A Case of Pregnancy in Patient with Pulmonary Lymphangioleiomyomatosis / 대한산부인과학회잡지

ABSTRACT

Pulmonary lymphangioleiomyomatosis is a rare disorder of unknown cause and characterised by hamartomatous proliferation of smooth muscle occurring in women of reproductive age exclusively. It causes dyspnea, recurrent pneumothorax, chylothorax, hemoptysis and respiratory failure eventually. Chest radiographs show diffuse interstitial infiltrates and cysts of uniform size, and pulmonary function tests often show airflow limitation with increase in residual volume. Hormonal factors are thought to play a role because it generally affects premenopausal women, but there is no definite treatment yet. We present an unusual case of pulmonary lymphangioleiomyomatosis during normal pregnancy with a review of literature.