• Title/Summary/Keyword: Pulmonary Fibrosis

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Recent Advances in Idiopathic Pulmonary Fibrosis

  • Park, Sanghoon;Lee, Eun Joo
    • Tuberculosis and Respiratory Diseases
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    • v.74 no.1
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    • pp.1-6
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    • 2013
  • The concept on idiopathic pulmonary fibrosis (IPF) pathogenesis has progressed from chronic inflammation to aberrant wounding healing and even more to the current paradigms of a multifactorial and heterogeneous disease process. Despite the growth of clinical trials for IPF, most of the results, including N-acetylcysteine combination, warfarin, and bosentan, were disappointing. On the other hand, there have been a number of important developments; the foremost is the licensing of pirfenidone in Europe and Asia. In this article, we briefly review the recent knowledge of pathogenesis of IPF. We also summarize the recent clinical trials regarding the management of IPF.

The Effects of Peucedani Radix on the Bleomycin-Induced Lung Fibrosis (전호(前胡)가 Bleomycin에 의한 폐 섬유화에 미치는 영향)

  • Kim, Hyun-Ji;Lee, Hai-Ja;Park, Eun-Jung
    • The Journal of Pediatrics of Korean Medicine
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    • v.22 no.2
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    • pp.37-49
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    • 2008
  • Objectives : Idiopathic pulmonary fibrosis (IPF) is chronic fibrotic interstitial pneumonia and the pathogenesis is unknown. Peucedani Radix is well-known for the treatment of respiratory diseases and pulmonary hypertension. This study was to evaluate the effectiveness of Peucedani Radix on the bleomycin-induced lung fibrosis model (BLFM) in mouse. Methods : We induced lung fibrosis by intratracheal instillation of bleomycin in C57BL/6J. We compared two groups BLFM without Peucedani Radix (group I) and BLFM with Peucedani Radix (group II). We performed bronchoalveolar lavages (BAL) and obtained lung specimens from both group I and II on the 7th (A) and 21st (B) day, and also for the normal group. We compared with group I and II to find BAL by using ANOVA test and to find pathologic symptoms by using semiquantitative histological index (SHI). Results : In BAL, total cell counts, lymphocytes, and neutrophils was increased in both group I and II comparing with normal group. However, lymphocyte level was decreased more in group IIB than group IB. It was statistically significant. In microscopic findings, scores of SHI in normal group, group IB and IIB were 0.33, 4.47, and 1.96 each. Conclusions : Peucedani Radix might have inhibitory effect on lung fibrosis by reducing inflammatory cells in bleomycin-induced lung fibrosis model in mouse.

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Efficacy of flavanone as a treatment for pulmonary fibrosis (Flavanone의 폐섬유증 치료물질로의 유용성)

  • Hee Young Kim;Hyerin Jeong;Young Mee Kim;Moonjae Cho
    • Journal of Applied Biological Chemistry
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    • v.65 no.4
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    • pp.357-365
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    • 2022
  • We examined the lung anti-fibrotic properties of flavanones and flavones, which are flavonoid compounds, in bleomycin- and TGF-β1-stimulated A549 cells. Taken together, treatment with Bleomycin and TGF-β1 increased intracellular ROS by increasing the expression of various NOX families in A549 cells; further, the increased ROS levels resulted in increased fibrosis markers and induced pulmonary fibrosis. Flavonoid treatment has been demonstrated to alleviate or inhibit pulmonary fibrosis by modulating Smad-dependent and -non-dependent TGF-β mechanisms by modulating intracellular NOX expression.

Giant Bulla with Pulmonary Fibrosis Caused by Gramoxon Toxicity -A case report- (그라목손 중독에 의한 폐섬유화와 동반된 거대 폐기포 - 1례 보고 -)

  • 정진악;금동윤;이재원
    • Journal of Chest Surgery
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    • v.33 no.9
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    • pp.773-776
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    • 2000
  • Accidental or suicidal fatalities of paraquat(Gramoxon) poisong are occasionally seen in the emergency room or intensive care unit in this country. In most cases, respiratory symptoms and eventual death by respiratory distress occur within several days. The most striking pathologic change is fibrosis of the lung due to widespread proliferation of fibroblastic cell. We experience a 21-year-old woman with huge bulla on left lung and diffuse fibrosis in other site, who ingested paraquat 10 months ago. After thoracoscopic removal of bulla, the patient survive without progression of pulmonary complication till now.

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Interstitial Lung Disease and Diffuse Alveolar Hemorrhage, the Two Key Pulmonary Manifestations in Microscopic Polyangiitis

  • Kim, Min Jung;Shin, Kichul
    • Tuberculosis and Respiratory Diseases
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    • v.84 no.4
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    • pp.255-262
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    • 2021
  • Microscopic polyangiitis (MPA) is an antineutrophil cytoplasmic antibody (ANCA)-associated necrotizing vasculitis, which mainly affects small vessels in various organs, especially the lungs. The two key pulmonary manifestations, interstitial lung disease (ILD) and diffuse alveolar hemorrhage (DAH), increase the morbidity and death rate of patients with MPA. ILD is more common in MPA than in other ANCA-associated vasculitis subsets and is primarily associated with myeloperoxidase-ANCA. Unlike alveolar hemorrhage due to pulmonary capillaritis, ILD can initially manifest as isolated pulmonary fibrosis. Of note, its most frequent radiographic pattern is the usual interstitial pneumonia pattern, similar to the characteristic pattern seen in idiopathic pulmonary fibrosis. In this review we present the pathogenesis, clinical manifestations, and radiographic and histopathologic features of ILD and DAH in MPA. We also briefly summarize the outcome and therapeutic options for the two conditions.

Role of Growth Factors and Cytokines on Bleomycin Induced Pulmonary Fibrosis (Bleomycin 유도 폐 섬유화에 있어서 성장인자 및 Cytokine의 역할)

  • Lee, Yong-Hee;Jung, Soon-Hee;Ahn, Chul-Min;Kim, Sung-Kyu;Cho, Sang-Ho
    • Tuberculosis and Respiratory Diseases
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    • v.44 no.4
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    • pp.871-888
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    • 1997
  • Background : It is now thought that the earliest manifestation of idiopathic pulmonary fibrosis is alveolitis, that is, an accumulation of inflammatory and immune effector cells within alveolar walls and spaces. Inflammatory cells including alveolar macrophages and resident normal pulmonary tissue cells participate through the release of many variable mediators such as inflammatory growth factors and cytokines, which contribute to tissue damage and finally cause chronic pulmonary inflammation and fibrosis. This study was performed to investigate the source and distribution pattern of transforming growth factor-${\beta}_1$(TGF-${\beta}_1$), platelet derived growth factor(PDGF), basic fibroblast growth factor(bFGF), interleukin 1(IL-1), interleukin 6(IL-6), tumor necrosis factor-$\alpha$ (TNF-$\alpha$) and the role of these mediators on bleomycin(BLM)-induced pulmonary injury and fibrosis in rats. Method : Wistar rats were divided into three groups(control group, BLM treated group, BLM and vitamine E treated group). Animals were sacrificed periodically at 1, 2, 3, 4, 5, 7, 14, 21, 28 days after saline or BLM administration. The effects were compared to the results of bronchoalveolar lavage fluid analysis, light microscopic findings, immunohistochemical stains for six different mediators(TGF-${\beta}_1$, PDGF, bFGF, IL-1, IL-6 and TNF-$\alpha$) and mRNA in situ hybridization for TGF-${\beta}_1$. Results : IL-1 and IL-6 are maximally expressed at postbleomycin 1~7th day which are mainly produced by neutrophils and bronchiolar epithelium. It is thought that they induce recruitment of inflammatory cells at the injury site. The expression of IL-1 and IL-6 at the bronchiolar epithelium within 7th day is an indirect evidence of contribution of bronchiolar epithelial cells to promote and maintain the inflammatory and immune responses adjacent to the airways. TNF-$\alpha$ is mainly produced by neutrophils and bronchiolar epithelial cells during 1~5th day, alveolar macrophages during 7~28th day. At the earlier period, TNF-$\alpha$ causes recruitment of inflammatory cells at the injury site and later stimulates pulmonary fibrosis. The main secreting cells of TGF-${\beta}_1$ are alveolar macrophages and bronchiolar epithelium and the target is pulmonary fibroblasts and extracellular matrix. TGF-${\beta}_1$ and PDGF stimulate proliferation of pulmonary fibroblasts and TGF-${\beta}_1$ and bFGF incite the fibroblasts to produce extracellular matrix. The vitamine E and BLM treated group shows few positive cells(p<0.05). Conclusion : After endothelial and epithelial injury, the neutrophils and bronchiolar epithelium secrete IL-1, IL-6, TNF-$\alpha$ which induce infiltration of many neutrophils. It is thought that variable enzymes and $O_2$ radicals released by these neutrophils cause destruction of normal lung architecture and progression of pulmonary fibrosis. At the 7~28th day, TGF-${\beta}_1$, PDGF, bFGF, TNF-$\alpha$ secreted by alveolar macrophages sting pulmonary fibroblasts into proliferating with increased production of extracellular matrix and finally, they make progression of pulmonary fibrosis. TNF-$\alpha$ compares quite important with TGF-${\beta}_1$ to cause pulmonary fibrosis. Vitamine E seems to decrease the extent of BLM induced pulmonary fibrosis.

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Expression of Intercellular Adhesion Molecule-1 in Human Idiopathic Pulmonary Fibrosis (인체 특발성 폐섬유증에서 Intercellular Adhesion Molecule-1의 발현에 관한 연구)

  • Park, Sung-Soo;Shin, Dong-Ho;Kim, Tae-Wha;Lee, Dong-Hoo;Lee, Jung-Hee;Lee, Jung-Dal
    • Tuberculosis and Respiratory Diseases
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    • v.40 no.2
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    • pp.185-191
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    • 1993
  • Background: Intercellular adhesion molecule-1(ICAM-1) is a 90 kD surface glycoprotein, associated with ${\alpha}_L{\beta}_2$ and ${\alpha}_M{\beta}_2$ subunit of integrins, that serve as cell-cell and cell-substratum adhesion molecules and help regulate cellular morphology, differentiation, and proliferation. The adhesion molecules likely play important roles in maintaining the normal structure and function of the lung. ICAM-1 system among many cell adhesion molecules is importantly issuing in the pathogenesis of idiopathic pulmonary fibrosis. Methods : By using $IgG_1$ monoclonal antibody for ICAM-1, we investigated immunohistochemically the expression of ICAM-1 in the formalin-fixed, paraffin-embedded tissue sections of the 3 normal cases and 6 pieces of tissues taken 3 cases with idiopathic pulmonary fibrosis. Results : In the 3 normal cases, the expressions of ICAM-1 were not discernible. Up-regulation of the ICAM-1 expression was showed in the interstitial fibroblast cells of alveolar septa in 5 pieces and proliferated alveolar pneumocytes in 1 piece among 6 pieces of tissues taken 3 cases with idiopathic pulmonary fibrosis. Conclusion : It was concluded from these findings that up-regulation of the ICAM-1 expression may be related to pathogenesis of idiopathic 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.