• Tuberculosis and Respiratory Diseases
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• v.49 no.2
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• pp.246-252
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• 2000

Lymphangioleiomyomatosis(LAM) is a rare disease of unknown etiology that occurs mainly in woman in her reproductive age. We recently experienced two cases of bilateral diffuse cystic lesion of the lung on chest X-ray and HRCT. The first case, a 26-year-old female, who had been diagnosed with tuberous sclerosis by the presence of clinical manifestation such as mental retardation, bilateral renal angiomyolipoma, adenoma sebaceum and generalized seizure, was admitted due to a recently developed hemoptysis. Chest PA showed diffuse ground-glass opacity with radiolucent cystic lesions of various sizes on both lung fields. HRCT showed innumerable small cystic lesions with suspicious diffuse ground-glass opacity on both lung fields The second case, a 30-year-old fema1e was admitted due to dyspnea and spewing of blood-tinged sputum for 2 weeks, shortly after delivery. Chest PA showed diffuse reticular and ground-glass opacities on both lung field. HRCT showed multiple well-defined and relatively uniform size air cysts with a uniform wall thickness on entire both lung fields, with small amount of right pleural effusion. By thoracoscopic lung biopsy she was diagnosed with pulmonary lymphangioleiomyomatosis. We report these cases with a brief review of the literatures.

• Tuberculosis and Respiratory Diseases
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• v.40 no.5
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• pp.519-531
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• 1993

Background: Lymphangioleiomyomatosis(LAM) is a very rare disease occurring in women of reproductive age and leading to progressive respiratory failure despite therapy. But the natural history of this disease is uncertain and although anti-estrogenic agents have been used for more than twenty years, it's efficacy is still in debate. This study was performed to enhance understanding of this fatal disease in Korea by examining clinical, radiological, and pathologic findings of all the previously reported cases of LAM on Korea along with four new cases of LAM whom we report in this paper. Method: Out of twlve cases of LAM previously unpublished and published in domestic papers, two cases whose diagnoses were considered doubtful after review of clinical, radiological, and pathologic findings at "Asian Congress on Lymphangioleiomyomatosis" at Kyoto, Japan in feburary of 1993 were excluded from this study. Six cases which were reported previously and four new cases of LAM whom we report in this paper were analysed for the clinical, radiological, and pathologic characteristics. Results: All ten patients were women with mean age of $33{\pm}7$. The most common symptom was exertional dyspnea and most patients had history of pneumothoraces. Pulmonary function tests showed decreased diffusing capacity. on high resolution computed tomography(HRCT), all the cases had characteristic cysts. Most of the patients did not respond to hormonal therapy. Conclusion: In women of reproductive age, presenting with dyspnea who has a history of pneumothorax, LAM should always be considered as one of the diagnostic possibilities. If suspected, HRCT should be done to look for characteristic cysts and if needed, open lung biopsy should be done to confirm the diagnosis.