• 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.

• 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.

• Journal of Yeungnam Medical Science
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• v.36 no.1
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• pp.8-15
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• 2019

Connective tissue diseases (CTDs) can affect all compartments of the lungs, including airways, alveoli, interstitium, vessels, and pleura. CTD-associated lung diseases (CTD-LDs) may present as diffuse lung disease or as focal lesions, and there is significant heterogeneity between the individual CTDs in their clinical and pathological manifestations. CTD-LDs may presage the clinical diagnosis a primary CTD, or it may develop in the context of an established CTD diagnosis. CTD-LDs reveal acute, chronic or mixed pattern of lung and pleural manifestations. Histopathological findings of diverse morphological changes can be present in CTD-LDs airway lesions (chronic bronchitis/bronchiolitis, follicular bronchiolitis, etc.), interstitial lung diseases (nonspecific interstitial pneumonia/fibrosis, usual interstitial pneumonia, lymphocytic interstitial pneumonia, diffuse alveolar damage, and organizing pneumonia), pleural changes (acute fibrinous or chronic fibrous pleuritis), and vascular changes (vasculitis, capillaritis, pulmonary hemorrhage, etc.). CTD patients can be exposed to various infectious diseases when taking immunosuppressive drugs. Histopathological patterns of CTD-LDs are generally nonspecific, and other diseases that can cause similar lesions in the lungs must be considered before the diagnosis of CTD-LDs. A multidisciplinary team involving pathologists, clinicians, and radiologists can adequately make a proper diagnosis of CTD-LDs.

• Journal of Chest Surgery
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• v.24 no.9
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• pp.903-906
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• 1991

Retrospective review of 26 patients undergoing open lung biopsy at the Yonsei University during 10 years period was conducted to evaluate open lung biopsy for DILD. From January 1980 to August 1990, open lung biopsy was performed in 26 patients through a limited thoracotomy incision[a limited anterior or a posterolateral thoracotomy]. Open lung biopsy was indicated for diffuse interstitial pulmonary diseases undiagnosed by indirect clinical and radiological diagnostic methods. The types of incision were limited anterior[11] and limited posterolateral[15]. Preoperative evaluation of the lung disease included sputum culture[26], sputum cytology [19], bronchoscopy[9] and TBLB[7]. In 23 patients the histologic appearances after open lung biopsy were sufficiently specific histologic pictures to confirm diagnosis. The results of the biopsies changed usual therapeutic plan in 17 patients among them. The complications were resp. insufficiency[3], pulmonary ed6ma[3], sepsis[2], and others[3] in 6 patients. Diagnosis from the open lung biopsy was included respiratory pneumonia[7], fibrosis[7], infection[5], malignancy[2], others[5]. 4 patients died of respiratory insufficiency. The causes of the other three death were not due to direct result of the biopsy itself. Open lung biopsy in the patient with a diffuse infiltrative lung disease is an one of the accurate diagnostic method and frequently leads to change of the therapeutic plans. So we conclude that open lung biopsy remains our diagnostic method of choice in diffuse infiltrative lung disease undetermined etiology.

• 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.

• Journal of Chest Surgery
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• v.28 no.1
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• pp.53-58
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• 1995

Open lung biopsy still has important roles for the marking of diagnosis of diffuse infiltrative lung disease even though transbronchial bronchoscopic lung biopsy and percutaneous needle aspiration biopsy gain popularity nowadays. This is clinical retrospective review of the 56 patients with diffuse infiltrative lung disease undergoing open lung biopsy by minithoracotomy from 1984 to Dec. 1992 in the Department of Thoracic & Cardiovascular Surgery of Catholic University Medical College. 27 men and 29 women, aged 17 to 73 year [mean 49 year , were enrolled & divided into 2 groups;Group A consisted of patients with immunocompromised state [n=19 , Group B patients with non-immunocompromised state[n=38 . Pathologic diagnosis was made in 54 cases[96.4% of these two groups and as follows: infectious; 12 patients[21.4% , Neoplastic; 10 patients[17.9% , granulomatous; 4 patients[7.1% , interstitial pneumonia; 12 patients[21.4% , Pulmonary fibrosis; 8 patients[14.3% , others; 3 patients[5.4% , nonspecific; 5 patients[8.9% , and undetermined; 2 patients[3.6% . Therapeutic plans were changed in 39 patients[69.6% after taking of tissue diagnosis by open lung biopsy. Group B has higher incidence of infectious diseases and change of therapeutic plan than the Group A. The postoperative complications developed in 8 cases[14.3% ,and there is no difference of incidence between the 2 groups. 4 patients belongs to group A, died of respiratory distress syndrome [2 and sepsis [2 which were not related with open lung biopsy procedure. In conclusion, open lung biopsy is a reliable method to obtain a diagnosis in diffuse pulmonary infiltrates and can be performed safely, even in acutely ill, immunosuppressed patients.

• Tuberculosis and Respiratory Diseases
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• v.77 no.2
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• pp.85-89
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• 2014

Intrathoracic involvement of immunoglobulin G4 (IgG4)-related disease has recently been reported. However, a subset of the disease presenting as interstitial lung disease is rare. Here, we report a case of a 35-year-old man with IgG4-related lung disease with manifestations similar to those of interstitial lung disease. Chest computed tomography showed diffuse ground glass opacities and rapidly progressive pleural and subpleural fibrosis in both upper lobes. Histological findings showed diffuse interstitial lymphoplasmacytic infiltration with an increased number of IgG4-positive plasma cells. Serum levels of IgG and IgG4 were also increased. The patient was diagnosed with IgG4-related lung disease, treated with anti-inflammatory agents, and showed improvement. Lung involvement of IgG4-related disease can present as interstitial lung disease and, therefore, should be differentiated when evaluating interstitial lung disease.

• Toxicological Research
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• v.26 no.3
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• pp.217-222
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• 2010

Many studies have reported that bleomycin, anti-cancer drug, induces pulmonary fibrosis as a side effect. However, few investigations have focused on the dose-response effects of bleomycin on pulmonary fibrosis. Therefore, in the present study, we investigated the effects of different doses of bleomycin in male mice. ICR mice were given 3 consecutive doses of bleomycin: 1, 2, or 4 mg/kg in bleomycin-treated (BT) groups and saline only in vehicle control (VC) groups. The animals were sacrificed at 7 and 24 days postinstillation. The severity of pulmonary fibrosis was evaluated according to inflammatory cell count and lactate dehydrogenase (LDH) activity in the broncho alveolar lavage fluid (BALF), and lung tissues were histologically evaluated after hematoxylin and eosin (H&E), and Masson's trichrome staining. BT groups exhibited changed cellular profiles in BAL fluid compared to the VC group, which had an increased number of total cells, neutrophils, and lymphocytes and a modest increase in the number of macrophages at 7 days post-bleomycin instillation. Moreover, BT groups showed a dose-dependent increase in LDH levels and inflammatory cell counts. However, at 24 days after treatment, collagen deposition, interstitial thickening, and granulomatous lesions were observed in the alveolar spaces in addition to a decrease in inflammatory cells. These results indicate that pulmonary fibrosis induced by 4 mg/kg bleomycin was more severe than that induced by 1 or 2 mg/kg. These data will be utilized in experimental animal models and as basic data to evaluate therapeutic candidates through non-invasive monitoring using the pulmonary fibrosis mouse model established in this study.

• Journal of Chest Surgery
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• v.49 no.6
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• pp.461-464
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• 2016

Coronary artery disease has historically been a contraindication to lung transplantation. We report a successful combined bilateral lung transplantation and off-pump coronary artery bypass in a 62-year-old man. The patient had a progressive decline in lung function due to idiopathic pulmonary fibrosis and a history of severe occlusive coronary artery disease.

• Korean Journal of Radiology
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• v.22 no.3
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• pp.454-463
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• 2021

Interstitial lung abnormalities (ILAs) are radiologic abnormalities found incidentally on chest CT that are potentially related to interstitial lung diseases. Several articles have reported that ILAs are associated with increased mortality, and they can show radiologic progression. With the increased recognition of ILAs on CT, the role of radiologists in reporting them is critical. This review aims to discuss the clinical significance and radiologic characteristics of ILAs to facilitate and enhance their management.