic pulmonary fibrosis (IPF) is a progressive disease caused by some risk factors, including smoking, viral infection, toxic substances, and radiation, that decline lung function of fresh oxygen and blood delivery throughout the body. Patients with pulmonary fibrosis have suffered from breathing and cough and the average survival rate is only 3 years after diagnosis. Therefore, it is significant to diagnose IPF and start treatment in enough time. Usually, lung biopsy is available to diagnose localized pulmonary fibrotic sites directly. However, it is insufficient to visualize whole lung tissue, and also it has a risk of infection for patients. In the clinic, medical imaging systems can diagnose pulmonary fibrosis non-invasively without infection. In this review, we introduce current medical imaging systems used to diagnose pulmonary fibrosis, including CT, MRI, and nuclear medicine. Further, we introduce several molecular imaging probes targeting specific biomarkers which are expressed in pulmonary fibrosis. Through this paper, it is expected that it would be helpful to understand the latest knowledge and research trends on pulmonary fibrosis diagnostic imaging.