• Title/Summary/Keyword: Pulmonary interstitial fibrosis

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The Effects of Astragali Radix on Bleomycin-induced Lung Fibrosis (황기가 C57BL/6J 생쥐의 Bleomycin유발 폐섬유화에 미치는 영향)

  • 이경희;정희재;정승기;윤유식;이형구
    • The Journal of Korean Medicine
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    • v.25 no.2
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    • pp.41-50
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    • 2004
  • Backgrounds & Objectives: Many acute and chronic lung disorders with variable degrees of pulmonary inflammation and fibrosis are collectively referred to as interstitial lung diseases. Idiopathic pulmonary fibrosis (IPF) is one of several idiopathic interstitial pneumonias with the pathogenesis unclear. Astragali Radix is known to inhibit the Th2 immune response. The effects of Astragali Radix on bleomycin-induced lung fibrosis were evaluated. Materials and Methods: Astragali Radix extract was daily given to the normal rats, control (bleomycin) and treated (bleomycin and Astragali Radix extract, 24.0 mg/10g body weight) rats for 14 days. After 14 days, we observed the change of total leukocyte count and percentage, IFN-gamma and IL-4 in BALF (Bronchoalveolar lavage fluid), and of semiquantitative histological index (SHI). Results: Compared to the control group, Astragali Radix decreased total leukocyte count (p<0.05), lymphocyte (p<0.05), neutrophil (no significance) percentage, SHI (p<0.05), IFN-gamma and IL-4 (p<0.05). Otherwise, macrophage percentage was increased (p<0.01). Conclusion: This study showed that Astragali Radix reduced the incidence of inflammatory cells and cytokines and prevented the fibrosis of tissue in bleomycin-induced lung fibrosis rats.

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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.

Interstitial Lung Abnormality in Asian Population

  • Gong Yong Jin
    • Tuberculosis and Respiratory Diseases
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    • v.87 no.2
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    • pp.134-144
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    • 2024
  • Interstitial lung abnormalities (ILAs) are radiologic abnormalities found incidentally on chest computed tomography (CT) that can be show a wide range of diseases, from subclinical lung fibrosis to early pulmonary fibrosis including definitive usual interstitial pneumonia. To clear up confusion about ILA, the Fleischner society published a position paper on the definition, clinical symptoms, increased mortality, radiologic progression, and management of ILAs based on several Western cohorts and articles. Recently, studies on long-term outcome, risk factors, and quantification of ILA to address the confusion have been published in Asia. The incidence of ILA was 7% to 10% for Westerners, while the prevalence of ILA was about 4% for Asians. ILA is closely related to various respiratory symptoms or increased rate of treatment-related complication in lung cancer. There is little difference between Westerners and Asians regarding the clinical importance of ILA. Although the role of quantitative CT as a screening tool for ILA requires further validation and standardized imaging protocols, using a threshold of 5% in at least one zone demonstrated 67.6% sensitivity, 93.3% specificity, and 90.5% accuracy, and a 1.8% area threshold showed 100% sensitivity and 99% specificity in South Korea. Based on the position paper released by the Fleischner society, I would like to report how much ILA occurs in the Asian population, what the prognosis is, and review what management strategies should be pursued in the future.

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 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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A Case of Giant Cell Interstitial Pneumonia (거대세포 간절성 폐렴(Giant Cell Interstitial Pneumonia) 1예)

  • Kang, Kyeong-Woo;Park, Sang-Joon;Suh, Gee-Young;Han, Joung-Ho;Chung, Man-Pyo;Kim, Ho-Joong;Kwon, O-Jung;Rhee, Chong-H.;Choi, Jae-Wook
    • Tuberculosis and Respiratory Diseases
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    • v.48 no.2
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    • pp.260-267
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    • 2000
  • Giant cell interstitial pneumonia. a synonym for hard metal pneumoconiosis, is a unique form of pulmonary fibrosis resulting from an exposure to hard metal dust. A case of biopsy-proved giant cell interstitial pneumonia in the absence of appropriate history of exposure to hard metal dust is reported. The patient presented with clinical features of chronic interstitial lung disease or idiopathic pulmonary fibrosis. He worked in a chemical laboratory at a fertilizer plant, where he had been exposed to various chemicals such as benzene and toluene. He denied having any other hobby in his house or job at work, which may have exposed him hard metal dust. High-resolution CT scan revealed multi-lobar distribution of ground glass opacity with peripheral and basal lung predominance. The retrieved fluid of bronchoalveolar lavage contained asbestos fiber and showed neutrotphil predominance. Surgical lung biopsy was performed for a definite diagnosis. Lung specimen showed alveolar infiltration of numerous multinucleated giant cells with mild interstitial fibrosis. Upon detailed examination of the lung tissue, one asbestos body was found. An analysis for mineral contents in lung tissue was performed. Compared with the control specimen, the amount of cobalt and several hard metal components in the lung tissue of this patient was ten times higher. We speculated that the inconsistency between occupational history and the findings of pathologic and mineralogical analyses could be explained by the difference in individual immunologic reactivity to hard metal dust despite the relatively small amount of unrecognized environmental exposure(ED: It's hard to understand what this phrase is trying to say).

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Progressive Pulmonary Fibrosis: Where Are We Now?

  • Hyung Koo Kang;Jin Woo Song
    • Tuberculosis and Respiratory Diseases
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    • v.87 no.2
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    • pp.123-133
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    • 2024
  • Interstitial lung diseases (ILDs) are a diverse collection of lung disorders sharing similar features, such as inflammation and fibrosis. The diagnosis and management of ILD require a multidisciplinary approach using clinical, radiological, and pathological evaluation. Progressive pulmonary fibrosis (PPF) is a distinct form of progressive and fibrotic disease, occurring in ILD cases other than in idiopathic pulmonary fibrosis (IPF). It is defined based on clinical symptoms, lung function, and chest imaging, regardless of the underlying condition. The progression to PPF must be monitored through a combination of pulmonary function tests (forced vital capacity [FVC] and diffusing capacity of the lung for carbon monoxide), an assessment of symptoms, and computed tomography scans, with regular follow-up. Although the precise mechanisms of PPF remain unclear, there is evidence of shared pathogenetic mechanisms with IPF, contributing to similar disease behavior and worse prognosis compared to non-PPF ILD. Pharmacological treatment of PPF includes immunomodulatory agents to reduce inflammation and the use of antifibrotics to target progressive fibrosis. Nintedanib, a known antifibrotic agent, was found to be effective in slowing IPF progression and reducing the annual rate of decline in FVC among patients with PPF compared to placebos. Nonpharmacological treatment, including pulmonary rehabilitation, supplemental oxygen therapy, and vaccination, also play important roles in the management of PPF, leading to comprehensive care for patients with ILD. Although there is currently no cure for PPF, there are treatments that can help slow the progression of the disease and improve quality of life.

Korean Guidelines for Diagnosis and Management of Interstitial Lung Diseases: Part 2. Idiopathic Pulmonary Fibrosis

  • Lee, Sang Hoon;Yeo, Yoomi;Kim, Tae-Hyung;Lee, Hong Lyeol;Lee, Jin Hwa;Park, Yong Bum;Park, Jong Sun;Kim, Yee Hyung;Song, Jin Woo;Jhun, Byung Woo;Kim, Hyun Jung;Park, Jinkyeong;Uh, Soo-Taek;Kim, Young Whan;Kim, Dong Soon;Park, Moo Suk;Korean Interstitial Lung Diseases Study Group
    • Tuberculosis and Respiratory Diseases
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    • v.82 no.2
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    • pp.102-117
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    • 2019
  • Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive fibrosing interstitial pneumonia, which presents with a progressive worsening dyspnea, and thus a poor outcome. The members of the Korean Academy of Tuberculosis and Respiratory Diseases as well as the participating members of the Korea Interstitial Lung Disease Study Group drafted this clinical practice guideline for IPF management. This guideline includes a wide range of topics, including the epidemiology, pathogenesis, risk factors, clinical features, diagnosis, treatment, prognosis, and acute exacerbation of IPF in Korea. Additionally, we suggested the PICO for the use of pirfenidone and nintendanib and for lung transplantation for the treatment of patients with IPF through a systemic literature review using experts' help in conducting a meta-analysis. We recommend this guideline to physicians, other health care professionals, and government personnel in Korea, to facilitate the treatment of patients with IPF.

Korean Guidelines for Diagnosis and Management of Interstitial Lung Diseases: Part 3. Idiopathic Nonspecific Interstitial Pneumonia

  • Lee, Jongmin;Kim, Yong Hyun;Kang, Ji Young;Jegal, Yangjin;Park, So Young;Korean Interstitial Lung Diseases Study Group
    • Tuberculosis and Respiratory Diseases
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    • v.82 no.4
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    • pp.277-284
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    • 2019
  • Idiopathic nonspecific interstitial pneumonia (NSIP) is one of the varieties of idiopathic interstitial pneumonias. Diagnosis of idiopathic NSIP can be done via multidisciplinary approach in which the clinical, radiologic, and pathologic findings were discussed together and exclude other causes. Clinical manifestations include subacute or chronic dyspnea and cough that last an average of 6 months, most of which occur in non-smoking, middle-aged women. The common findings in thoracic high-resolution computed tomography in NSIP are bilateral reticular opacities, traction bronchiectasis, reduced volume of the lobes, and ground-glass opacity in the lower lungs. These lesions can involve diffuse bilateral lungs or subpleural area. Unlike usual interstitial pneumonia, honeycombing is sparse or absent. Pathology shows diffuse interstitial inflammation and fibrosis which are temporally homogeneous, namely NSIP pattern. Idiopathic NSIP is usually treated with steroid only or combination with immunosuppressive agents such as azathioprine, cyclophosphamide, cyclosporine, and mycophenolate mofetil. Prognosis of idiopathic NSIP is better than idiopathic pulmonary fibrosis. Many studies have reported a 5-year survival rate of more than 70%.