• Tuberculosis and Respiratory Diseases
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• v.49 no.5
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• pp.601-613
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• 2000

Background : Idiopathic pulmonary fibrosis (IPF) is a fatal progressive fibrous disease of the lung of unknown etiology. Recently it has been classified into several distinct entities on the basis of pathologic and clinical characteristics, ie : usual interstitial pneumonia (UIP), desquamative interstitial pneumonia (DIP), acute interstitial pneumonia (AIP), bronchiolitis obliterans with organizing pneumonia (BOOP), and nonspecific interstitial pneumonia (NSIP). IPF is now applied only for UIP, which has the worst prognosis. The previous reports of 3-5 year median survival appears to be overoptimistic because other types with better prognosis like NSIP or BOOP might have been included. Therefore, this study was performed to determine the clinical course and the prognostic factors of UIP as diagnosed by surgical lung biopsy. Methods : The subjects were 72 UIP patients (age $58.2{\pm}11.6$ years, M : F=45 : 27, median follow up period : 18.1 months (0.7-103.6) diagnosed by surgical lung biopsy at the Asan Medical Center (68 patients) and the Paik Hospital in Seoul (4 patients). Clinical scores (level of dyspnea : 1-20 points), radiologic score (honeycombing : HC score 0-5 points, ground glass : GG score 0-5 points), and physiologic scores (FVC : 1-12 points, $FEV_1$ : 0-3 points, TLC : 0-10 points, $D_{LCO)$ : 0-5 points, $AaDO_2$ : 0-10 points) were summed into a total CRP score. Results : 1) The one year survival rate was 78.3%, while the rate for three year survival was 58.1%, and the median survival period was 42.5months. 2) Short term (1 year) prognosis : The patients who died within one year of diagnosis (14 patients) had the higher initial total CRP score ($28.6{\pm}8.3$ vs. $16.6{\pm}9.7$) than those who lived longer than one year (46 patients). The difference in the total CRP score was attributed to the symptom score ($8.4{\pm}2.1$ vs. $5.7{\pm}3.9$) and the physiologic score ($15.7{\pm}7.1$ vs. $6.7{\pm}5.7$) including FVC, $D_{LCO)$ and $AaDO_2$. 3) Long-term (3year) prognosis : The total CRP score ($12.2{\pm}6.7$ vs. $28.7{\pm}7.9$ : including symptom score, FVC, $D_{LCO)$ and $AaDO_2$) at the time of diagnosis were also different for the long-term survivors and those who lived less than 3 years. 4) Cox regression analysis showed $D_{LCO)$ (${\geq}$60%) (Hazard ratio : 4.56, 95% CI : 2.30-16.04) was the independent prognostic factors of UIP (P<0.05). Conclusion : These results suggest that $D_{LCO)$ at the time of diagnosis seem to be a prognostic markers of biopsy-proven UIP.

• Tuberculosis and Respiratory Diseases
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• v.45 no.1
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• pp.128-139
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• 1998

Background: It is well known that various cytokines and growth factors secreted mainly from alveolar macrophages do the key role in the pathogenesis of IPF. But recently it has been known that structural cells like fibroblast can also release cytokines. So the phenotypic changes in fibroblasts of IPF may do a role in continuous progression of fibrosis. The aim of this study is to find out whether there is a change in the biologic properties of the lung fibroblasts of IPF. Subjects and Method: The study was done on 13 patients with IPF diagnosed by open or thoracoscopic lung biopsy and 7 control patients who underwent resectional surgery for lung cancer. Lung fibroblast cell lines (FB) were established by explant culture technique from the biopsy or resected specimen Result: Basal proliferation of the fibroblast of IPF(IFB) measured by BrdU uptake tended to be highter than control fibroblast(NFB) (0.212 0.107 vs $0.319{\pm}0.143$, p=0.0922), also there was no significant difference in proliferation after the stimulation with PDGF or 10% serum. On the contrary, the degree of inhibition in proliferation by PGE2 was significantly lower($33.0{\pm}13.1%$) in IFB than control($46.7{\pm}10.0%$, p=0.0429). The IFB secreted significantly higher amount of MCP-l($l574{\pm}1283$ pg/ml) spontaneously than NFB($243{\pm}100$ pg/ml) and also after the stimulation with TGF-$\beta$($3.23{\pm}1.31$ ng/ml vs $0.552{\pm}0.236$ ng/ml, p=0.0012). Similarly IL-8 and IL-6 seretion of IFB was significantly higher than NFB at basal state and with TGF-$beta$ stimulation. But after the maximal stimulation with IL-1,8, no significant difference in cytokine secretion was found between IFB and NFB. Conclusion : Above data suggest that the fibroblasts of IPF were phenotypically changed and these change may do a role in the pathogenesis of IPF.

• Tuberculosis and Respiratory Diseases
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• v.40 no.3
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• pp.283-291
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• 1993

Background: Long-term low flow oxygen therapy not only increases survival, but also improves the quality of life in patients with chronic obstructive pulmonary disease (COPD) with chronic hypoxemia. For the assessment and improvement of the status of home oxygen therapy, we analyzed clinical experience of 26 patients who have been administered low flow oxygen at home. Method: Twenty-six patients (18 men and 8 women) who have been received long-term oxygen therapy (LTOT) at home were examined. We reviewed physical characteristics, clinical history, pulmonary function test, ECG, arterial blood gas analysis, hemoglobin and hematocrit, types of oxygen devices, inhalation time per day, concentration of administered $O_2$, duration of $O_2$ therapy, and problems in the home oxygen therapy. Results: The underlying diseases of patients were COPD 14 cases, far advanced old pulmonary tuberculosis 9 cases, bronchiectasis 2 cases, and idiopathic pulmonary fibrosis 1 case. The reasons for LTOT at home were noted for cor pulmonale 21 cases, for dyspnea on exertion and severe ventilatory impairment 4 cases, and for oxygen desaturation during sleep 1 case. The mean values of aterial blood gas analysis before home oxygen therapy were $PaO_2$ 57.7 mmHg, $PaCO_2$ 48.2 mmHg, and $SaO_2$ 87.7%. And the mean values of each parameters in the pulmonary function test were VC 2.05 L, $FEV_1$ 0.92 L, and $FEV_1$/FVC% 51.9%. Nineteen patients have used oxygen tanks as oxygen devices, 1 patient oxygen concentrator, 2 patients oxygen tank and liquid oxygen, and other 4 patients oxygen tank together with portable oxygen. The duration of oxygen therapy was below 1 year in 3 cases, 1~2 years in 15 cases, 3~5 years in 6 cases, 9 years in 1 case, and 10 years in 1 case. All patients have inhalated oxygen with flow rate less than 2.5 L/min. And only 10 patients have inhalated oxygen more than 15 hours per day, but most of them short time per day. Conclusion: For the effective oxygen administration, it is necessary that education for long-term low flow oxygen therapy to patients, their family and neighbor should be done, and also the institutional backup for getting convenient oxygen devices is required.