Lymphangioleiomyomatosis (LAM) is a rare lung disease that results in disorderly smooth muscle proliferation throughout the bronchioles, alveolar septa, perivascular spaces, and lymphatics, resulting in the obstruction of small airways (leading to pulmonary cyst formation and pneumothorax) and lymphatics (leading to chylous pleural effusion). LAM o...

Is anyone aware of a reliable source to obtain demographic info regarding LAM?

The etiology of lymphangioleiomyomatosis (LAM) is unknown; however, the fact that the condition occurs primarily in women who are premenopausal and is exacerbated by high ER states suggests a role for hormones in this condition. The link with tuberous sclerosis (TSC) suggests a genetic component (see Other Problems to be Considered). ...

Initial symptoms are dyspnea and, less commonly, cough, chest pain, and hemoptysis. There are few signs of disease, but some women have crackles and rhonchi. Many patients present with spontaneous pneumothorax. They may also present with manifestations of lymphatic obstruction, including chylothorax, chylous ascites, and chyluria. Symptoms are thou...

Respiratory Function Testing Respiratory function may be normal initially but disease progression is associated with increasing airflow obstruction.[5-7,18] Reversibility testing in patients with airflow obstruction can result in modest improvements in airflow.[6,8] Gas transfer is generally reduced, often to a greater degree than the changes in ai...

The association of LAM with women of childbearing age suggests that hormonal stimulation plays a role in the disease process, and several approaches to treatment involve diminishing the effect of estrogen. At one time or another, therapeutic approaches have included progesterone oophorectomy tamoxifen gonadotropin-releasing hormone (GnRH) a...