• Tuberculosis and Respiratory Diseases
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• v.67 no.2
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• pp.113-120
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• 2009

Background: Reactive oxygen species (ROS) by oxidative stress may play an important role in the pathogenesis of various chronic diseases such as diabetes mellitus, obesity, hyperlipidemia, hypertension and malignancy that are linked to metabolic syndrome. Oxidative stress has been implicated in the pathogenesis of idiopathic pulmonary fibrosis (IPF). We examined the relationship between IPF and presenting factors associated with metabolic disorders. Methods: One hundred fourteen patients who met the current consensus of IPF definition were enrolled from March 2000 to April 2006 in Gil Hospital and Samsung Medical Center in Korea. One hundred thirty-four control subjects without pulmonary diseases were selected from subjects who visited Gil hospital for routine medical examinations, including low-dose chest computed tomography from January 2002 to July 2006. Retrospectively, we analyzed the clinical characteristics, the results of blood examinations, and lung function tests from medical records of both groups. Results: IPF patients and control subjects differed in the prevalence of diabetes mellitus as assessed by univariate analysis. Multivariate analysis demonstrated that diabetes mellitus and obesity were associated with IPF. The adjusted odds ratios for diabetes mellitus were 2.733 (95% confidence interval [CI], 1.282~5.827) and 2.001 (95% [CI], 1.063~3.766) for obesity. The remaining factors tested showed no differences between the patient group and the control. Conclusion: Diabetes mellitus and obesity may be associated with IPF development.

• The Journal of Internal Korean Medicine
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• v.25 no.1
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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.

• The Journal of Pediatrics of Korean Medicine
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• v.22 no.2
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• pp.37-49
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• 2008

Objectives : Idiopathic pulmonary fibrosis (IPF) is chronic fibrotic interstitial pneumonia and the pathogenesis is unknown. Peucedani Radix is well-known for the treatment of respiratory diseases and pulmonary hypertension. This study was to evaluate the effectiveness of Peucedani Radix on the bleomycin-induced lung fibrosis model (BLFM) in mouse. Methods : We induced lung fibrosis by intratracheal instillation of bleomycin in C57BL/6J. We compared two groups BLFM without Peucedani Radix (group I) and BLFM with Peucedani Radix (group II). We performed bronchoalveolar lavages (BAL) and obtained lung specimens from both group I and II on the 7th (A) and 21st (B) day, and also for the normal group. We compared with group I and II to find BAL by using ANOVA test and to find pathologic symptoms by using semiquantitative histological index (SHI). Results : In BAL, total cell counts, lymphocytes, and neutrophils was increased in both group I and II comparing with normal group. However, lymphocyte level was decreased more in group IIB than group IB. It was statistically significant. In microscopic findings, scores of SHI in normal group, group IB and IIB were 0.33, 4.47, and 1.96 each. Conclusions : Peucedani Radix might have inhibitory effect on lung fibrosis by reducing inflammatory cells in bleomycin-induced lung fibrosis model in mouse.

• Tuberculosis and Respiratory Diseases
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• v.66 no.2
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• pp.141-151
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• 2009

Background: There is limited data on the epidemiology and relative frequency of idiopathic interstitial pneumonia (IIP) worldwide. This survey was performed to assess the epidemiology and relative frequency of IIP in Korea. Methods: The patients with IIP and who were confirmed by lung biopsy, except those patients with idiopathic pulmonary fibrosis, (IPF) over a 5 year period (from Jan. $1^{st}$, 2003 to Dec. $31^{st}$, 2007) were registered by a web-base questionnaire. Results: A total of 3,156 cases were registered, but 970 cases were excluded due to duplicative registration, inadequate data and the unmet ATS/ERS diagnostic criteria. A total of 2,186 cases were analyzed. The male to female ratio was about 2 : 1 and their mean age was 65 (range: 11-94). The most frequent disease was IPF (77.1%), followed in decreasing order by nonspecific interstitial pneumonia (NSIP) (11.9%), cryptogenic organizing pneumonia (COP) (8.5%), acute interstitial pneumonia (AIP) (1.1%), desquamative interstitial pneumonia (DIP) (0.9%), respiratory bronchiolitis-interstitial lung disease (RB-ILD) (0.4%) and lymphocytic interstitial pneumonia (LIP) (0.1%). The mean age of the patients with IPF, NSIP and COP was 67.8, 57.1 and 57.7 years old, respectively. The most frequent symptom was dyspnea on exertion (69%) followed by coughing (61%) and sputum (33%) for the whole population. The three year survival rate was 62% for the patients with IPF and the five year survival rate was 85% in both the NSIP and COP patients. Conclusion: This survey provides helpful information for the management of IIP and to produce management guidelines for this illness in Korea.

• Tuberculosis and Respiratory Diseases
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• v.85 no.2
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• pp.185-194
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• 2022

Background: The Korea Interstitial Lung Disease Study Group has made a new nationwide idiopathic pulmonary fibrosis (IPF) registry because the routine clinical practice has changed due to new guidelines and newly developed antifibrotic agents in the recent decade. The aim of this study was to describe recent clinical characteristics of Korean IPF patients. Methods: Both newly diagnosed and following IPF patients diagnosed after the previous registry in 2008 were enrolled. Survival analysis was only conducted for patients diagnosed with IPF after 2016 because antifibrotic agents started to be covered by medical insurance of Korea in October 2015. Results: A total of 2,139 patients were analyzed. Their mean age at diagnosis was 67.4±9.3 years. Of these patients, 76.1% were males, 71.0% were ever-smokers, 14.4% were asymptomatic at the time of diagnosis, and 56.9% were at gender-age-physiology stage I. Occupational toxic material exposure was reported in 534 patients. The mean forced vital capacity was 74.6% and the diffusing capacity for carbon monoxide was 63.6%. Treatment with pirfenidone was increased over time: 62.4% of IPF patients were treated with pirfenidone initially. And 79.2% of patients were treated with antifiboritics for more than three months during the course of the disease since 2016. Old age, acute exacerbation, treatment without antifibrotics, and exposure to wood and stone dust were associated with higher mortality. Conclusion: In the recent Korean IPF registry, the percentage of IPF patients treated with antifibrotics was increased compared to that in the previous IPF registry. Old age, acute exacerbation, treatment without antifibrotics, and exposure to wood and stone dust were associated with higher mortality.

• The Journal of Internal Korean Medicine
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• v.44 no.3
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• pp.366-386
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• 2023

Objective: In this study, a questionnaire survey was conducted for doctors and patients to obtain basic data necessary for the development of an integrated medical system for idiopathic pulmonary fibrosis (IPF). Methods: Questionnaires were developed separately for doctors and patients through an expert group meeting. The survey subjects were recruited online and offline, and finally, responses from 231 doctors and 59 patients were used for statistical analysis. Results: The most important parts in the treatment of IPF for both doctors and patients were the "improvement of respiratory symptoms," "improvement of quality of life," and "prevention of disease progression." Antifibrotic agents were prescribed at a high rate, and 100% of the specialists in Western medicine (WM) and 45.8% of patients reported experiencing side effects. As for the additional payment costs that patients considered as affordable for an integrated medical system, "under 50,000 won (about 38$)" was reported the most in both doctor and patient groups. With regard to the reasons for their reluctance to recommend an integrated medical system for IPF, specialists in WM responded highly to "uncertain evidence for treatment effectiveness." Regarding complementary and alternative medicine therapies that can be beneficial in patients with IPF, "lifestyle management," "diet management," "herb," "relaxation therapy," and "psychotherapy" were ranked high in both doctor and patient groups. Conclusions: In this study, a questionnaire survey on IPF was conducted to review actual treatment status, analyze shortcomings, and identify considerations for the development of an integrated medical system for IPF in the future.

• Tuberculosis and Respiratory Diseases
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• v.59 no.1
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• pp.77-85
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• 2005

Background : According to the 2002 consensus report of the American Thoracic Society/European Respiratory Society (ATS/ERS), idiopathic pulmonary fibrosis (IPF) was classified as biopsy proven or probable IPF. Probable IPF is defined as those with distinctive features that allow for a confident diagnosis of IPF/usual interstitial pneumonia (UIP) within an appropriate clinical setting. The determination of the clinical course of probable IPF, as diagnosed by the ATS/ERS criteria, was studied. Methods : Between March 1995 and August 2002, 36 patients with probable IPF, from two tertiary referral hospitals, were enrolled in this study. The clinical characteristics, prognostic factors and treatment efficacy of these patients were retrospectively evaluated. Results : The mean age of the subjects was $65{\pm}6$ years. The one and 3 year survival rates were 82.4 and 50.3%, respectively, and a median survival period of 42.0 months. The total cell count of bronchoalveolar lavage was higher in the death than the survival group (p<0.05). No survival benefits were found in the cytoxan and steroid treatment groups compared with other treatment groups. Conclusion : These results suggest that the clinical course of probable IPF may be similar to that of biopsy-proven UIP. However, atypical patients must undergo an open lung biopsy for confirmation of the diagnosis.

• The Journal of Internal Korean Medicine
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• v.43 no.3
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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.

• BMB Reports
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• v.57 no.3
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• pp.143-148
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• 2024

Pulmonary fibrosis is a serious lung disease that occurs predominantly in men. Genistein is an important natural soybean-derived 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 α-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.

• Tuberculosis and Respiratory Diseases
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• v.55 no.5
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• pp.478-487
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• 2003

Background : There have been several studies showing that the angiotensin II and angiotensin converting enzyme(ACE) contributes to the apoptosis of alveolar epithelial cells in idiopathic interstitial pneumonia and the activation of fibroblasts during the process of pulmonary fibrosis. These results suggest that the pulmonary fibrosis can be inhibited by the angiotensin II receptor antagonist(AGIIRA). This study was performed to identify the therapeutic effect of AGIIRA in idiopathic pulmonary fibrosis(IPF). Method : Thirteen patients with IPF, who were diagnosed with an open lung biopsy(6 patients) and furfilling the ATS criteria(7 patients) between March 1999 and October 2001 at the Gachon medical center, were enrolled in this study. Of these patients, eight patients were treated with a regimen including AGIIRA(AT group), and five were treated without AGIIRA(NT group). The pulmonary function tests and dyspnea(ATS scale) were measured at diagnosis and 1 year after treatment. All the data was collected to analyze the therapeutic effect of AGIIRA on the patients with IPF. Results : The AT group contained 8 patients(M:F=4:4) and the NT group contained 5 patients(M:F=3:2). There was no significant difference in the serum angiotensin II level between the two groups($202.5{\pm}58.5$ vs $163.7{\pm}47.3pg/ml$, p>0.05). The AT group showed an upward trend in TLC(+3%), FVC(+4%), FEV1(+3%) and DLco(+2%) compared to the NT group(TLC(-14%), FVC(-3%), FEV1(-4%) except for DLco(+5%)). The dyspnea score in the AT group improved significantly but not in the NT group. Conclusion : These results suggest that the angiotensin II receptor antagonist may have an effect on stabilizing IPF.