• Tuberculosis and Respiratory Diseases
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• v.55 no.2
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• pp.175-187
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• 2003

Background : Although corticosteroid and cytotoxic agent such as cyclophosphamide have been used for the treatment idiopathic interstitial pneumonia (IIP), efficacy of these toxic drugs are unclear because previous reports included the patients who did not undergo surgical lung biopsy and none evaluated the response according to histopathologic entities of IIP. To answer this, we retrospectively analyzed the treatment response and side effects of corticosteroids and cyclophosphamide therapy in patients with idiopathic UIP and NSIP. Methods : Among 61 patients with UIP and 26 patients with NSIP diagnosed by surgical lung biopsy at Samsung Medical Center from July 1996 to June 2002, those who received corticosteroid or cyclophosphamide therapy for at least 6 months and were followed for at least one year after the initiation of treatment were enrolled (32 UIP, 23 NSIP). Treatment response of 55 patients was assessed by ATS response criteria (clinical symptoms, pulmonary function test and radiological findings). Adverse reactions to either agent (42 cases of cyclophosphamide${\pm}$low-dose prednisolone, 49 cases of prednisolone alone) were also analyzed. Results : Irrespective of treatment regimen, NSIP showed more favorable response than UIP (6 months: 78.3% vs. 9.4%, 12 months: 69.6% vs. 9.4%, p<0.001). Cyclophosphamide showed comparable response to corticosteroid in NSIP while its efficacy was as poor as those of corticosteroid therapy in UIP. Significant adverse reaction to drug more frequently occurred in corticosteroid group (35.7%) than cyclophosphamide group (14.3%) (p=0.017). Conclusion : Cyclophosphamide is effective and more tolerable than corticosteroids in the treatment of idiopathic nonspecific interstitial pneumonia.

• Journal of Chest Surgery
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• v.29 no.7
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• pp.741-746
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• 1996

Diffuse interstitial lung disease due to a wide variety of conditions are amenable to diagnosis by means of clinical evaluation, bronchoalveolar lavage, transbronchial biopsy and lung biopsy. The recently introduced technique of thoracoscopic wedge biopsy provides the potential advantages of greater selection of biopsy site and reduced postoperative chest pain compared with the standard open lung biopsy Video assisted thoracoscopic lung biopsy was performed in twenty patients for diagnosis of diffuse in- terstitial lung disease during the period from January 1993 to June 1994. The ages of the patients ranged from 18 to 69 years(mean 48.5 years), and the patients consisted of 10 males and 10 females. In all twenty, thoracoscpic lung biopsy samples were obtained; 9 from left lower lobe, 7 from right lower lobe, 2 from right upper lobe, 1 from left upper lobe, and 1 from right middle lobe. All patients were correctly diagnosed. The two common diseases were usual in erstitial pneumonia(UIP)(8120) and mil- liary tuberculo si s(4120) . Mean duration of the chest drainage was 3.2 days. There was no complication.

• Tuberculosis and Respiratory Diseases
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• v.53 no.3
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• pp.309-318
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• 2002

Background : Usual interstitial pneumonia (UIP) is a fatal progressive fibrotic disorder of the lung with unknown etiology and characterized by a poor response to conventional immunosuppressive therapy. The histologic hallmark of UIP is parchy distribution of subpleural fibrosis and fibroblastic foci(FBF) with interposed normal appearing lung. Because FBF is a collection of actively proliferating myofibroblasts, it can be a marker of activity and prognosis of UIP. However, there were contradictory reports about the correlation between the degree of FBF and survival. Therefore we performed this study to investigate the value of FBF as prognostic marker of UIP. Methods : This was a retrospective study on the 46 patients(M:F=33:13, mean age:$59{\pm}12$ years) with UIP diagnosed by the surgical lung biopsy at the Asan Medical Center, Seoul, Korea between 1990 and 2000 and had follow-up of more than a year. All the biopsy specimens were reevaluated and diagnosed as UIP according to the ATS/ERS classification. Semiquantitative grading of FBF(absent, 0; mild 1; moderate 2; marked 3) by the experienced pathologists who did not know the clinical findings were compared to the clinical data and the follow up course. Results : Thirteen patients(28.2%) died of UIP progression during the study period. The median survival time of all the subjects was 26 months after the biopsy. At the univariate analysis, FVC, $D_Lco$, smoking history and the grade of FBF were significantly related to the survial. The survival was longer in subjects with lesser degrees of FBF, higher DLco, higher FVC and history of smoking. However the multivariate analysis with Cox regression test showed the extent of FBF was the only independent prognostic marker of UIP. Conclusion : These data suggested that the extent of FBF on the surgical lung biopsy can be used as a prognostic marker of UIP.

• Tuberculosis and Respiratory Diseases
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• v.51 no.6
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• pp.540-549
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• 2001

Background : Usual interstitial pneumonia (UIP) is a progressive fibrous lung disease with occasional fatal outcomes. However, the extent and rate of progression varies markedly from one patient to another. As a result, it is difficult to determine the time of the initial treatment and assess the disease activity and course. Fibroblast foci (FF) is well known to synthesize collagen actively by their myofibroblasts component. However, the prognostic value of the FF have not been evaluated in patients with VIP. Therefore this study was undertaken to determine how the number of fibroblastic foci can reflect the disease activity and progression. Methods: Twenty patients with UIP(M : F=13 : 7), who were diagnosed by a surgical lung biopsy. The number of fibroblastic foci was analyzed in terms of its correlation with the clinical manifostations, pulmonary function test, arterial blood gas analysis, and a bronchoalveolar lavage(BAL). Results : The number of fibroblastic foci did not correlate with the various lung function tests and the other clinical parameters. Interestingly, the percentage of neutrophils in the bronchoalveolar lavage fluid did correlate with the quantity of the normalized Vv of FF(r=0.60, p<0.05). The patients were divided into 2 groups, group I and II, arbitrarily, according to the value of the normalized Vv. The clinical parameters and the PIT results were not different between the two groups. In particular, the survival rate between the two groups according to the Kaplan-Meier analysis were not different. Conclusion : A large number of FF does not imply a bad prognosis in patients with UIP.

• Tuberculosis and Respiratory Diseases
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• v.45 no.2
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• pp.465-469
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• 1998

Acute respiratory failure with diffuse pulmonary infiltration was occurred in a patient with malignant lymphoma 1month after the $8^{th}$ CHOP chemotherapy. The ground glass and consolidation appearances on chest C-T in this immunodeficient patient could be presented in many clinical situations such as pneumonia by opportunistic infections (fungal, parasites, viral, and usual bacterial pathogens), anti-tumor drug's pulmonary toxicity and tumor invasion. And the other diseases of acute interstitial pneumonitis, alveolar proteinosis, BOOP, puhnonary edema and alveolar hemorrhage, which could present the same radiological findings, should included in differential diagnosis. This patient was diagnosed as the opportunistic pneumonia by Pneumocystis carinii and probably Cytomegalovirus through bronchoalveolar lavage and transbronchial lung biopsy

• Journal of Chest Surgery
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• v.24 no.9
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• pp.903-906
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• 1991

Retrospective review of 26 patients undergoing open lung biopsy at the Yonsei University during 10 years period was conducted to evaluate open lung biopsy for DILD. From January 1980 to August 1990, open lung biopsy was performed in 26 patients through a limited thoracotomy incision[a limited anterior or a posterolateral thoracotomy]. Open lung biopsy was indicated for diffuse interstitial pulmonary diseases undiagnosed by indirect clinical and radiological diagnostic methods. The types of incision were limited anterior[11] and limited posterolateral[15]. Preoperative evaluation of the lung disease included sputum culture[26], sputum cytology [19], bronchoscopy[9] and TBLB[7]. In 23 patients the histologic appearances after open lung biopsy were sufficiently specific histologic pictures to confirm diagnosis. The results of the biopsies changed usual therapeutic plan in 17 patients among them. The complications were resp. insufficiency[3], pulmonary ed6ma[3], sepsis[2], and others[3] in 6 patients. Diagnosis from the open lung biopsy was included respiratory pneumonia[7], fibrosis[7], infection[5], malignancy[2], others[5]. 4 patients died of respiratory insufficiency. The causes of the other three death were not due to direct result of the biopsy itself. Open lung biopsy in the patient with a diffuse infiltrative lung disease is an one of the accurate diagnostic method and frequently leads to change of the therapeutic plans. So we conclude that open lung biopsy remains our diagnostic method of choice in diffuse infiltrative lung disease undetermined etiology.

• Tuberculosis and Respiratory Diseases
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• v.46 no.5
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• pp.685-696
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• 1999

Background : Idiopathic pulmonary fibrosis (IPF) is a diffuse inflammatory and fibrosing process that occurs within the interstitium and alveolus of the lung with invariably poor prognosis. The major problem in management of IPF results from the variable rate of disease progression and the difficulties in predicting the response to therapy. The purpose of this retrospective study was to evaluate the short-term efficacy of steroid and immunosuppressive therapy for IPF and to identify the pre-treatment determinants of favorable response. Method : Twenty patients of IPF were included. Diagnosis of IPF was proven by thoracoscopic lung biopsy and they were presumed to have active progressive disease. The baseline evaluation in these patients included clinical history, pulmonary function test, bronchoalveolar lavage (BAL), and chest high resolution computed tomography (HRCT). Fourteen patients received oral prednisolone treatment with initial dose of 1mg/kg/day for 8 to 12 weeks and then tapering to low-dose prednisolone (0.25mg/kg/day). Six patients who previously had experienced significant side effects to steroid received 2mg/kg/day of oral cyclophosphamide with or without low-dose prednisolone. Follow-up evaluation was performed after 6 months of therapy. If patients met more than one of followings, they were considered to be responders : (1) improvement of more than one grade in dyspnea index, (2) improvement in FVC or TLC more than 10% or improvement in DLco more than 20% (3) decreased extent of disease in chest HRCT findings. Result : One patient died of extrapulmonary cause after 3 month of therapy, and another patient gave up any further medical therapy due to side effect of steroid. Eventually medical records of 18 patients were analyzed. Nine of 18 patients were classified into responders and the other nine patients into nonresponders. The histopathologic diagnosis of the responders were all nonspecific interstitial pneumonia (NSIP) and that of nonresponders were all usual interstitial pneumonia (UIP) (p<0.001). The other significant differences between the two groups were female predominance (p<0.01), smoking history (p<0.001), severe grade of dyspnea (p<0.05), lymphocytosis in BAL fluid ($23.8{\pm}16.3%$ vs $7.8{\pm}3.6%$, p<0.05), and less honeycombing in chest HRCT findings (0% vs $9.2{\pm}2.3%$, p<0.001). Conclusion : Our results suggest that patients with histopathologic diagnosis of NSIP or lymphocytosis in BAL fluid are more likely to respond to steroid or immunosuppressive therapy. Clinical results in large numbers of IPF patients will be required to identify the independent variables.

• Journal of Chest Surgery
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• v.30 no.12
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• pp.1232-1236
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• 1997

Diffuse lung disease is amenable to diagnosis by means of clinical evaluation, bronchoalveolar lavage, transbronchial biopsy, and lung biopsy. The recently introduced technique of thoracoscopic wedge biopsy provides the potential advantages of greater selection of biopsy sites and reduced postoperative pain compared with those associated with standard open lung biopsy. Video assisted thoracoscopic lung biopsy was performed in 22 patients for the diagnosis of diffuse lung disease during the period from August 1994 to December 1996, and the following results were obtained: 1. The patients were 14 men and 8 women. The average age was 54.6 years. 2. 13 lung biopsy specimens were obtained from the right lower lobe, 4 from he right upper lobe, 3 from the right middle lobe, 3 from the left upper lobe, and 3 from the left lower lobe. 3. A comparison of pulmonary function tests between preoperative and postoperative values showed no significant differences in FVC, FEVI, and FEVl/FVC(p>0.05). All patients were pathologically diagnosed and the most common disease was usual interstitial pneumonia(8/22). In conclusion, thoracoscopic lung biopsy was a safe and effective diagnostic method of diffuse lung disease.

• Tuberculosis and Respiratory Diseases
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• v.70 no.3
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• pp.257-260
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• 2011

A 65-year-old woman was admitted due to poor oral intake and a dry cough over the previous 3 months. The physical examination was remarkable for bibasilar crackles, and plain chest radiography showed reticulation in both lower lung fields. A pulmonary function test demonstrated a restrictive pattern with a reduced diffusing capacity of the lung for carbon monoxide. High resolution computed tomography showed reticulation and honey-combing in both peripheral lung zones, which was consistent with usual interstitial pneumonia pattern. Her skin showed livedo reticularis. The erythrocyte sedimentation rate and C-reactive protein level were elevated, and hematuria was noted on urinary analysis. A serologic test for auto-antibodies showed seropositivity for Myeloperoxidase-Anti-neutrophil cytoplasmic antibody (MPO-ANCA). A kidney biopsy was performed and showed focal segmental glomerulosclerosis. She was diagnosed as having pulmonary fibrosis with microscopic polyangiitis (MPA) and treated with high dose steroids. Here we report a case of pulmonary fibrosis coexistent with microscopic polyangiitis.

• Tuberculosis and Respiratory Diseases
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• v.43 no.3
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• pp.388-402
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• 1996

Background : To compare the diagnostic accuracies of High-resolution CT(HRCI) and chest radiography in the diagnosis of diffuse infiltrative lung disease(DILD). Methods : This study included ninety-nine patients with a diagnosis of acute or chronic DILD, representing 20 different diseases. Twelve normal subjects were included as control. The disease state was confirmed either pathologically or clinically. Radiographs and CT scans were evaluated separately by three independent observers without knowledge of clinical and pathologic results. The observers listed three most likely diagnoses and recorded degree of confidence. Results : The sensitivity of HRCT in the detection of DILD was 98.9% compared to 97.9% of chest radiography. Overall, a correct first-choice diagnosis was made in 48% using chest radiographs and in 60% using HRCT images. The correct diagnosis was among the top-three choices in 64% when chest radiographs were used, and in 75% when HRCT images were reviewed. Overally a confident diagnosis was reached more often with HRCT(55%) than with chest radiography(26%). The correct first-choice diagnosis increased remarkably when the HRCT was used in usual interstitial pneumonia, miliary tuberculosis, diffuse panbronchiolitis and lymphangitic carcinomatosis. Conclusion : HRCT is confirmed to be superior to conventional radiography in the detection and accurate diagnosis of DILD. HRCT is especially valuable in the diagnosis of usual interstitial pneumonia, miliary tuberculosis, diffuse panbronchiolitis, and lymphangitic carcinomatosis.