| Lymphangioleiomyomatosis in Korea. |
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Eun Kyung Mo1, Man Pyo Jung1, Chul Gyu Yoo1, Young Whan Kim1, Sung Koo Han1, Jung Gi Im2, Jeong Wook Seo3, Seung Sook Lee3, Young Soo Shim1, Keun Youl Kim1, Yong Chol Han1 |
1Department of Internal Medicine, Seoul National University College of Medicine, Seoul, Korea
2Department of Radiology, Seoul National University College of Medicine, Seoul, Korea
3Department of Pathology, Seoul National University College of Medicine, Seoul, Korea |
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| Abstract |
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Out of twlve cases of LAM previously unpublished and published in domestic papers, two cases whose diagnoses were considered doubtful after review of clinical, radiological , and pathologic findings at “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
AII ten patients were women with mean age of 33 ± 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. |
| Key Words:
Lymphangioleiomyomatosis, Tuberous sclerosis, Lung, Korea |
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