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Tuberous sclerosis is a syndrome characterized by the facial skin lesion, epilepsy, and mental retardation. Pulmonary involvement is uncommon, but when the lung is involved by tuberous sclerosis it shows characteristic reticulonodular infiltration and cystic changes. We experienced a 34-year-old female patient of known tuberous sclerosis admitted due to pneumothorax. Chest PA showed diffuse fine reticulonodular infiltration and chest CT revealed diffuse cystic changes of the lung. Pleurodesis with tetracycline was done to prevent recurrence, but chylothorax occurred four months after the treatment. Pleurosis was tried again and anti estrogen treatment began.

Variety of pulmonary lesions are thought to be associated with rheumatoid arthritis (RA). These lesions traditionally have included pleurisy with or without effusion, Caplan’s syndrome, pulmonary rheumatoid nodules, diffuse interstitial fibrosis, and pulmonary arteritis and hypertension. But little attention has been paid to the airways in RA. Recently, several repots have suggested an association between airflow limitation and RA, but its incidence is not known. Also whether there exists a parameter of disease activity of RA, suggesting the presence of small airway disease (SAD) is not clear. To answer these questions, the serologic parameters which reflect the disease activity of RA and pulmonary function tests which reflect small airway dysfunction were performed on 36 lifetime nonsmokers with RA who had normal chest x-ray findings. The prevalence of SAD and the relationships between the disease activity parameters of RA and pulmonary function were observed. The results were as follows. The percentages of patients with abnormal values for diffusing capacity, frequency dependence of compliance (C1.0/C0.0), forced expiratory flow25–75%, Vmax50% and Vmax75% were 45.5%, 62.5%, 40%, 22.8% and 11.4%, respectively. There was statistically significant negative correlation between C1.0/C0.0 and ESR. But consistent correlation between other pulmonary function tests and clinical and serologic parameters of RA, and differences in pulmonary function between patients who were serologically positive and negative for CRP and FANA, were not found. In conclusion, SAD, without the influence of smoking, is frequently associated with RA, but, the presence of SAD cannot be predicted from any clinical and seologic parameters of RA currently in use.

Background: Although there have been many studies on the pathogenetic mechanism of acute lung injury, it is still elusive. Recently interests have been focused on the role of oxygen free radicals. But the effect of hydroxyl radical on the neutrophil mobilization and the alveolar-capillary permeability is not clear especially in the endotoxin-induced acute lung injury model of rats. This investigation was performed to evaluate the pathogenetic role of hydroxyl radical on the neutrophil accumulation into the lung and the increased alveolar-capillary permeability in the endotoxin-induced acute lung injury in rats. Method: Fifty rats were divided into four groups: vehicle control group (n=5, 6hrs; n=5, 24hrs), endotoxin-treated group (n=10, 6hrs; n=10, 24hrs), Dimethylthiourea (DMTU)-pretreated group (n=10, 6hrs), and deferoxamine (DFX)-pretreated group (n=10, 6hrs). Thirty minutes before sacrifice, -tagged bovine serum albumin was injected. Six and twenty four hours after endotoxin injection, the rats were sacrificed, and the radioactivity of lung tissue and peripheral blood was counted. Permeability index was defined as the ratio of radioactivity between lung tissue and peripheral blood. Another set of rats (n=52) were divided into the same four groups as before [vehicle control group (n=5, 6hrs; n=5, 24hrs), endotoxin·treated group (n=7, 6hrs; n=8, 24hrs), DMTU-pretreated group (n=6, 6hrs; n=9, 24hrs), and DFX-pretreated group (n=5, 6hrs; n=7, 24hrs)], and were sacrificed 6 and 24 hours after endotoxin injection. In these rats, cell profile of peripheral blood and bronchoalveolar lavage fluid was evaluated, and the pathologic examination of lung tissue was performed. Results: 1) Increased alveolar-capillary permeability was observed 6 hours after endotoxin injection, which was normalized after 24 hours, and this increase was attenuated by pretreatment with DMTU and DFX. 2) Neutrophil sequestration into the lung was observed 24 hours after endotoxin administration, but this was not influenced by DMTU and DFX pretreatment. Conclusion: These results suggest that hydroxyl radical would not be involved in the sequestration of neutrophils into the lung, but plays an important role in the increase of alveolar-capillary permeability in the endotoxin-induced acute lung injury in rats.

Diffuse panbronchiolitis (DPB), which is prevalent in Japan, is known to be rare outside Japan. Although a case of diffuse panbronchiolitis in a second generation Korean was reported in Japan in 1986, no case has been reported in Korea. Recently we found 5 cases of diffuse panbronchiolitis in Korea, two histologically proven and three clinically and radiologically suspected. All 5 cases had the typical respiratory symptoms and signs and a history of chronic sinusitis. In three clinically and radiologically suspected cases, high resolution computed tomography showed the typical findings of DPB and other diseases such as pulmonary emphysema, bronchial asthma, chronic bronchitis and bronchiectasis could be ruled out. More cases of DPB are expected to be found in Korea in the near future.(Internal Medicine 31 : 695-701, 1992)

Dark-blue pigmentation has been thought to be related to smoking or occupational exposure and has been regarded as anthracotic pigmentation. It is also frequently observed in non-smokers without occupational exposure, but there is no proven mechanism of pigmetation. To investigate clinical features and to find other causes of dark-blue pigmentaion, retrospective analysis was done in 59 patients who showed anthracotic pigmentation on bronchoscopy during recent 5 years in Seoul National University Hospital. The results were as follows; 1) Forty cases were non-smokers, while smokers were 19 cases. 2) Fifteen cases had history of tuberculosis, but there was no history of environmental exposure. 3) Mediastinal calcification was observed in 89.7%. 4) There was significant bleeding without exception when biopsy was done at the pigmentation site. 5) In patients with pigmentation only, hemoptysis and productive cough were main chief complaints, and chest X-ray showed atelectasis, infiltration, mass, or pleural change. 6) The number of patients whose lesion of X-ray corresponds to pigmentation site were 19/30 in tuberculosis, 4/30 in DILD and 7/30 in other diseases.

Endobronchial tuberculosis is a serious disease which is frequently complicated with bronchial stenosis, and it may simulate a bronchial asthma or bronchogenic carcinoma. We have analyzed the clinical characteristics of endobronchial tuberculosis in 166 patients, and we introduce new classification of endobronchial tuberculosis. Endobronchial tuberculosis is classified into seven subtypes as stenotic type with fibrosis, stenotic type without fibrosis, actively caseating type, tumorous type, ulcerative type, granular type and nonspecific bronchitic type, according to the bronchoscopic findings. Actively caseaing type, stenotic type without fibrosis, nonspecific bronchitic type and stenotic type with fibrosis are predominant in the order of frequency, but ulcerative type and granular type are relatively rare. Stenotic type with or without fibrosis, actively caseating type and tumorous type of endobronchial tuberculosis are closely related to bronchial stenoses. We believe that our new classification of endobronchial tuberculosis, which is based on bronchoscopic features, is helpful to understand the pathogenesis of disease and to make a therapeutic plan for preventing or minimizing bronchial stenosis.

Intravascular lymphomatosis(IVL) which was first described by pfleger and Tappeiner in 1959 is rare malignancy characterized by neoplastic proliferation of lymphoid cell lineage within the vascular lumen with little or no adjacent parenchymal involvement Its usual sites of involvement are central nervous system and skin or infrequently heart, lungs, pancreas, liver, spleen, kidney, adrenal glands, genitourinary tract, and bone marrow. Pulmonary involvement of IVL is not common. Symptoms of pulmonary involvement include dyspnea, cough and fever. Radiologicially, the disease is manifested with diffuse interstitial infiltrates. We report a recently experienced case of pulmonary intravascular lymphomatosis which was manifested with fever and chest pain.

Fibrosing mediastinitis is a rare disease which is characterized by excessive fibrosis of mediastinum and symptoms caused by compression and obstruction of mediastinal structures. Although the pathogenesis of this disease is unknown, granulomatous infection is cinsidered to be the most common cause of this disease. Histoplasmosis is the most common etiology, especially in the endemic areas in United States. Tuberculosis is another etiology of fibrosing mediastinitis. We experienced two cases of fibrosing mediastinitis associated with tuberculous infection.

Hamartoma is one of the most common benign lung tumors. Most of them are located in the lung parenchyme, but very rarely it can originate endobronchially. We report a case of endobronchial hamartoma in a 59 year old woman and a review of the literature.

Alveolar hemorrhage is a life threatening condition which requires an urgent diagnosis and treatment. We report a case of alveolar hemorrhage associated with crescentic glomerulonephritis without immune complex deposition with a review of the literature.

Endobronchial metastasis occurs in about 2% to 5% of patients dying of solid tumors of extrathoracic origin. These metastatic tumors can simulate bronchogenic carcinoma and presenting symptoms include cough, hemoptysis, dyspnea and wheezing. In most cases of endobronchial metastasis, the histologic finding of the bronchoscope biopsy suggests the correct diagnosis. There are only a few reports of endobronchial metastasis from malignant melanoma. We report a case of malignant melanoma with endobronchial metastasis presenting with hemoptysis simulating primary bronchogenic carcinoma.

A 38-year-old female was admitted due to progressive exertional dyspnea and intermittent blood tinged sputum. Chest PA showed diffuse reticular infiltration accompanied by hyperinflation. was normal in resting state but profoundly decreased during exercise. Spirometry showed severe obstructive pattern but DLCO was markedly decreased. Lung volumes measured by helium equilibrium method was increased. On HRCT, numerous and relatively uniform sized cysts were evenly distributed throughout the bilateral lung fields. Open lung biopsy was performed and pulmonary lymphangioleiomyomatosis(LAM) was diagnosed based on the findings of abnormal proliferation of smooth muscle cells in the walls of lymphatic vessels, bronchioles, and small pulmonary veins. Immunohistochemical staining was negative for estrogen receptor but positive for progesterone receptor. Medroxyprogesterone therapy was initiated.

An experimental pathological study was performed to observe the effect of praziquantel treatment on the pulmonary lesions of the rat lung fluke, Paragonimus iloktsuenensis. The metacercariae were obtained from the freshwater crab, Sesarma dehaani, and 40 rats (wistar) were fed each with 10 metacercariae. On 20 rats praziquantel treatment (100mg/kg/day x 5 days) was done at 5 weeks after the infection while remaining 20 rats were kept untreated for use as controls. The drug-treated rats and the untreated ones were sacrificed 3, 7, 14, 21 or 28 days later for the observation of lung pathology. The rats infected with P. iloktsuenensis showed remarkable pulmonary changes; gross features of hemorrhagic and nodular worm capsules protruded on to the surface of the lung, and histologically local atelectasis, inflammatory cell infiltration, and egg granuloma around the worm capsules each containing one or two worms. Praziquantel treatment of the rats was shown to be highly effective in killing the worms and to lead them to degenerate, as early as in 3 days post-treatment. Almost all worms in the lung were dead and absorbed by the host cells in 21 days post-treatment, except a few living ones seen in a rat of 14-day post-treatment group. In most of the rats treated the pulmonary lesions showed the signs of resolution; regression of worm capsules with mummification of worms, decrease of inflammatory cell infiltration, improvement in the degree of atelectasis, and decreases in the frequency and size of the egg granuloma. From the results it is concluded that praziquantel is highly effective for the treatment of rat P. iloktsuenensis infection in the lung, not only by its direct killing effect of the worms but also due to the excellent resolution capacity of the pulmonary tissues.