• Title/Summary/Keyword: Pulmonary fibrosis

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A Case of Systemic Sclerosis Sine Scleroderma Presenting as Pulmonary Interstitial Fibrosis (피부병변없이 간질성 폐섬유화로 표현된 경피증 1례)

  • Kwak, Jin-Ho;Choi, Won-II;Lee, Seung-Hyun;Seo, Chang-Gyun;Kim, Kyung-Chan;Kim, Min-Su;Kwon, Kun-Young;Suh, Soo-Ji;Park, Chang-Kwon;Jeon, Young-June
    • Tuberculosis and Respiratory Diseases
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    • v.50 no.4
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    • pp.493-498
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    • 2001
  • Lung involvement in systemic sclerosis(SSC) is common but usually occurs late in the course. Skin changes usually occur before the pulmonary findings. In this report, a patient who developed pulmonary interstitial fibrosis without skin changes is presented. A diagnosis of SSC lung involvement was made histologically. The a nti-scl-70 antibody test was positive. Esophageal manometry revealed a lower amplitude in the lower two-third of the esophagus and pressure in the lower esophageal sphincter. Here we report a case of systemic sclerosis sine scleroderma presenting as pulmonary interstitial fibrosis with a review of the relevant literatures.

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Searching for Novel Candidate Small Molecules for Ameliorating Idiopathic Pulmonary Fibrosis: a Narrative Review

  • Kyung-il Kim;Rajib Hossain;Xin Li;Hyun Jae Lee;Choong Jae Lee
    • Biomolecules & Therapeutics
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    • v.31 no.5
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    • pp.484-495
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    • 2023
  • Idiopathic pulmonary fibrosis (IPF) can be defined as a progressive chronic pulmonary disease showing scarring in the lung parenchyma, thereby resulting in increase in mortality and decrease in the quality of life. The pathophysiologic mechanism of fibrosis in IPF is still unclear. Repetitive microinjuries to alveolar epithelium with genetical predisposition and an abnormal restorative reaction accompanied by excessive deposition of collagens are involved in the pathogenesis. Although the two FDA-approved drugs, pirfenidone and nintedanib, are under use for retarding the decline in lung function of patients suffered from IPF, they are not able to improve the survival rate or quality of life. Therefore, a novel therapeutic agent acting on the major steps of the pathogenesis of disease and/or, at least, managing the clinical symptoms of IPF should be developed for the effective regulation of this incurable disease. In the present review, we tried to find a potential of managing the clinical symptoms of IPF by natural products derived from medicinal plants used for controlling the pulmonary inflammatory diseases in traditional Asian medicine. A multitude of natural products have been reported to exert an antifibrotic effect in vitro and in vivo through acting on the epithelial-mesenchymal transition pathway, transforming growth factor (TGF)- β-induced intracellular signaling, and the deposition of extracellular matrix. However, clinical antifibrotic efficacy of these natural products on IPF have not been elucidated yet. Thus, those effects should be proven by further examinations including the randomized clinical trials, in order to develop the ideal and optimal candidate for the therapeutics of IPF.

Inhibitory Effect of Fucoidan on TGF-β1-Induced Activation of Human Pulmonary Fibroblasts (후코이단에 의한 인간 폐 섬유모세포의 활성 억제 효과)

  • Yim, Mi-Jin;Lee, Dae-Sung;Choi, Grace;Lee, Jeong Min;Choi, Il-Whan
    • Korean Journal of Fisheries and Aquatic Sciences
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    • v.49 no.6
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    • pp.807-814
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    • 2016
  • Fucoidan, one of the dominant sulfated polysaccharides extracted from brown seaweed, possesses a wide range of biological activities. Transforming growth $factor-{\beta}$ ($TGF-{\beta}$) plays a pivotal role in the pathogenesis of pulmonary fibrosis, by stimulating the synthesis of profibrotic factors. In this study, we investigated the in vitro effects of fucoidan on collagen synthesis, ${\alpha}-smooth$ muscle actin (${\alpha}-SMA$) expression, and interleukin (IL)-6 production in $TGF-{\beta}$-stimulated human pulmonary fibroblasts. The expression of type I collagen and ${\alpha}-SMA$ was detected by Western blot, and the production of IL-6 by enzyme-linked immunosorbent assay. $TGF-{\beta}1$ treatment of pulmonary fibroblasts enhanced the expression of ${\alpha}-SMA$, type I collagen, and IL-6 whereas these effects were inhibited in cells pretreated with fucoidan. The activation of Smad2/3, p38 mitogen-activated protein kinases (MAPKs), and Akt was also inhibited in fucoidan-pretreated, $TGF-{\beta}1-stimulated$ human pulmonary fibroblasts. These data demonstrate the anti-fibrotic potential of fucoidan in $TGF-{\beta}-induced$ human pulmonary fibroblasts, via the inhibition of Smad2/3, p38 MAPKs, and Akt phosphorylation. Our results suggest the therapeutic potential of fucoidan in the prevention or treatment of pulmonary fibrosis.

Diagnostic Approaches for Idiopathic Pulmonary Fibrosis

  • Jae Ha Lee;Jin Woo Song
    • Tuberculosis and Respiratory Diseases
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    • v.87 no.1
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    • pp.40-51
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    • 2024
  • Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive, fibrosing interstitial pneumonia with a very poor prognosis. Accurate diagnosis of IPF is essential for good outcomes but remains a major medical challenge due to variability in clinical presentation and the shortcomings of existing diagnostic tests. Medical history collection is the first and most important step in the IPF diagnosis process; the clinical probability of IPF is high if the suspected patient is 60 years or older, male, and has a history of cigarette smoking. Systemic assessment for connective tissue disease is essential in the initial evaluation of patients with suspected IPF to identify potential causes of interstitial lung disease (ILD). Radiologic examination using high-resolution computed tomography plays a pivotal role in the evaluation of patients with ILD, and prone and expiratory computed tomography images can be considered. If additional tests such as surgical lung biopsy or transbronchial lung cryobiopsy are needed, transbronchial lung cryobiopsy should be considered as an alternative to surgical lung biopsy in medical centers with experience performing this procedure. Diagnosis through multidisciplinary discussion (MDD) is strongly recommended as MDD has become the cornerstone for diagnosis of IPF, and the scope of MDD has expanded to monitoring of disease progression and suggestion of appropriate treatment options.

Pulmonary Sarcoidosis (폐 유육종증 1례 보고)

  • 백효채
    • Journal of Chest Surgery
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    • v.28 no.10
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    • pp.928-931
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    • 1995
  • Sarcoidosis is a granulomatous disease of unknown etiology. It frequently involves thoracic lymph node and pulmonary parenchyme characterized by non-caseating granulomas and varying degrees of fibrosis. We have experienced a pulmonary sarcoidosis in the left lower lobe of the lung in a 40 year old man who underwent left lower lobectomy by video-assisted thoracic surgery.

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CHANGES IN SUBPOPULATION OF BRONCHOALVEOLAR LAVAGE FLUID IN THE PULMONARY FIBROSIS INDUCED BY BLEOMYCIN OR PEPLOMYCIN

  • Kim, Dae-Joong
    • Toxicological Research
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    • v.9 no.2
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    • pp.241-251
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    • 1993
  • Present studies were carried out in order to estabilish the bronchoalveolar lavage method and to examine the response of bleomycin and peplomycin on the total cell number and the subpoulations of bronchoalveolar lavage fluid. A total of 24 male F344 rats, weighing 300-350 mg, were divided into 3 groups. Animals recelved either belomycin (BLM` 0.75 mg/0.2 ml/rat), peplomycin (PLM` 0.25mg/0.2ml/rat) for groups 2 and 3 or an equal volume of sterile saline lacking drugs for controls (group 1).

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The Role of HRCT in the Follow-Up Evaluation of Diffuse Interstitial Pulmonary Fibrosis (미만성 간질성 폐섬유증의 경과 관찰에서의 고해상도 CT(HRCT)의 의의)

  • Kim, Hong-Kyu;Kwak, Seung-Min;Song, Koun-Sik;Lim, Chae-Man;Koh, Youn-Suck;Kim, Woo-Sung;Kim, Dong-Soon;Kim, Won-Dong
    • Tuberculosis and Respiratory Diseases
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    • v.41 no.6
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    • pp.597-603
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    • 1994
  • Background: Because of the power of HRCT to detect the minute changes in lung parenchyme and the advantage of noninvasiveness, it may be the ideal method of follow-up evaluation of the patients with diffuse interstitial pulmonary fibrosis. So the aim of the study is to find out whether the change of HRCT during the course of the disease can represent the change in symptom and pulmonary function test. Method: Thirteen patients with diagnosis of diffuse interstitial pulmonary fibrosis who had more than two times of HRCT during the course of disease were studied. The extent of the disease in HRCT was measured at three levels using image analyzer. The ralationship between the percent change in the disease extent in HRCT, symptom score, and pulmonary function tests were analysed. Results: The change of disease extent in HRCT has good correlation with the change in dyspnea and diffusion capacity(r=0.716, p=0.0012). But there was no correlation between the change of HRCT and FVC or TLC. Also significant inverse correlation was noted between the change in dyspnea and diffusion capacity(r=-0.707, p=0.0047). Conclusion: These data suggest that HRCT may be a good method of follow-up in diffuse interstitial pulmonary fibrosis.

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Pulmonary Fibrosis caused by Asbestos Fibers in the Respiratory Airway

  • Jung, Ji-Woo;Kim, Eung-Sam
    • Biomedical Science Letters
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    • v.27 no.3
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    • pp.111-120
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    • 2021
  • Asbestos products had been widely used until 2007 in Korea since the 1930s. A total ban on their production and applications has been imposed because of the toxic effect of asbestos fibers on the human health. The inhaled asbestos fibers increase reactive oxygen species and inflammatory reactions in the respiratory airway including the alveolar sac, resulting in DNA damages and secretion of several inflammatory cytokines or chemokines. These paracrine communications promote the proliferation of fibroblasts and the synthesis of collagen fibers, thereby depositing them into the extracellular matrix at the interstitial space of alveoli. The fibrotic tissue hindered the gas exchange in the alveolus. This reviews describes not only the cytotoxic effects of asbestos fibers with different physical or chemical characteristics but also the interaction of cells that make up the respiratory airway to understand the molecular or cellular mechanisms of asbestos fiber-induced toxicity. In addition, we propose a pulmonary toxicity research technique based on the mini-lung that can mimic human respiratory system as an alternative to overcome the limitations of the conventional risk assessment of asbestos fibers.

Adverse Effects of Air Pollution on Pulmonary Diseases

  • Ko, Ui Won;Kyung, Sun Young
    • Tuberculosis and Respiratory Diseases
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    • v.85 no.4
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    • pp.313-319
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    • 2022
  • Environmental exposure to air pollution is known to have adverse effects on various organs. Air pollution has greater effects on the pulmonary system as the lungs are directly exposed to contaminants in the air. Here, we review the associations of air pollution with the development, morbidity, and mortality of pulmonary diseases. Short-and long-term exposure to air pollution have been shown to increase mortality risk even at concentrations below the current national guidelines. Ambient air pollution has been shown to be associated with lung cancer. Particularly long-term exposure to particulate matter with a diameter <2.5 ㎛ (PM2.5) has been reported to be associated with lung cancer even at low concentrations. In addition, exposure to air pollution has been shown to increase the incidence risk of chronic obstructive pulmonary disease (COPD) and has been correlated with exacerbation and mortality of COPD. Air pollution has also been linked to exacerbation, mortality, and development of asthma. Exposure to nitrogen dioxide (NO2) has been demonstrated to be related to increased mortality in patients with idiopathic pulmonary fibrosis. Additionally, air pollution increases the incidence of infectious diseases, such as pneumonia, bronchitis, and tuberculosis. Furthermore, emerging evidence supports a link between air pollution and coronavirus disease 2019 transmission, susceptibility, severity and mortality. In conclusion, the stringency of air quality guidelines should be increased and further therapeutic trials are required in patients at high risk of adverse health effects of air pollution.