Annual Change in Pulmonary Function and Clinical Characteristics of Combined Pulmonary Fibrosis and Emphysema and Idiopathic Pulmonary Fibrosis: Over a 3-Year Follow-up

( Yu Jin Kim ),( Seong Hyun Shin ),( Jeong Woong Park ),( Sun Young Kyung ),( Shin Myung Kang ),( Sang Pyo Lee ),( Yon Mi Sung ),( Yoon Kyung Kim ),( Sung Hwan Jeong ) 대한결핵 및 호흡기학회 2014 Tuberculosis and Respiratory Diseases Vol.77 No.1

Background: Combined pulmonary fibrosis and emphysema (CPFE) have different pulmonary function tests (PFTs) and outcomes than idiopathic pulmonary fibrosis (IPF). The intention of this study was to identify unknown differences between CPFE and IPF by a retrospective comparison of clinical data including baseline and annual changes in pulmonary function, comorbidities, laboratory findings, clinical characteristics and cause of hospitalization. Methods: This study retrospectively enrolled patients with CPFE and IPF who had undergone PFTs once or several times per year during a follow-up period of three years. Baseline clinical characteristics and the annual changes in the pulmonary function during the follow-up period were compared between 26 with CPFE and 42 patients with IPF. Results: The baseline ratio of forced expiratory volume in one second to forced vital capacity (FEV1/FVC%) in patients with CPFE was lower than that in patients with IPF (78.6±1.7 vs. 82.9±1.1, p=0.041). The annual decrease in FEV1/FVC in the CPFE was significantly higher than in the IPF. The annual decreases in diffusion capacity of carbon monoxide and FVC showed no significant differences between the two groups. The symptom durations of cough and sputum were in the CPFE significantly lower than in the IPF. The serum erythrocyte sedimentation rate level at the acute stage was significantly higher than in the IPF. There were no significant differences in the hospitalization rate and pneumonia was the most common cause of hospitalization in both study groups. Conclusion: The annual decrease of FEV1/FVC was in patients with CPFE significantly higher than in the patients with IPF.

Toxlcology : Two Successfully Treated Cases of Pulmonary Fibrosis Due to Paraquat Poisoning

( Woong Ki Lee ),( Choong Sil Seong ),( Byung Sun Kim ),( Hye Mi Choi ),( Hyeuk Soo Lee ),( Hyun Ju Yoon ),( Jeong Gwan Kim ),( Mi Sook Lee ),( Kwang Young Lee ),( In O Sun ) 대한응급의학회 2014 대한응급의학회지 Vol.25 No.4

Paraquat poisoning can cause severe multiple organ failure involving the kidneys, liver, lungs, adrenals, and central nervous system. The toxic effect of paraquat on the lung manifests as pulmonary edema, hypoxia, respiratory failure, and pulmonary fibrosis. However, optimal guidelines for treatment of lung fibrosis following paraquat ingestion are not available. We experienced two cases, a 45-year-old Korean male and a 66-year-old Korean male, who visited the emergency center because of paraquat poisoning. They initially received methylprednisolone pulse therapy and cyclophosphamide. Then they experienced pulmonary fibrosis approximately 10 days after admission during renal recovery. Although steroid pulse therapy with cyclophosphamide was reported to reduce mortality due to paraquat poisoning, the side effects of cyclophosphamide treatment were concerning in our patients, who had already received cyclophosphamide. Therefore, we decided to repeat steroid pulse therapy without cyclophosphamide. Fortunately, pulmonary fibrosis in these two patients resolved after repeated steroid pulse therapy. Thus, steroid pulse therapy alone could benefit patients with lung fibrosis, who have already received steroid and cyclophosphamide treatment. Herein, we report on two cases of pulmonary fibrosis due to paraquat poisoning that were treated successfully with repeated steroid pulse therapy.

Role of MicroRNAs in TGF-β Signaling Pathway-Mediated Pulmonary Fibrosis

MDPI 2017 INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES Vol.18 No.12

<P>Pulmonary fibrosis is the most common form of interstitial lung disease. The transforming growth factor-β (TGF-β) signaling pathway is extensively involved in the development of pulmonary fibrosis by inducing cell differentiation, migration, invasion, or hyperplastic changes. Accumulating evidence indicates that microRNAs (miRNAs) are dysregulated during the initiation of pulmonary fibrosis. miRNAs are small noncoding RNAs functioning as negative regulators of gene expression at the post-transcriptional level. A number of miRNAs have been reported to regulate the TGF-β signaling pathway and consequently affect the process of pulmonary fibrosis. A better understanding of the pro-fibrotic role of the TGF-β signaling pathway and relevant miRNA regulation will shed light on biomedical research of pulmonary fibrosis. This review summarizes the current knowledge of miRNAs regulating the TGF-β signaling pathway with relevance to pulmonary fibrosis.</P>

Genistein alleviates pulmonary fibrosis by inactivating lung fibroblasts

( Seung-hyun Kwon ),( Hyunju Chung ),( Jung-woo Seo ),( Hak Su Kim ) 생화학분자생물학회 2024 BMB Reports Vol.57 No.3

Pulmonary fibrosis is a serious lung disease that occurs predominantly in men. Genistein is an important natural soybeanderived 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 β-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. [BMB Reports 2024; 57(3): 143-148]

Annual Change in Pulmonary Function and Clinical Characteristics of Combined Pulmonary Fibrosis and Emphysema and Idiopathic Pulmonary Fibrosis: Over a 3-Year Follow-up

정성환,김유진,신성현,박정웅,경선영,강신명,이상표,성연미,김윤경 대한결핵및호흡기학회 2014 Tuberculosis and Respiratory Diseases Vol.77 No.1

Background: Combined pulmonary fibrosis and emphysema (CPFE) have different pulmonary function tests (PFTs) and outcomes than idiopathic pulmonary fibrosis (IPF). The intention of this study was to identify unknown differences between CPFE and IPF by a retrospective comparison of clinical data including baseline and annual changes in pulmonary function, comorbidities, laboratory findings, clinical characteristics and cause of hospitalization. Methods: This study retrospectively enrolled patients with CPFE and IPF who had undergone PFTs once or several times per year during a follow-up period of three years. Baseline clinical characteristics and the annual changes in the pulmonary function during the follow-up period were compared between 26 with CPFE and 42 patients with IPF. Results: The baseline ratio of forced expiratory volume in one second to forced vital capacity (FEV1/FVC%) in patients with CPFE was lower than that in patients with IPF (78.6±1.7 vs. 82.9±1.1, p=0.041). The annual decrease in FEV1/FVC in the CPFE was significantly higher than in the IPF. The annual decreases in diffusion capacity of carbon monoxide and FVC showed no significant differences between the two groups. The symptom durations of cough and sputum were in the CPFE significantly lower than in the IPF. The serum erythrocyte sedimentation rate level at the acute stage was significantly higher than in the IPF. There were no significant differences in the hospitalization rate and pneumonia was the most common cause of hospitalization in both study groups. Conclusion: The annual decrease of FEV1/FVC was in patients with CPFE significantly higher than in the patients with IPF.

Annual Change in Pulmonary Function and Clinical Characteristics of Combined Pulmonary Fibrosis and Emphysema and Idiopathic Pulmonary Fibrosis: Over a 3-Year Follow-up

Kim, Yu Jin,Shin, Seong Hyun,Park, Jeong-Woong,Kyung, Sun Young,Kang, Shin Myung,Lee, Sang-Pyo,Sung, Yon Mi,Kim, Yoon Kyung,Jeong, Sung Hwan The Korean Academy of Tuberculosis and Respiratory 2014 Tuberculosis and Respiratory Diseases Vol.77 No.1

Background: Combined pulmonary fibrosis and emphysema (CPFE) have different pulmonary function tests (PFTs) and outcomes than idiopathic pulmonary fibrosis (IPF). The intention of this study was to identify unknown differences between CPFE and IPF by a retrospective comparison of clinical data including baseline and annual changes in pulmonary function, comorbidities, laboratory findings, clinical characteristics and cause of hospitalization. Methods: This study retrospectively enrolled patients with CPFE and IPF who had undergone PFTs once or several times per year during a follow-up period of three years. Baseline clinical characteristics and the annual changes in the pulmonary function during the follow-up period were compared between 26 with CPFE and 42 patients with IPF. Results: The baseline ratio of forced expiratory volume in one second to forced vital capacity ($FEV_1$/FVC%) in patients with CPFE was lower than that in patients with IPF ($78.6{\pm}1.7$ vs. $82.9{\pm}1.1$, p=0.041). The annual decrease in $FEV_1$/FVC in the CPFE was significantly higher than in the IPF. The annual decreases in diffusion capacity of carbon monoxide and FVC showed no significant differences between the two groups. The symptom durations of cough and sputum were in the CPFE significantly lower than in the IPF. The serum erythrocyte sedimentation rate level at the acute stage was significantly higher than in the IPF. There were no significant differences in the hospitalization rate and pneumonia was the most common cause of hospitalization in both study groups. Conclusion: The annual decrease of $FEV_1$/FVC was in patients with CPFE significantly higher than in the patients with IPF.

폐섬유증의 약물치료

최원일 대한의사협회 2020 대한의사협회지 Vol.63 No.1

Idiopathic pulmonary fibrosis (IPF) is a condition that has been described as alveolar collapse and thickening, which correlate with dysregulated surfactant production and injury to type 2 alveolar cells. As resolution of chest computed tomography has improved, especially with the development of high-resolution computed tomography (HRCT), the diagnostic measures adopted for pulmonary fibrosis has gradually shifted from biopsy to HRCT. This shift towards HRCT has aided in diagnostic evaluation and detection of the therapeutic and adverse effects of drugs for pulmonary fibrosis. Further, after the endpoint was changed to forced vital capacity, significant improvements are being observed in clinical trial outcomes. Currently active clinical trials are replacing lung biopsy with HRCT. In 2014, pirfenidone and nintedanib gained approval for tandem use in patients with IPF. These drugs were found to not only reduce the progression of pulmonary fibrosis, but also the acute exacerbation and mortality associated with the condition. These drugs showed consistent benefits regardless of the severity of patients’ symptoms. Additionally, both nintedanib and pirfenidone were found to be effective in patients with advanced pulmonary fibrosis that was not classified as IPF. Nintedanib has been shown to reduce forced vital capacity in interstitial lung diseases associated with systemic sclerosis. In the next three to five years, many changes in treatment are expected, not only for IPF, but also for the entire spectrum of pulmonary fibrotic diseases. Pirfenidone and nintedanib are now considered standard treatments for IPF and few other fibrotic lung diseases. Clinicians treating patients with pulmonary fibrosis should keep themselves updated with the results of clinical trials that are currently underway.

Medical Imaging and Nuclear Molecular Imaging Probes for Pulmonary Fibrosis Diagnosis

Heesu Ahn,Yong Jin Lee 대한방사성의약품학회 2022 Journal of radiopharmaceuticals and molecular prob Vol.8 No.2

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.

The Effects of Chronic Intermittent Hypoxia in Bleomycin-Induced Lung Injury on Pulmonary Fibrosis via Regulating the NF-κB/Nrf2 Signaling Pathway

( Hyeon Hui Kang ),( In Kyoung Kim ),( Chang Dong Yeo ),( Sei Won Kim ),( Hea Yon Lee ),( Jeong Hyeon Im ),( Hee Young Kwon ),( Sang Haak Lee ) 대한결핵 및 호흡기학회 2020 Tuberculosis and Respiratory Diseases Vol.83 No.-

Background: Obstructive sleep apnea (OSA) is associated with pulmonary fibrosis. Chronic intermittent hypoxia (CIH) is considered to be a surrogate of OSA. However, its exact role in pulmonary fibrosis remains uncertain. Therefore, we investigated the mechanism underlying CIH-induced pulmonary fibrosis and the role of the anti-fibrotic agent in bleomycin (BLE) induced lung injury. Methods: Mice were divided into eight groups: the normoxia (NOR), CIH, NOR plus BLE, CIH plus BLE, NOR plus pirfenidone (PF), CIH plus PF, NOR plus BLE and PF, and CIH plus BLE and PF groups. BLE was administered intratracheally on day 14 following CIH or NOR exposure. Subsequently, the mice were exposed to CIH or NOR for an additional 4 weeks. PF was administered orally on day 5 after BLE instillation once daily for 3 weeks. Results: In the BLE-treated groups, CIH-induced more collagen deposition in lung tissues than NOR, and significantly increased hydroxyproline and transforming growth factor-β expression. The CIH and BLE-treated groups showed increased lung inflammation compared to NOR or CIH groups. Following CIH with BLE treatment, nuclear factor-κB (NF-κB) protein expression was significantly increased, whereas nuclear factor-erythroid-related factor 2 (Nrf2) and heme oxygenase-1 protein levels were decreased. After PF treatment, NF-κB and Kelch-like ECH-associated protein 1 expression were suppressed, and Nrf2 expression was increased. Conclusion: CIH accelerated lung fibrosis in BLE-induced lung injury in mice, potentially by regulating the NF-κB/Nrf2 signaling pathway. Our results implicate PF as a potential therapeutic agent for treating pulmonary fibrosis in individuals with OSA and idiopathic pulmonary fibrosis.

Post–Coronavirus Disease 2019 Pulmonary Fibrosis: Wait or Needs Intervention

어수택,Hee-Young Yoon 대한결핵및호흡기학회 2022 Tuberculosis and Respiratory Diseases Vol.85 No.4

Coronavirus disease 2019 (COVID-19) has become a major health burden worldwide, with over 450 million confirmed cases and 6 million deaths. Although the acute phase of COVID-19 management has been established, there is still a long way to go to evaluate the long-term clinical course or manage complications due to the relatively short outbreak of the virus. Pulmonary fibrosis is one of the most common respiratory complications associated with COVID-19. Scarring throughout the lungs after viral or bacterial pulmonary infection have been commonly observed, but the prevalence of post– COVID-19 pulmonary fibrosis is rapidly increasing. However, there is limited information available about post–COVID-19 pulmonary fibrosis, and there is also a lack of consensus on what condition should be defined as post–COVID-19 pulmonary fibrosis. During a relatively short follow-up period of approximately 1 year, lesions considered related to pulmonary fibrosis often showed gradual improvement; therefore, it is questionable at what time point fibrosis should be evaluated. In this review, we investigated the epidemiology, risk factors, pathogenesis, and management of post–COVID-19 pulmonary fibrosis.