Pulmonary lymphangioleiomyomatosis in Korea

Background—Pulmonary lymphangio-leiomyomatosis (LAM) is a rare disease occurring in women of reproductive age and leading to progressive respiratory failure in spite of treatment. In Korea the first case was reported in 1984 and by 1997 a total of 23 cases had been reported. The clinical findings of these Korean cases are reviewed. Methods—The details of 10 cases of LAM on file at Seoul National University Hospi-tal were reviewed together with those of 13 cases previously reported from other Korean institutes. Two, including the only one to be reported in a man, were excluded after reviewing the clinical, ra-diological, and pathological findings, leav-ing a total of 21 cases in the present study. Results—All 21 patients were women and in all cases the disease was proven patho-logically. The mean (SD) age at onset of symptoms was 32 (8.6) years. The most common symptoms were dyspnoea and pneumothorax which were seen in 19 (90%) and 13 (76%) patients, respectively. Pulmonary function tests showed de-creased transfer factor (TLCO) (100%) and airflow limitation (67%). All the cases had characteristic cysts on high resolution computed tomographic (HRCT) scan-ning. The overall severity score based on HRCT scans correlated with the percent-age predicted TLCO/VA (p = 0.03) and FEV1/FVC (p = 0.02). The patients were all treated with medroxyprogesterone and/or tamoxifen. Follow up was possible in 10 cases. Two of these patients appeared to stabilise with no appreciable change clini-cally or in lung function on medroxypro-gesterone and/or tamoxifen, but the remaining patients all deteriorated with two dying of respiratory insuYciency and one of infection following lung transplan-tation. Conclusions—As in other countries, in Korea LAM occurs exclusively in women and progresses despite hormonal treat-ment