• Tuberculosis and Respiratory Diseases
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• 제82권2호
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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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• 제52권5호
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• pp.550-556
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• 2002

Microscopic polyangiitis is a systemic small-vessel vasculitis that is associated primarily with necrotizing glomerulonephritis and pulmonary capillaritis. A recurrent and diffuse alveolar hemorrhage due to pulmonary capillaritis is the main clinical manifestation of lung involvement. Recently, and interstitial lung disease that mimics idiopathic pulmonary fibrosis was reported to be rarely associated with microscopic polyangiitis. Here we report two patients with microscopic polyangiitis who showed a honeycomb lung at the time of the initial diagnosis with a brief review of relevant literature.

• Biomolecules & Therapeutics
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• 제27권3호
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• pp.318-326
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• 2019

Sphingosine 1-phosphate (S1P) levels are often found to be elevated in serum, bronchoalveolar lavage, and lung tissue of idiopathic pulmonary fibrosis patients and experimental mouse models. Although the roles of sphingosine kinase 1 and S1P receptors have been implicated in fibrosis, the underlying mechanism of fibrosis via Sphingosine 1-phosphate receptor 2 ($S1P_2$) has not been fully investigated. Therefore, in this study, the roles of $S1P_2$ in lung inflammation and fibrosis was investigated by means of a bleomycin-induced lung fibrosis model and lung epithelial cells. Bleomycin was found to induce lung inflammation on day 7 and fibrosis on day 28 of treatment. On the $7^{th}$ day after bleomycin administration, $S1P_2$ deficient mice exhibited significantly less pulmonary inflammation, including cell infiltration and pro-inflammatory cytokine induction, than the wild type mice. On the $28^{th}$ day after bleomycin treatment, severe inflammation and fibrosis were observed in lung tissues from wild type mice, while lung tissues from $S1P_2$ deficient mice showed less inflammation and fibrosis. Increase in TGF-${\beta}1$-induced extracellular matrix accumulation and epithelial-mesenchymal transition were inhibited by JTE-013, a $S1P_2$ antagonist, in A549 lung epithelial cells. Taken together, pro-inflammatory and pro-fibrotic functions of $S1P_2$ were elucidated using a bleomycin-induced fibrosis model. Notably, $S1P_2$ was found to mediate epithelial-mesenchymal transition in fibrotic responses. Therefore, the results of this study indicate that $S1P_2$ could be a promising therapeutic target for the treatment of pulmonary fibrosis.

• 대한한방내과학회지
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• 제25권1호
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• pp.71-80
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• 2004

Objective : Idiopathic Lung Fibrosis(IPF) is chronic fibrotic interstitial pneumonia. The pathogenesis is unclear. Lonicerae Flos is known to prevent the inflammation and reinforce the immune system. The effects of Lonicerae Flos on bleomycin-induced lung fibrosis is evaluated. Material and Methods: Lonicerae Flos extract was given to the Normal rats, control(bleomycin) rats everyday and treated(bleomycin and lonicerae flos) rats 21.0 mg per body weight 109 for 14 days. 14 days after, we observed the change of leukocyte count and percentage of IFN-gamma and IL-4 in BALF. and that of Semiquantative histological index(SHI). Results : Compared to control rats, Lonicerae Flos decreased leukocyte count(P<0.01) lymphocyte, neutrophil percentage(P<0.05), SHI(P<0.01), IFN-gamma and IL-4(P<0.05) in Treated rats. Otherwise, macrophage percentage was increased(P<0.01) in Treated rats. Conclusion : This study showed that Lonicerae Flos reduced the change of inflammatory cells and cytokines in bleomycin-induced lung fibrosis and reduced the fibrosis of tissue. And, we needed many other distinct researches on lung fibrosis.

• Tuberculosis and Respiratory Diseases
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• 제73권3호
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• pp.162-168
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• 2012

Background: Idiopathic pulmonary fibrosis (IPF) is a progressive disease. Effective treatment is not currently available and the prognosis is poor. The aim of our study was to identify clinical predictors of survival in patients with IPF. Methods: By using medical record database of a university hospital, we reviewed the records of patients who had been diagnosed as having IPF from January 1996 through December 2007. Results: Among 89 patients considered as having interstitial lung disease (ILD) on computed tomography (CT) of the chest, 22 were excluded because of the diagnosis of other ILDs or connective tissue disease, and finally, 67 met the criteria of IPF. The mean age at the diagnosis of IPF was 70 years (range, 41~87 years) and 43 (64%) were male. The mean survival time following the diagnosis of IPF was 40 months (range, 0~179 months). Among them, 28 cases were diagnosed as the progressive state of IPF on the follow-up CT examination, and the mean duration between diagnosis of IPF and progression was 31 months. Multivariate analysis using Cox regression model revealed that body mass index (BMI) less than 18.5 $kg/m^2$ (p=0.030; hazard ratio [HR], 12.085; 95% confidence interval [CI], 1.277~114.331) and CT progression before 36 months from the diagnosis of IPF (p=0.042; HR, 13.564; 95% CI, 1.101~167.166) were independently associated with mortality. Conclusion: Since low BMI at the diagnosis of IPF and progression on follow-up CT were associated with poor prognosis, IPF patients with low BMI and/or progression before 36 months following the diagnosis should be closely monitored.

• 대한한방내과학회지
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• 제43권3호
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• pp.327-343
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• 2022

Objective: The purpose of this study was to develop a standard tool for identifying idiopathic pulmonary fibrosis patterns. Methods: Textbooks, published literature, and references with comments on patterns were reviewed. Using the Delphi method, we determined pattern identification based on the advice of a committee consisting of 11 Korean respiratory internal medicine professors. Results: Four pattern identifications were selected by the Delphi method: qi difficiency (氣虛), yin difficiency (陰虛), phlegm dampness (痰飮), blood stasis (瘀血). The tool was developed in a question-and-answer format containing 38 questions. Conclusions: An IPF pattern identification tool that can analyze IPF patterns for standardized diagnostics was developed with the consent of experts. Further research is needed on its reliability.

• The Korean journal of internal medicine
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• 제39권2호
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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.

• Tuberculosis and Respiratory Diseases
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• 제87권2호
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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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• 제84권4호
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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.

• Tuberculosis and Respiratory Diseases
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• 제55권5호
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• pp.478-487
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• 2003

연구배경 : Angiotensin II가 폐포상피세포의 세포사멸을 유도하고 폐섬유모세포에서 TGF-${\beta}$등의 발현을 증가시켜 폐섬유화증을 촉진시킬 수 있다고 알려져 있어 angiotensin II receptor의 차단이 폐섬유화증을 감소시키는 효과가 있을 것으로 예상하고 특발성 폐섬유화증 환자에게 angiotensin II receptor antagonist(AGIIRA)를 투여하여 치료효과를 알아보고자 하였다. 방 법 : 저자들은 가천의대 길병원에서 특발성 폐섬유화증으로 진단된 13명의 환자를 대상으로 하였다. 이들 중 8명 의 환자에게는 angiotensin II type 1 receptor antaginist인 losartan(cozaar$^{(R)}$) 을 투여하였고 나머지 5명의 환자에게는 losartan을 투여 하지 않았으며, 치료 직전과 치료 l년 후에 모든 환자에게 폐기능 검사와 호흡곤란 지수의 정도의 변화를 측정하여 그 결과를 비교하였다. 결 과 : AGIIRA 복용 군에서는 폐기능이 전체적으로 약간의 호전을 보였으며, 미 복용 군에서는 DLco%가 5%로 증가하였으나 TLC가 14%로 감소하는 등 전반적으로 페기능이 감소하는 소견을 보였다. 폐기능 변화가 두 군 모두 통계적으로 유의성이 없었고 두 군간의 변화율의 변화에서도 통계적 유의성은 없었다. 호흡곤란 지수는 AGIIRA 복용한 군에서만 통계적으로 유의하게 더 호전을 보였다. 결 론 : 특발성 폐섬유화증의 치료에 AGIIRA가 일부 환자들에게 임상적 안정화에 도움을 줄 수 있을 것으로 사료된다. 차후 더 많은 환자를 대상으로 오랜 기간의 연구와 함께 폐섬유화에 있어서 anglotensin II와 그 receptor에 대한 기전과 역할에 대하여 더 많은 연구가 필요할 것으로 생각된다.