Hormone Receptors in Lymphangioleiomyomatosis

Abstract

Corticosteroids are the primary treatment modality for most interstitial lung diseases. That some interstitial processes may be responsive to other hormonal agents is generally not appreciated. One component of the lung repair process is proliferation of bronchiolar and alveolar smooth muscle. In one rare and poorly understood disease, lymphangioleiomyomatosis (LAM), the predominant pathologic lesion is proliferation of smooth muscle throughout the lung, giving rise to emphysema with airway obstruction, interstitial disease, chylous effusions, and recurrent pneumothoraces. Smooth muscle proliferation is not confined to the chest, but may involve the abdomen and may result in lymphogenous cysts and chylous ascites. There is increasing evidence that the muscular hyperplasia of LAM is regulated by sex hormones; LAM occurs solely in women and usually during the child-bearing years. Following the appearance of radiographic abnormalities, the disease progresses rapidly, resulting in death within a few years. Following menopause, the pace of the disease is slowed and long-term survival is possible.