• Title/Summary/Keyword: 특발성 폐섬유증

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Expression of Intercellular Adhesion Molecule-1 in Human Idiopathic Pulmonary Fibrosis (인체 특발성 폐섬유증에서 Intercellular Adhesion Molecule-1의 발현에 관한 연구)

  • Park, Sung-Soo;Shin, Dong-Ho;Kim, Tae-Wha;Lee, Dong-Hoo;Lee, Jung-Hee;Lee, Jung-Dal
    • Tuberculosis and Respiratory Diseases
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    • v.40 no.2
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    • pp.185-191
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    • 1993
  • Background: Intercellular adhesion molecule-1(ICAM-1) is a 90 kD surface glycoprotein, associated with ${\alpha}_L{\beta}_2$ and ${\alpha}_M{\beta}_2$ subunit of integrins, that serve as cell-cell and cell-substratum adhesion molecules and help regulate cellular morphology, differentiation, and proliferation. The adhesion molecules likely play important roles in maintaining the normal structure and function of the lung. ICAM-1 system among many cell adhesion molecules is importantly issuing in the pathogenesis of idiopathic pulmonary fibrosis. Methods : By using $IgG_1$ monoclonal antibody for ICAM-1, we investigated immunohistochemically the expression of ICAM-1 in the formalin-fixed, paraffin-embedded tissue sections of the 3 normal cases and 6 pieces of tissues taken 3 cases with idiopathic pulmonary fibrosis. Results : In the 3 normal cases, the expressions of ICAM-1 were not discernible. Up-regulation of the ICAM-1 expression was showed in the interstitial fibroblast cells of alveolar septa in 5 pieces and proliferated alveolar pneumocytes in 1 piece among 6 pieces of tissues taken 3 cases with idiopathic pulmonary fibrosis. Conclusion : It was concluded from these findings that up-regulation of the ICAM-1 expression may be related to pathogenesis of idiopathic pulmonary fibrosis.

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Acute Exacerbation of Idiopathic Pulmonary Fibrosis with Lung Cancer: A Comparative Analysis of the Incidence, Survival Rate, and CT Findings with the Patients without Lung Cancer (폐암을 동반한 특발성 폐섬유증 환자에서 폐섬유증의 급성 악화: 폐암 비동반군과의 발병률, 생존율 및 전산화단층촬영 소견의 비교)

  • Bumsang Cho;Hee Kang;Je Hun Kim;Jung Gu Park;Sekyoung Park;Jong Hyouk Yun
    • Journal of the Korean Society of Radiology
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    • v.81 no.3
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    • pp.688-700
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    • 2020
  • Purpose To compare the incidence, survival rate, and CT findings of acute exacerbation (AE) of idiopathic pulmonary fibrosis (IPF) between patients with and without lung cancer. Materials and Methods From June 2004 to July 2018, 89 consecutive patients diagnosed with IPF were included. Among them, 26 patients had IPF with lung cancer (IPF-LCA), and 63 patients had IPF alone. The clinical characteristics and CT findings associated with IPF, lung cancer, and AE were reviewed. Surgery and chemotherapy were performed for 6 and 23 cases of lung cancer, respectively, as the first- or second-line anticancer treatment. The overall survival, CT findings, disease-free period before AE, and duration from the onset of AE to death were compared. Results The incidence of AE was 61.5% in the IPF-LCA group and 58.7% in the IPF group (p = 0.806). The mean overall survival in the IPF-LCA and IPF groups were 16.8 and 83.0 months, respectively (p < 0.001). The mean durations from the start of the lung cancer treatment to the onset of AE were 16.0 and 4.6 months in cases of surgical treatment and chemotherapy, respectively. In comparison of death from AE, the survival rate was significantly lower in the IPF-LCA group than in the IPF group (p = 0.008). In the CT findings associated with AE, the IPF-LCA group tended to have a peribronchial (p < 0.001) or asymmetric distribution (p = 0.016). Conclusion In patients with IPF who develop lung cancer, the rate of death from AE is higher than that in patients with IPF alone. They tend to have unusual CT patterns associated with AE, such as a peribronchial or asymmetric distribution.

Expression of Transforming Growth Factor-Beta in Patients with Interstitial Lung Diseases (ILD) (간질성 폐질환환자에서 Transforming growth factor-beta의 발현에 관한 연구)

  • Park, Sung-Soo;Lee, Kyung-Sang;Yang, Suck-Chul;Yoon, Ho-Joo;Shin, Dong-Ho;Lee, Dong-Hoo;Lee, Jung-Dal;Lee, Jung-Hee
    • Tuberculosis and Respiratory Diseases
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    • v.43 no.2
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    • pp.164-172
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    • 1996
  • Background : Transforming growth factor-$\beta$(TGF-$\beta$) may play a role in a variety of fibroproliferative disorders including pulmonary fibrosis via the induction of extracellular matrix accumulation. TGF-$\beta$ not only stimulates extracellular matrix production, but also decreases matrix degradation. Interstial lung diseases have demonstrated marked expression of TGF-$\beta$. Methods : To evaluate the possible role of TGF-$\beta$ in human pulmonary fibrosis, by using neutralizing antibody of TGF-$\beta$ we investigated immunohistochemically the expression of TGF-$\beta$ in the formalin-fixed, paraffin-embedded tissue sections of the 5 normal cases for the control, and a couple of pieces of tissues taken out of 3 cases with idiopathic pulmonary fibrosis, 3 cases with ILD from bleomycin toxicity, 3 cases with ILD from sarcoidosis, and 3 cases with ILD from eosinophilic granuloma. Results : In the 5 normal cases for the control, the TGF-$\beta$ was expressed in bronchial and alveolar epithelial cells. Up-regulation of the TGF-$\beta$ expression was showed in the interstitial fibroblast cells of alveolar septa in 5 pieces and proliferated alveolar pneumocytes in 1 piece among 6 pieces tissues taken out of 3 cases with idiopathic pulmonary fibrosis. Also up-regulation of the TGF-$\beta$ expression was showed in alveolar lining pneumocytes, intra-alveolar mononuclear cells, and epithelioid cells in most of cases of ILD from bleomycin toxicity, sarcoidosis and eosinophilic granuloma. Conclusion : These findings suggest that up-regulation of the TGF-$\beta$are involved in pathogenesis of interstitial lung fibrosis from variety of causes.

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Update in Diagnosis of Idiopathic Pulmonary Fibrosis and Interstitial Lung Abnormality (특발폐섬유증 진단의 최신 지견과 간질성폐이상)

  • Bo Da Nam;Jung Hwa Hwang
    • 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.

Diagnosis of Diffuse Lung Disease by Quantitative Analysis (정량적 방법에 의한 미만성 폐질환 진단)

  • 원철호;김명남;이종민;최태진;강덕식
    • Journal of Biomedical Engineering Research
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    • v.20 no.5
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    • pp.545-557
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    • 1999
  • 본 논문에서는 호흡 연동 장치와 EBT로부터 획득한 폐실질 영상에 대하여 동적 윤곽선 모델 방법과 영역 성장법을 이용하여 폐실질 영역을 검출하였다. 그런 다음 , 검출된 폐실질 영역내에서의 각종 정량적 요소들을 도출하여 농도 분포 곡선에대한 분석을 하였다. 동적 윤곽선 모델방법에서 페실질 영역의 낮은 휘도 준위와 폐의 윤곽선 벡터 방향을 고려한 에너지 함수를 제안하였다. 그리고 폐실질 영역 성장법에서는 폐실질 영역내의 분포한 공기 성분에 대한 화소를 확장시켜 효과적으로 폐실질 영역을 검출하였다. 추출된 폐실질 영역내의 빈도 분포 곡선을 분석하여 정상군과 비교한 결과 만성 폐쇄성 폐질환자에서는 정상인에 비하여 평균 농도,최대 빈도 농도, 최대 상승 기울기 농도가 낮았으며, 농도 분포곡선은 더 낮은 쪽으로 이동하였음을 알 수 있었다. 또한, 특발성 폐섬유증 환자에서는 평균 농도, 최대 빈도 농도, 최대 상승 기울기 농도가 모두 증가되었고 농도 분포 곡선은 더 높은쪽으로 이동하였다. 폐실질 영역을 추출하여 히스토그램 분포에 대한 정량적 분석을 함으로써 정상인으로부터 만성 폐쇄성 질환자의 폐섬유증 환자를 구분할 수 있었다.

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A Case of Spontaneous Pneumomediastinum and Pneumopericardium in a Patient with Acute Exacerbation of Idiopathic Pulmonary Fibrosis (자발성 종격동기흉과 심낭기종을 동반한 특발성 폐섬유증의 급성 악화 1예)

  • Yun, Se-Young;Kim, Yong-Ho;Choi, Eun-Kyoung;Hong, Seuk-Kyun;Ji, Young-Ku;Lee, Kye-Young;Choi, Young-Hi;Myong, Na-Hye;Park, Jae-Seuk
    • 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.

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The Clinical Characteristics of Lung Cancer in Patients with Idiopathic Pulmonary Fibrosis (특발성 폐섬유화증에 동반된 폐암 환자의 임상적 특정)

  • Park, Joo-Hun;Lee, Jin-Seong;Song, Koun-Sik;Shim, Tae-Sun;Lim, Chae-Man;Koh, Youn-Suck;Lee, Sang-Do;Kim, Woo-Sung;Kim, Won-Dong;Kim, Dong-Soon
    • Tuberculosis and Respiratory Diseases
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    • v.46 no.5
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    • pp.674-684
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    • 1999
  • Background : It has been generally known that the incidence of lung cancer is higher in the patients with idopathic pumonary fibrosis (IPF) than those in general population. The reported incidence was variable from 4.8 to 43.2%. There were controversies on the most frequent cell type (squamous cell carcinoma vs. adenocarcinoma) and no study was done about the real concordance of cancer and the fibrotic lesion. And the pulmonary fibrosis may influence not only the development of cancer but also the treatment and prognosis of the cancer, but there was no report on that point. Method : Total 63 patients ($66.8{\pm}7.8$ year, M : F=61 : 2) were diagnosed as IPF combined with lung cancer (IFF-CA) at Asan Medical Center. A retrospective analysis was done about the risk factors of the lung cancer, pulmonary function test, the site of cancer(especially the relationship of the cancer with the fibrotic lesion), the histologic types, and the stage of cancer. The histologic types were compared with those of 2,660 patients with lung cancer who were diagnosed at the same institute for the same period. The effect of IPF on the treatment of the cancer was evaluated with the survival time after the detection of lung cancer. Results : The lung cancer was found in 63(22.9%) out of 281 patients with IPF. But in most of them(45 patients), lung cancer was detected at the same time with IPF and only in 18 patients, the cancer was diagnosed during the follow-up($25.2{\pm}17.7$ months) of IPF. So in our study, 6.7% of patients with IPF developed lung cancer during the course of the disease. The age ($66.8{\pm}7.84$ vs. $63.4{\pm}11.1$ years), percentage of smoker (88.9 vs. 67.2%), and the male gender (96.8 vs. 67.6%) were significantly higher in IPF-CA compared with lone IPF (p<0.05). The odds ratio of smoking was 4.7 compared with non smoking IPF controls. The lung cancer was located more frequently in the upper lobe and 55.5% was in the periphery of lung. The cancer was developed in the fibrotic lesion in 23 patients (35.9%), and in the majority of the patients, the cancer was separated from the fibrosis. The cell type of the lung cancer in IPF-CA was squamous cell carcinoma 34.9%, adenocarcinoma 30.2%, small cell carcinoma 19.0%, large cell undifferenciated carcinoma 6.3%, and others 9.5%. No significant difference in the distribution of histologic type of the lung cancer was found between IPF-CA and lone lung cancer. There was no significant difference in demographic features, cell types, location and the stage of the cancer between the group with concurrent IPF-CA and the group with cancer diagnosed during the follow up of IPF. There was a tendency (but statistically not significant : p=0.081) of higher incidence of adenocarcinoma among the cancers developed in the fibrotic area(43.5%) (F-CA) than in the cancers in non-fibrotic area (22.5%) (NF-CA). The prognosis of the patients with F-CA was poor (median survival : 4 months) compared with the patients with NF-CA (7 months, p=0.013), partly because the prevalence of severe IPF (the extent of fibrosis in HRCT 50%) was higher in F-CA group. Conclusion : These data suggest that the lung cancer in the patients with IPF has similar features to the ordinary lung cancer.

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The Usefulness of F-18 FDG PET to Discriminate between Malignant and benign Nodule in Idiopathic Pulmonary Fibrosis (특발성 폐섬유증에서 발견된 폐결절의 악성여부 감별에서 F-18 FDG PET의 유용성)

  • Kim, Bom-Sahn;Kang, Won-Jun;Lee, Dong-Soo;Chung, June-Key;Lee, Myung-Chul
    • Nuclear Medicine and Molecular Imaging
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    • v.40 no.3
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    • pp.163-168
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    • 2006
  • Purpose: Incidence of lung canter in patients with idiopathic pulmonary fibrosis (IPF) is known to be higher than that in general population. However, it is difficult to discriminate pulmonary nodule in patients with IPF, because underlying IPF can be expressed as lung nodules. We evaluated the diagnostic performance of FDG PET in discriminating lung nodule in patients with IPF. Methods: We retrospectively reviewed 28 lung nodules in 16 subjects (age; $67.53{\pm}9.53$, M:F=14:2). Two patients had previous history of malignant cancer (small cell lung cancer and subglottic cancer). The diagnostic criteria on chest CT were size, morphology and serial changes of size. FDG PET was visually interpreted, and maximal SUV was calculated for quantitative analysis. Results: from 28 nodules, 18 nodules were interpreted as benign nodules, 10 nodules as malignant nodules by histopahthology or follow-up chest CT. The sensitivity and specificity of FDG PET were 100% and 94.4%, while those of CT were 70.0% and 44.4%, respectively. Malignant nodule was higher maxSUV than that of benign lung nodules ($7.68{\pm}3.96\;vs.\;1.22{\pm}0.65$, p<0.001). Inflammatory lesion in underlying IPF was significantly lower maxSUV than that of malignant nodules ($1.80{\pm}0.43$, p<0.001). The size of malignant and benign nodule were $23.95{\pm}10.15mm\;and\;10.83{\pm}5.23mm$ (p<0.01). Conclusion: FDG PET showed superior diagnostic performance to chest CT in differentiating lung nodules in patients with underlying IPF. FDG PET could be used to evaluate suspicious malignant lung nodule detected by chest in patients with IPF.

The Effect of Vitamin E on Bleomycin-Induced Pulmonary Injury and Fibrosis in Rat - Comparison of Penicillamine- or Deferoxamine-Treated Group - (백서에서 Bleomycin 투여로 인한 폐손상 및 폐섬유화에 대한 Vitamin E의 영향 - Penicillamine, Deferoxamine 투여군과 비교 -)

  • Jung, Soon-Hee;Yong, Suk-Joong;Ahn, Chul-Min;Shin, Kye-Chul;Choi, In-Joon;Cho, Sang-Ho
    • Tuberculosis and Respiratory Diseases
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    • v.42 no.2
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    • pp.184-205
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    • 1995
  • Background: Pulmonary toxicity by bleomycin has multiple mechanisms including direct tissue toxicity due to oxygen-derived free radicals and indirect toxicity through amplification of pulmonary inflammation. To evaluate the effect of chelators or free radical scavenger to lung damage induced by bleomycin, penicillamine as a copper chelator, deferoxamine as an iron chelator and vitamin E as a free radical scavenger were administered. Methods: Two hundred Wistar rats were divided into five groups: Control, bleomycin treated, bleomycin-penicillamine treated, bleomycin-deferoxamine treated, and bleomycin-vitamin E treated groups. Rats sacrificed on day 1, day 3, day 4, day 7, day 14, and day 28 after treatment. Bronchoalveolar lavage, light microscopic and immunohistologic studies for type I, III, IV collagens, fibronectin, laminin and NBD phallicidin were evaluated. Results: There was a significant increase in the total cell counts of bronchoalveolar lavage on day 1 from all treated animals and vitamin treated group showed an abrupt decrease in total cell counts with decrease of neutrophils on day 3. Bleomycin-vitamin E treated group had the least histologic changes such as pulmonary fibrosis. The alveolar basement membranes were positive for type IV collegen and laminin. Basement membranes of bleomycin, bleomycin-penicillamine, or bleomycin-deferoxamine treated groups were disrupted and fragmented on day 4 or 7. The bleomycin-vitamin E treated group had intact basement membranes until day 28. Conclusion: Bleomycin-induced pulmonary fibrosis was related to the severity of acute injury to oxygen radicals or activation of neutrophils and disruption of basement membrane. Vitamin E seemed to be the most effective antioxidant in the inhibition of bleomycin-induced pulmonary injury and fibrosis.

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Research for the Development of an Integrated Medical System for Idiopathic Pulmonary Fibrosis: Based on an Analysis of Questionnaire Survey in Doctor and Patient Groups (특발성 폐섬유증의 통합의료 협진 모델 개발을 위한 의료진 및 환자 대상 설문조사 연구)

  • Jiwon Park;Beom-Joon Lee;Jeong-Won Shin;Su-Hyun Chin;Jung-Min Yang;Sooduk Kim;Kwan-Il Kim;Hye Sook Choi;Hee-Jae Jung
    • 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.