• Tuberculosis and Respiratory Diseases
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• v.50 no.6
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• pp.704-709
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• 2001

Background : Spontaneous pneumopericardium is a very rare condition. Spontaneous pneumothorax and pneumomediastinum have been reported to be associated with an idiopathic pulmonary fibrosis (IPF). However, spontaneous pneumopericardium has not yet been reported in association with IPF. Here we report a case of spontaneous pneumomediastinum and pneumopericardium in a patient with acute exacerbation of IPF with a review of the relevant literature.

• Proceedings of the Korean Institute of Information and Commucation Sciences Conference
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• 2021.05a
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• pp.144-146
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• 2021

Idiopathic pulmonary fibrosis (IPF) is one of the most dreadful lung diseases which effects the performance of the lung unpredictably. There is no any authentic natural history discovered yet pertaining to this disease and it has been very difficult for the physicians to diagnosis this disease. With the advent of Artificial intelligent and its related technologies this task has become a little bit easier. The aim of this paper is to develop and to explore the machine learning models for the prediction and diagnosis of this mysterious disease. For our study, we got IPF dataset from Haeundae Paik hospital consisting of 2425 patients. This dataset consists of 502 features. We applied different data preprocessing techniques for data cleaning while making the data fit for the machine learning implementation. After the preprocessing of the data, 18 features were selected for the experiment. In our experiment, we used different machine learning classifiers i.e., Multilayer perceptron (MLP), Support vector machine (SVM), and Random forest (RF). we compared the performance of each classifier. The experimental results showed that MLP outperformed all other compared models with 91.24% accuracy.

• The Korean journal of internal medicine
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• v.39 no.2
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• pp.295-305
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• 2024

Background/Aims: The prognosis of patients with idiopathic pulmonary fibrosis (IPF) and respiratory failure requiring mechanical ventilation is poor. Therefore, mechanical ventilation is not recommended. Recently, outcomes of mechanical ventilation, including those for patients with IPF, have improved. The aim of this study was to investigate changes in the use of mechanical ventilation in patients with IPF and their outcomes over time. Methods: This retrospective, observational cohort study used data from the National Health Insurance Service database. Patients diagnosed with IPF between January 2011 and December 2019 who were placed on mechanical ventilation were included. We analyzed changes in the use of mechanical ventilation in patients with IPF and their mortality using the Cochran-Armitage trend test. Results: Between 2011 and 2019, 1,227 patients with IPF were placed on mechanical ventilation. The annual number of patients with IPF with and without mechanical ventilation increased over time. However, the ratio was relatively stable at approximately 3.5%. The overall hospital mortality rate was 69.4%. There was no improvement in annual hospital mortality rate. The overall 30-day mortality rate was 68.7%, which did not change significantly. The overall 90-day mortality rate was 85.3%. The annual 90-day mortality rate was decreased from 90.9% in 2011 to 83.1% in 2019 (p = 0.028). Conclusions: Despite improvements in intensive care and ventilator management, the prognosis of patients with IPF receiving mechanical ventilation has not improved significantly.

• Journal of the Korean Society of Radiology
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• v.82 no.4
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• pp.770-790
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• 2021

Idiopathic pulmonary fibrosis (IPF), based on the 2018 international clinical practice guidelines, can be diagnosed with a usual interstitial pneumonia (UIP) pattern on high-resolution computed tomography (HRCT) and compatible clinical findings. Given that imaging is pivotal for IPF evaluation and diagnosis, more emphasis should be placed on the integration of clinical, radiological, and pathologic findings for multidisciplinary diagnosis. Interstitial lung abnormality (ILA), on the other hand, has a purely radiological definition based on the incidental identification of CT abnormalities. Taken together, differentiation between ILA and clinically significant interstitial lung disease (ILD) must be based on proper clinical evaluation. With this review, the recent updates in IPF diagnosis and the radiologic considerations for ILA can be well understood, which can be helpful for the proper diagnosis and management of patients with diffuse interstitial pulmonary fibrosis.

• Journal of Radiopharmaceuticals and Molecular Probes
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• v.8 no.2
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• pp.103-111
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• 2022

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.

• 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.

• Tuberculosis and Respiratory Diseases
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• v.56 no.6
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• pp.611-618
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• 2004

Background : Idiopathic pulmonary fibrosis(IPF), a subtype of IIP(idiopathic interstitial pneumonia), is a fatal disease with a 3-5 year median survival. Many attempts at treating this condition have failed to demonstrate a survival benefit in IPF. Recently Ziesche et $al^{12}$ reported the efficacy of IFN-${\gamma}$ for treating IPF but there is still some controversy. The aim of this study was to determine the efficacy of IFN-${\gamma}$ in patients with advanced IPF who had not been responsive to steroid and cytotoxic agents. Method : Nine patients with advanced IPF(age: $55.4{\pm}15.3$ years, Male: Female=8:1) were enrolled. One year treatment regime with 2 million IU of IFN-${\gamma}$ administered subcutaneously three times a week, and low dose prednisolone(10-30 mg/d) was also used. In the case of a definite aggravation and serious side effects, the IFN-${\gamma}$ was discontinued. During the IFN-${\gamma}$ trial, a pulmonary function test and chest radiography were checked every three month throughout the study. Result : 1) Among 9 patients, only 4 patients were able to complete the 12 month treatment with IFN-${\gamma}$, and 5 patients died during the treatment period. 2) No improvement either in the respiratory symptoms or pulmonary functions were observed any of the patients, even in those who completed the 12 months trial of IFN-${\gamma}$, 3) At the time of IFN-${\gamma}$ trial, the survivors who finished the IFN-${\gamma}$ treatment for 12 months had a higher oxygen level($81.3{\pm}2.8$ vs. $67.4{\pm}8.4$, P=0.024) and a better pulmonary function(FVC: $61.3{\pm}5.1$ % predicted vs. $45.7{\pm}12.3%$, P=0.048, and $D_Lco$: $45.0{\pm}5.0%$ predicted vs. $30.8{\pm}11.2%$, P=0.048) than the non-survivors. Conclusion : Our data suggested that IFN-${\gamma}$ therapy was not effective in the patients with advanced IPF refractory compared with other therapeutic agents. Furthermore, these results suggest that severe impairment of the pulmonary function and hypoxemia during the IFN-${\gamma}$ therapy requires special attention.

• 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.

• Tuberculosis and Respiratory Diseases
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• v.85 no.2
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• pp.122-136
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• 2022

Although chronic obstructive pulmonary disease (COPD) and interstitial lung disease (ILD) have distinct clinical features, both diseases may coexist in a patient because they share similar risk factors such as smoking, male sex, and old age. Patients with both emphysema in upper lung fields and diffuse ILD are diagnosed with combined pulmonary fibrosis and emphysema (CPFE), which causes substantial clinical deterioration. Patients with CPFE have higher mortality compared with patients who have COPD alone, but results have been inconclusive compared with patients who have idiopathic pulmonary fibrosis (IPF). Poor prognostic factors for CPFE include exacerbation, lung cancer, and pulmonary hypertension. The presence of interstitial lung abnormalities, which may be an early or mild form of ILD, is notable among patients with COPD, and is associated with poor prognosis. Various theories have been proposed regarding the pathophysiology of CPFE. Biomarker analyses have implied that this pathophysiology may be more closely associated with IPF development, rather than COPD or emphysema. Patients with CPFE should be advised to quit smoking and undergo routine lung function tests, and pulmonary rehabilitation may be helpful. Various pharmacologic agents and surgical approaches may be beneficial in patients with CPFE, but further studies are needed.

• Molecules and Cells
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• v.41 no.3
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• pp.224-233
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• 2018

Primary cilia are solitary, non-motile, axonemal microtubule-based antenna-like organelles that project from the plasma membrane of most mammalian cells and are implicated in transducing hedgehog signals during development. It was recently proposed that aberrant SHH signaling may be implicated in the progression of idiopathic pulmonary fibrosis (IPF). However, the distribution and role of primary cilia in IPF remains unclear. Here, we clearly observed the primary cilia in alveolar epithelial cells, fibroblasts, and endothelial cells of human normal lung tissue. Then, we investigated the distribution of primary cilia in human IPF tissue samples using immunofluorescence. Tissues from six IPF cases showed an increase in the number of primary cilia in alveolar cells and fibroblasts. In addition, we observed an increase in ciliogenesis related genes such as IFT20 and IFT88 in IPF. Since major components of the SHH signaling pathway are known to be localized in primary cilia, we quantified the mRNA expression of the SHH signaling components using qRT-PCR in both IPF and control lung. mRNA levels of SHH, the coreceptor SMO, and the transcription factors GLI1 and GLI2 were upregulated in IPF compared with control. Furthermore, the nuclear localization of GLI1 was observed mainly in alveolar epithelia and fibroblasts. In addition, we showed that defective KIF3A-mediated ciliary loss in human type II alveolar epithelial cell lines leads to disruption of SHH signaling. These results indicate that a significant increase in the number of primary cilia in IPF contributes to the upregulation of SHH signals.