• Tuberculosis and Respiratory Diseases
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• v.51 no.4
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• pp.354-363
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• 2001

Background : Although lung involvement has been reported in 5 to 46% of dermatomyositis/polymyositis(DM/PM) patients, reports of the condition in Korea are rare. This study evaluated the clinical features of lung involvement in DM/PM patients. Methods : The medical records, laboratory results and radiologic findings of 79 DM/pM patients, who attended the Seoul National University Hospital (SNUH) between 1989 and 1999, were reviewed retrospectively. Results : A total 79 patients of whom 24 patients(33%) showed lung involvement, were enrolled in this study. More patients with lung involvement were female(F:M=11:1), and older compared with those without lung involvement. Patients with lung involvement presented with dyspnea(79%), coughing(67%), an elevated ESR, and CK/LD. Anti-Jo 1 antibody test was positive in 30%, which is significantly higher in patients with lung involvement. A simple chest X-ray of the patients with lung involvement exhibited reticular opacity(50%), reticulonodular opacity(30%), patchy opacity(29%), nodular opacity(13%) and linear opacity(4%). HRCT(n=24) showed ground glass opacity(75%), linear or septal thickening(50%), patchy consolidation(42%), honey-combing(33%) and nodular opacity(17%). The pulmonary function test showed a restrictive ventilatory pattern(77%) and a lower diffusing capacity(62%). The patients were followed up during a mean duration of $30{\pm}28$ months. They were treated with steroid only(50%) or a combination of steroids and cytotoxic agents(46%). Muscle symptoms were improved in 89% with treatment, but an improvement in the respiratory symptoms or in the pulmonary function test was rare. Patients with lung involvement had a higher mortality rate(21%) than those without lung involvement(10%) during the follow-up periods. Conclusion : DM/PM patients with lung involvement were mostly female, older and had a higher positive rate Anti-Jo 1 antibodies, but there was no significant difference in prognosis.

• Tuberculosis and Respiratory Diseases
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• v.77 no.2
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• pp.85-89
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• 2014

Intrathoracic involvement of immunoglobulin G4 (IgG4)-related disease has recently been reported. However, a subset of the disease presenting as interstitial lung disease is rare. Here, we report a case of a 35-year-old man with IgG4-related lung disease with manifestations similar to those of interstitial lung disease. Chest computed tomography showed diffuse ground glass opacities and rapidly progressive pleural and subpleural fibrosis in both upper lobes. Histological findings showed diffuse interstitial lymphoplasmacytic infiltration with an increased number of IgG4-positive plasma cells. Serum levels of IgG and IgG4 were also increased. The patient was diagnosed with IgG4-related lung disease, treated with anti-inflammatory agents, and showed improvement. Lung involvement of IgG4-related disease can present as interstitial lung disease and, therefore, should be differentiated when evaluating interstitial lung disease.

• Tuberculosis and Respiratory Diseases
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• v.67 no.4
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• pp.345-350
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• 2009

Erdheim-Chester disease (ECD) is a proliferative non-Langerhans cell histiocytosis of multiple organs. This is a rare disease of unknown etiology with a high mortality. We present the case report of a 26-year-old man diagnosed with ECD. He was referred to our hospital with elevated levels of aminotransferases. Although the diagnosis was uncertain, the patient was lost to follow up at that time. One year later, the patient returned to the hospital with generalized edema. Although a specific bone lesion was not found, the patient was experiencing the following: glomerulonephritis, aplastic anemia, hepatitis, and lung involvement. A lung biopsy was performed: the immunohistochemical stain were positive for CD68 and negative for S-100 protein and CD1a. We diagnosed as the patient as havinf ECD. Approximately 50% of ECD cases present with extraskeletal involvement. ECD should be considered as part of the differential diagnosis when multiple organs are involved.

• Journal of Chest Surgery
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• v.44 no.6
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• pp.448-451
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• 2011

Microscopic polyangiitis (MPA) is a necrotizing vasculitis involving the small vessels without granulomatous inflammation. Most MPA initially presents with renal involvement without pulmonary involvement. Isolated and initially presenting alveolar hemorrhage is very rare. The patient was a 39-year-old female with a progressive cough, dyspnea, and blood-tinged sputum for the previous 5 days. We determined that her condition was MPA though VATS lung biopsy and renal biopsy. After 2 months of steroid therapy, the chest lesions had improved. We report here a rare case of MPA with isolated and initial involvement of the lung with a review of the literature.

• Oral Biology Research
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• v.42 no.4
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• pp.269-271
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• 2018

Ankylosing spondylitis (AS) is a chronic inflammatory joint disorder characterized by sacroiliitis, spondylitis and enthesitis. Patients suffering from AS may also have extra-articular symptoms, such as uveitis, bowl disease, heart, lung, skin, bone and kidney involvement, but vary widely in severity and prevalence. Facial manifestation of AS include eye involvement and temporomandibular joint involvement. In this study, a case of an AS that mimicked dental pain was presented.

• Tuberculosis and Respiratory Diseases
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• v.44 no.3
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• pp.669-676
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• 1997

Castleman's disease is uncommon lymphoproliferative disorder as giant lymph node hyperplasia and angiofollicular lymph node hyperplasia. Multicentric variant of Castleman's disease, plasma cell type has been described that has more generalized lymph node involvement as well as involvement of other organ systems than localized type. Multicentric plasma cell type is frequently accompanied by systemic manifestations, such as weight loss, lowgrade fever and weakness. But the reported cases of pulmonary parenchymal involvement are rare and have almost consisted of hyalinized granuloma adjacent to a bronchus. We report a patient with Castleman's disease of the lung, pathologically proven interstitial pulmonary involvement.

• IMMUNE NETWORK
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• v.15 no.4
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• pp.206-211
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• 2015

Pulmonary edema is a major cause of mortality due to acute lung injury (ALI). The involvement of protein kinase C-${\delta}$ (PKC-${\delta}$) in ALI has been a controversial topic. Here we investigated PKC-${\delta}$ function in ALI using PKC-${\delta}$ knockout (KO) mice and PKC inhibitors. Our results indicated that although the ability to produce proinflammatory mediators in response to LPS injury in PKC-${\delta}$ KO mice was similar to that of control mice, they showed enhanced recruitment of neutrophils to the lung and more severe pulmonary edema. PKC-${\delta}$ inhibition promoted barrier dysfunction in an endothelial cell layer in vitro, and administration of a PKC-${\delta}$-specific inhibitor significantly increased steady state vascular permeability. A neutrophil transmigration assay indicated that the PKC-${\delta}$ inhibition increased neutrophil transmigration through an endothelial monolayer. This suggests that PKC-${\delta}$ inhibition induces structural changes in endothelial cells, allowing extravasation of proteins and neutrophils.

• Tuberculosis and Respiratory Diseases
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• v.46 no.2
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• pp.281-284
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• 1999

The lung is one of the most frequent sites of metastasis for extrathoracic tumors. Certain malignancies show a particular tendency to metastasize to lung, and in 15 to 25% of these cases, lung involvement is the only manifestation of metastatic disease. The most common presentation is the form of multiple bilateral nodules or masses of varying sizes. Lymphangitic metastasis is comprising 6 to 8% of all pulmonary metastasis. A striking variant of parenchymal involvement occures when dyscohesive tumor cells fill alveoli to produce a tumoral pneumonia. Carcinomatous lymphangitis of prostate origin is infrequent and usually carries a poor prognosis, and tumoral pneumonia of it is extremely rare. We report herein a case of prostate carcinoma metastasizing to lung, simulates atypical pneumonia, and disclosed by acute chest illness.

• Tuberculosis and Respiratory Diseases
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• v.67 no.4
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• pp.359-363
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• 2009

Immunoglobulin G4 (IgG4) related autoimmune diseases are characterized by high serum IgG4 concentrations, sclerosing inflammation of numerous IgG4-positive lymphoplasma cells of varying origin, and a positive response to steroid treatment. Autoimmune pancreatitis, sclerosing cholangitis, and retroperitoneal fibrosis are representative presentations of IgG4 related autoimmune disease. Herein, we describe 2 patients (40-years-old woman and 47-years-old man) diagnosed with pulmonary involvement of IgG4-related autoimmune disease. The patients were admitted for an evaluation of the lung mass or multiple lung nodules found on chest radiography. Surgical lung biopsies were performed and pathologic finding revealed lymphoplasmacytic sclerosing inflammation with numerous IgG4 positive cells. The patients had elevated serum total IgG and IgG4 levels. Treatment consisted of high dose methylpredinisolone (1 mg/kg/day) and demonstrated good responsiveness. However, one patient experienced 2 relapses while being tapered off of steroid treatment.

• Tuberculosis and Respiratory Diseases
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• v.51 no.6
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• pp.597-602
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• 2001

A 47-years-old woman presented with a 2-month history of a dry mouth and dry cough. The patient had been taking medication for Sj$\ddot{o}$gren's syndrome for approximately 7 years. The chest radiography showed multiple cystic lesions and a hazy density in both lower lung fields. The HRCT showed a diffuse ground glass like appearance and multiple variable sized cystic lesions in both lung fields. After medication, the symptoms were aggravated. Bronchoscopy was preformed with a transbronchial lung biopsy. The biopsies showed an infiltration of lymphocytes, neutrophils, monocytes and histiocytes through the interstitial space of the alveola and a widening of the alveolar septa. However, the histological findings of the cysts were not obtained. Sj$\ddot{o}$gren's syndrome is a slowly progressive inflammatory autoimmune disease, which is characterized by lymphocyte mediated destruction of the exocrine glands, with pulmonary involvement in approximately 19-65%, High-resolution CT is a sensitive technique for assessing the pulmonary involvement in patients with Sj$\ddot{o}$gren's syndrome. Although a lung biopsy is not always necessary for establishing a diagnosis of an interstitial lung disease in Sj$\ddot{o}$gren's syndrome. A lung biopsy may reveal a wide spectrum of changes ranging from a mild inflammatory response to end stage fibrosis with honeycombing. Because of the predominantly peribronchiolar inflammatory infiltration and inspissated secretions the cysts were suspected to have been formed by the ball-valve phenomen. However, no definite evidence was obtained.