• Journal of Yeungnam Medical Science
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• v.39 no.2
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• pp.153-160
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• 2022

Multicentric Castleman disease (MCD) is an uncommon systemic lymphoproliferative disorder that may cause multiple organ damage. Castleman disease-associated diffuse parenchymal lung disease (DPLD) has not been well studied. A 32-year-old man was referred to our hospital for progressive generalized weakness, light-headedness, and dyspnea on exertion for more than one year. Laboratory evaluations showed profound anemia, an elevated erythrocyte sedimentation rate, and an increased C-reactive protein level with polyclonal hypergammaglobulinemia. Chest radiography, computed tomography (CT), and positron emission tomography-CT scan demonstrated diffuse lung infiltration with multiple cystic lesions and multiple lymphadenopathy. In addition to these clinical laboratory findings, bone marrow, lung, and lymph node biopsies confirmed the diagnosis of idiopathic MCD (iMCD). Siltuximab, an interleukin-6 inhibitor, and glucocorticoid therapy were initiated. The patient has been tolerating the treatment well and had no disease progression or any complications in 4 years. Herein, we report this case of human herpesvirus-8-negative iMCD-associated DPLD accompanied by multiple cystic lesions, multiple lymphadenopathy, and polyclonal hypergammaglobulinemia with elevated immunoglobulin G (IgG) and IgG4 levels. We recommend a close evaluation of MCD in cases of DPLD with hypergammaglobulinemia.

• Tuberculosis and Respiratory Diseases
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• v.42 no.4
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• pp.624-628
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• 1995

Lymphangioleiomyomatosis is a rare disease which afflicts young women of childbearing age. We experienced a 32-year-old female who was admitted because of worsening exertional dyspnea after hormonal therapy with Clomifen for five months and intermittent hemoptysis. Chest PA showed diffuse ground glass appearance with some reticular infiltrations. High resolution CT scan showed multiple small thin walled cysts distributed homogeneously throughout the entire lung. Pulmonary function test showed characteristic obstructive pattern despite the restrictive interstitial pattern of chest radiography. Thoracoscopic lung biopsy specimen showed abnormal proliferation of smooth muscle cells in the walls of lymphatic vessels, bronchioles, and pulmonary vessels typical of pulmonary lymphangioleiomyomatosis. Hormonal therapy with medroxyprogesterone was initiated.

• Tuberculosis and Respiratory Diseases
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• v.56 no.3
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• pp.315-320
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• 2004

Gemcitabine is an effective newly developed chemotherapeutic agent, which is increasingly being used to treat non-small cell lung, ovarian and breast cancers. Pulmonary toxicity is usually self-limiting mild dyspnea, bronchospasm, but severe pulmonary toxicity is rarely reported. Herein, we report drug induced interstitial lung disease associated with gemcitabine treatment. High resolution computerized tomogram (HRCT) showed an increased ground glass opacity and thickened septal lines. The patient showed a rapid good response with prednisolone treatment.

• Tuberculosis and Respiratory Diseases
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• v.65 no.4
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• pp.323-327
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• 2008

Systemic lupus erythematosus (SLE) is a multisystem disorder where the etiology is not clearly known. Symptomatic chronic interstitial pneumonitis is an uncommon manifestation, with a reported prevalence of 3~13%. Achalasia is rare disease that presents with failure in the relaxation of the esophagus sphincter. A 22-year-old woman was admitted to our hospital because of fever, cough and dyspnea. The patient had a history of pericardial effusion and Raynaud's phenomenon. The results of laboratory tests indicated the presence of lymphopenia and included positive antibody tests for antinuclear antibody and anti Sm antibody. A chest X-ray demonstrated the presence of peribronchial infiltration on both lung fields. A Chest CT image showed interlobar septal thickening, ground-glass opacity and a honeycomb appearance in both lung fields and esophageal dilatation with air fluid level. An esophagogram showed the presence of dilated esophagus ends that represented the non-relaxed lower esophageal sphincter. Manometry demonstrated incomplete sphincter relaxation. The case was diagnosed as systemic lupus erythematosus associated with interstitial pneumonia and achalasia.

• Tuberculosis and Respiratory Diseases
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• v.42 no.4
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• pp.548-554
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• 1995

Background: Cardiogenic pulmonary edema increases nonspecific airway responsiveness in humans and animals. Increased extravascular lung water from overt pulmonary edema to subclinical interstitial edema is a common finding in patients with chronic renal failure. Several studies carried out to assess pulmonary function disturbances in this condition have documented a reduction in forced expiratory volume that usually reverses after hemodialysis, suggesting airway edema as the underlying mechanism. This interstitial edema may also lead to nonspecific bronchial hyperresponsiveness. We hypothesized that patients with chronic renal failure may present nonspecific bronchial hyperresponsiveness due to subclinical interstitial pulmonary edema. Methods: We studied 18 chronic renal failure undergoing regular hemodialysis 3 times a week(New York Heart Association Class II) without concomittent disease. These patients were checked pulmonary function test and metacholine provocation test before hemodialysis and same procedure was repeated if responsive, after hemodialysis. Results: 1) 12 out of 18 patients before hemodialysis were reactive in metacholine provocation test(66.7%) before hemodialysis. This airway hyperresponsiveness were decreased after hemodialysis. 2) Pulmonary function was improved after hemodialysis and change in $FEV_1$ was correlated with change in weight(r=-0.62, p<0.01). 3) There was a close correlation between log $PD_{20}$ and $FEF_{25}$, which is one of the variables of the peripheral airways(r=0.58, p<0.05). Conclusion: We speculated interstitial pulmonary edema may play a significant role in bronchial hyperresponsiveness and lung function impaired in patients with chronic renal failure.

• Tuberculosis and Respiratory Diseases
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• v.40 no.5
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• pp.602-609
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• 1993

Lymphocytic interstitial pneumonitis (LIP) is one of parenchymal pulmonary infiltrative diseases first described at 1966 by Carrington and Liebow. In LIP, there is a predominance of mature small lymphocytes in the interstitium of the lung which form germinal centers. The disease process surrounds, but dose not invade lung parenchyme, tracheobronchial tree and vascular structures. The etiology remains still unknown and the clinical features of this disorder have not been clearly defined. Therefore, the therapeutic modality is obscure. Development of LIP association with AIDS is often reported currently and possibility of progress to malignant lymphoma is emphasized. We experienced a case of primary LIP with pnemomediastinum. She was adimitted due to chest and anterior nuchal pain with chronic coughing, and diagnosed as pneumomediastinum with LIP. Medication with steroid was begun and some improvement of symptoms was observed, but an X-ray film of the chest remained same without improvement. We report above case with review of the literatures.

• Tuberculosis and Respiratory Diseases
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• v.84 no.3
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• pp.200-208
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• 2021

Background: Hypersensitivity pneumonitis (HP) is an increasingly recognized form of diffuse parenchymal lung disease. Krebs von den Lungen-6 (KL-6) is now classified as a human MUC1 mucin protein, and regenerating type II pneumocytes are the primary cellular source of KL-6/MUC1 in the affected lungs of patients with interstitial lung diseases (ILD). Serum KL-6/MUC1 levels have been demonstrated to be useful for the evaluation of various ILD. To determine the role of circulating KL-6 in evaluating the disease activity and management of HP. Methods: An observational cross-sectional study was conducted on 51 patients with HP and 20 healthy controls. Serum KL-6 levels were measured in both groups. Patients were further assessed based on chest high-resolution computed tomography (HRCT), pulmonary function test, 6-minute walk test, echocardiography, bronchioalveolar lavage, and/or transbronchial biopsy. Patients were divided into the fibrotic and non-fibrotic groups according to the HRCT findings. Results: The median serum KL-6 levels were significantly higher in HP patients as compared to the control group. The median serum KL-6 levels were found to be higher in the non-fibrotic HP group (1,900 IU/mL) as compared to the fibrotic group (1,200 IU/mL). There was a significant inverse correlation between serum KL-6 serum level and the dose of steroids as well as the duration of steroid therapy. Conclusion: The presence of higher KL-6 levels in the non-fibrotic HP group implies its enhanced production by regenerating pneumocytes in response to alveolar injury. The significant association between serum KL-6 levels and the dose and the duration of steroid therapy emphasizes the significant role of steroids in the stabilization of the disease.

• Tuberculosis and Respiratory Diseases
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• v.57 no.1
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• pp.72-77
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• 2004

Background : A tracheobronchial obstruction in lung cancer is associated with significant morbidity and mortality due to dyspnea, cough, hemoptysis, and recurrent respiratory infection. It is well known that one of standard treatments is photodynamic therapy (PDT) in tracheobronchial obstruction after radiotherapy, chemotherapy, and/or surgery. We reported here the role of PDT in airway obstruction in patients advanced lung cancer. Method : Pre-treatment protocol consisted of clinical, radiologic, and bronchoscopic examination, pulmonary function test, and assessment of Karnofsky performance status. A 2 mg/kg of porfimer sodium was injected intravenously, and then followed by cylindrical and/or interstitial irradiation with 630 nm of laser after 48 hours. The repeated bronchoscopy for debridement of necrotic tissue and re-illumination was performed after 48 hours. Result : Improved airway obstruction and selective tumor necrosis were achieved by photodynamic therapy in all cases. Dyspnea and performance status were improved in three cases. A purulent sputum, fever and hemoptysis were improved in one of five cases. After PDT, all patients showed temporarily aggravation of dyspnea, two of five showed febrile reaction for a few days and nobody presented photosensitivity reaction, hemoptysis and respiratory failure. Conclusion : Our experiences of PDT are effective in palliation of inoperable advanced lung cancer in terms of tracheobronchial obstruction.

• Tuberculosis and Respiratory Diseases
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• v.50 no.3
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• pp.367-372
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• 2001

Bacillus Calmette-Guerin(BCG) has been widely used for the prophylaxis of superficial bladder tumor recurrence and for the treatment of bladder carcinoma in situ. More than 95% of patients who receive BCG instillation tolerate the treatment well and side reactions have been reported in less than 5% of patients. Most side effects are minor and self-limiting. However, a rare occurrence of severe systemic reactions have been reported. Among the severe systemic reactions, hypersensitivity pneumonitis should be considered in patients with pneumonic complications after BCG instillation in cases where the culture for mycobacteria is negative in the sputwn, brochoalveolar lavage and blood specimen. In addition, a fiberoptic bronchoscopy with transbronchial lung biopsy demonstrates a fibrosis of the alveolar septums, where there is and an increased lymphocyte count with out tuberculous inflammatory changes, the and CD4 : CD8 ratio is increased and no symptomatic response to antituberculosis chemotherapy is observed. Here we report a 68 years old man with interstitial pneumonitis following intravesical BCG instillation.

• Journal of the Korean Society of Radiology
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• v.83 no.5
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• pp.1175-1181
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• 2022

Progressive massive fibrosis (PMF) with atypical findings is often misdiagnosed as lung cancer. Atypical features of PMF have been described in some reports; however, these reports only introduced their cases with a short literature review. We report two cases of solitary PMFs with no underlying simple pneumoconiosis or rapid growth at atypical location that were mistaken for lung cancer. We also suggest the useful CT findings to aid in the differential diagnosis.